Publications by authors named "Young-Il Koh"

49 Publications

Efficacy of add-on therapy with intravenous immunoglobulin in steroid hyporesponsive DRESS syndrome.

Clin Transl Sci 2021 Nov 18. Epub 2021 Nov 18.

Division of Allergy, Asthma, and Clinical Immunology, Department of Internal Medicine, Chonnam National University Medical School, Chonnam National University Hospital, Gwangju, Korea.

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare, potentially life-threatening, delayed, drug-induced hypersensitivity reaction. Immediate withdrawal of the culprit drug and administration of systemic corticosteroids is the most widely accepted treatment. However, it is difficult to manage patients with DRESS syndrome who are not responsive to systemic steroids. We studied the efficacy of intravenous immunoglobulins (IVIGs) in patients with DRESS syndrome unresponsive to systemic steroids. We retrospectively reviewed patients with DRESS syndrome who received IVIG in addition to systemic steroids during 2012-2017 and compared the clinical features and course of DRESS syndrome, before and after IVIG treatment. Eighteen DRESS patients (9 men) were included. The most frequent offending drugs were dapsone in five patients, followed by vancomycin in three patients, and carbamazepine in three patients. Rash, fever, lymphadenopathy, atypical lymphocytes, and hepatic involvement were common clinical findings. IVIG treatment was added within a median time of 7 days from the commencement of systemic steroid therapy. After IVIG treatment (total dosage: 1-2 g/kg), the fever resolved within a median time of 1 day (range, 0-3) and liver enzymes improved substantially within a median time of 13 days (range, 0-27). No severe adverse reactions related to IVIG therapy were observed in this study; however, there was one case of mortality. The addition of IVIG in DRESS syndrome in cases refractory to systemic steroid treatment may be helpful in hastening recovery. However, comparative studies using a placebo group are needed.
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http://dx.doi.org/10.1111/cts.13201DOI Listing
November 2021

High occurrence of simultaneous multiple-drug hypersensitivity syndrome induced by first-line anti-tuberculosis drugs.

World Allergy Organ J 2021 Jul 28;14(7):100562. Epub 2021 Jul 28.

Department of Allergy and Clinical Immunology, Chonnam National University Hospital, Chonnam National University Medical School, Gwangju, South Korea.

Background: Multiple drug hypersensitivity syndrome (MDHS) results in treatment delay or failure and often results in severe drug hypersensitivity reactions. There are few reports of MDHS in response to anti-tuberculosis drugs; however, clinical information is scarce. Understanding the frequency and clinical characteristics of simultaneous MDHS against first-line anti-tuberculosis drugs in patients with non-severe drug hypersensitivity reactions is necessary.

Methods: We reviewed 27 patients with drug fever or maculopapular exanthem in response to first-line anti-tuberculosis drugs between January 2010 and June 2019. Drug fever or maculopapular exanthem occurred when isoniazid, rifampin, ethambutol, and pyrazinamide were administered simultaneously. Drug provocation tests for the 4 drugs were performed to identify the culprit drugs.

Results: All patients showed positive reactions to 1 or more drugs. MDHS was diagnosed in 13 (48%) patients, of whom 11 and 2 patients reacted to 2 and 3 drugs, respectively. In comparison to the patients with single-drug hypersensitivity, the patients with MDHS did not exhibit any differences in characteristics. Ethambutol and rifampin were the common drugs that induced a reaction, and the combination of these 2 drugs induced MDHS most frequently. Among the patients with MDHS, there were no differences between the drugs that caused drug fever and maculopapular exanthem. All patients with MDHS were successfully treated with alternative drugs.

Conclusions: Simultaneous MDHS may occur frequently in patients with drug fever or maculopapular exanthem caused by first-line anti-tuberculosis drugs, indicating the need to evaluate the allergy responses for all 4 drugs, even in patients without severe drug hypersensitivity. The combination of ethambutol and rifampin was the most common trigger that induced MDHS.
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http://dx.doi.org/10.1016/j.waojou.2021.100562DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8339324PMC
July 2021

Cutaneous T-cell lymphoma in Asian patients: a multinational, multicenter, prospective registry study in Asia.

Int J Hematol 2021 Sep 24;114(3):355-362. Epub 2021 Jul 24.

Department of Internal Medicine, Division of Hemato-Oncology, Kangdong Sacred Heart Hospital, Hallym University, Seoul, South Korea.

Cutaneous T-cell lymphomas (CTCLs) are a group of T-cell lymphomas with low incidence. Due to their indolent characteristics, treatment strategies have not yet been established for advanced CTCLs. In this study, relative incidence of CTCLs in Asia was estimated and the therapeutic outcomes presented based on various treatments currently used in clinics for advanced CTCLs. As part of a prospective registry study of peripheral T-cell lymphoma (PTCL) conducted across Asia, including Korea, China, Taiwan, Singapore, Malaysia, and Indonesia, subgroup analysis was performed for patients with CTCLs. Among 486 patients with PTCL, 37 with CTCL (7.6%) were identified between April 2016 and February 2019. Primary cutaneous ALK-negative anaplastic large cell lymphoma (ALCL, 35.1%) was the most common subtype. With a median follow-up period of 32.1 months, median progression-free survival (PFS) was 53.5 months (95% CI 0.0-122.5), and overall survival was not reached. 14 patients (48.2%) underwent subsequent treatment after the first relapse, but the response rate was 20% with a PFS of 2.2 months (95% CI 0.3-4.0). Six patients received autologous stem cell transplantation (auto-SCT). However, auto-SCT did not result in better outcomes. Additional studies are needed on standard care treatment of advanced or refractory and relapsed CTCLs.
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http://dx.doi.org/10.1007/s12185-021-03179-7DOI Listing
September 2021

Specialist Perception of Severe Asthma in Korea: A Questionnaire Survey.

Allergy Asthma Immunol Res 2021 May;13(3):507-514

Department of Internal Medicine, Soonchunhyang University Bucheon Hospital, Bucheon, Korea.

The Working Group on Severe Asthma of the Korean Academy of Allergy and Clinical Immunology recently published an expert opinion paper on the management of severe asthma in Korea. When developing a consensus, the working group encountered several diagnostic and treatment issues and decided to perform a questionnaire survey of Korean specialists with regard to severe asthma. An e-mail with a uniform resource locator link to the questionnaire was sent to 121 asthma specialists, of whom 44.6% responded. The most commonly accepted definitions of severe asthma were a history of fatal exacerbation or an asthma-triggered need for mechanical ventilation, 3-4 oral corticosteroid (OCS) bursts/year, and maintenance of OCS therapy for 3-6 months per year. Before diagnosing severe asthma, most physicians contemplate chest computed tomography, seek to control chronic rhinosinusitis, and consider poor inhaler compliance. For patients with uncontrolled severe asthma accompanied by type 2 (T2)-high inflammation, most biologics available in Korea were considered appropriate, but gaps were apparent in terms of T2-low asthma treatments. These findings about specialist perception of diagnosis and treatment of severe asthma will inform the use of emerging new drugs and facilitate personalized therapy.
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http://dx.doi.org/10.4168/aair.2021.13.3.507DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7984944PMC
May 2021

Fixed drug eruption and anaphylaxis induced concurrently by erdosteine: a case report.

Allergy Asthma Clin Immunol 2021 Feb 5;17(1):15. Epub 2021 Feb 5.

Division of Allergy, Asthma, and Clinical Immunology, Department of Internal Medicine, Chonnam National University Medical School, 42 Jebong-ro, Dong-gu, Gwangju, 61469, Republic of Korea.

Background: Erdosteine is used as a mucolytic agent and has a low incidence of adverse drug reactions, most of which are gastrointestinal and mild. Moreover, drug antigens rarely induce multiple simultaneous immunologic reactions. Only one previous case report has demonstrated hypersensitivity reaction induced by erdosteine. Here, we report a case of fixed drug eruption and anaphylaxis, which were concurrently induced by erdosteine. The association between the symptoms and erdosteine was proven by a drug provocation test.

Case Presentation: A 35-year-old woman presented with recurrent angioedema and pruritic rash on the hands, which developed within 2 h following the administration of drugs, including erdosteine, for acute upper respiratory infection. Her rash was characterized by well-defined erythematous plaques, which recurred at the same site following the administration of the medications. She also experienced angioedema of the lips. Fixed drug eruption was considered after excluding other possible causes for the presented skin lesions. A drug provocation test confirmed that fixed drug eruption on both hands had occurred after administration of erdosteine, suggesting that erdosteine was the cause of the allergic reaction. However, she also experienced angioedema, isolated wheal, and laryngeal edema; thus, IgE-mediated type I hypersensitivity could also be concurrently occurring with the fixed drug eruption.

Conclusions: We report about a patient who was diagnosed with two different hypersensitivity reactions concurrently induced by erdosteine. We also demonstrate that patients may exhibit multiple simultaneous symptoms that usually arise from overlapping of different hypersensitivity mechanisms. Physicians should be aware of the possibility that some patients who are allergic to certain drugs could exhibit several symptoms caused by different mechanisms of hypersensitivity reactions simultaneously.
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http://dx.doi.org/10.1186/s13223-021-00517-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7866636PMC
February 2021

Hypersensitivity reactions to multiple anti-tuberculosis drugs.

PLoS One 2021 4;16(2):e0246291. Epub 2021 Feb 4.

Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Chonnam National University Hospital, Gwangju, South Korea.

Objective: This study aimed to evaluate hypersensitivity reactions to anti-tuberculosis (TB) drugs.

Methods: We retrospectively compared the clinical manifestations and treatment outcomes of single and multiple drug hypersensitivity reactions (DHRs).

Results: Twenty-eight patients were diagnosed with anti-TB DHRs using oral drug provocation tests. Of these 28 patients, 17 patients (60.7%) had DHRs to a single drug and 11 (39.3%) had multiple DHRs. The median age of patients was 57.5 years (interquartile range [IQR], 39.2-73.2). Of the total patients, 18 patients (64.3%) were men. The median number of anti-TB drugs causing multiple DHRs was 2.0 (IQR 2.0-3.0). Rifampin was the most common drug that caused DHRs in both the single and multiple DHR groups (n = 8 [47.1%] and n = 9 [52.9%], respectively). The treatment success rate was lower in the multiple DHR group than in the single DHR group; however, the difference was not statistically significant (81.8% vs. 94.1%; P = 0.543).

Conclusions: Multiple anti-TB DHRs were common in all patients who experienced DHRs, and rifampin was the most common causative drug. The treatment outcomes appeared to be poorer in patients with multiple DHRs than in those with single DHRs.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0246291PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7861523PMC
July 2021

Severe Cutaneous Adverse Reactions to Anti-tuberculosis Drugs in Korean Patients.

Allergy Asthma Immunol Res 2021 Mar;13(2):245-255

Department of Internal Medicine, Pusan National University College of Medicine, Busan, Korea.

Purpose: Anti-tuberculosis drugs (ATDs) can cause severe cutaneous adverse reactions (SCARs) such as Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and drug reaction with eosinophilia and systemic symptoms (DRESS). Underlying tuberculous infection and co-administration of multiple drugs may contribute to the complexity of ATD-related SCARs. This study aimed to investigate the clinical characteristics and outcomes of ATD-related SCARs.

Methods: We analyzed ATD-related SCAR cases in 2010-2015, selected from a web-based Database of the Korean Registry of SCAR.

Results: Among 783, 53 patients with ATD-induced SCARs were enrolled, including 12 with SJS/TEN (22.6%) and 41 with DRESS (77.4%). When comparing the ATD and non-ATD groups, the prevalence of DRESS patients was higher in the ATD group than in the non-ATD group (77.4% vs. 45.8%, < 0.001). Among patients with ATD-related SCARs, those with SJS/TEN were significantly older, had higher intensive care unit admissions, and had higher mortality than those with DRESS (70.5 vs. 50.0 years, < 0.001; 41.7% vs. 6.1%, = 0.010; and 33.3% vs. 2.5%, = 0.003, respectively). ATDs were challenged in 14 cases. The ATD associated most often with SCAR cases was rifampin (81.8%), followed by isoniazid (66.7%), ethambutol (50.0%), pyrazinamide (33.3%). Six patients (42.9%) had hypersensitivity reactions to 2 or more drugs.

Conclusions: DRESS was more common among the ATD-related SCAR cases. Although treatment with most ATDs carries the risk of SCAR development, the use of rifampin was most frequently involved in the occurrence of SCARs. Multiple hypersensitivity was frequently observed in ATD-related SCARs.
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http://dx.doi.org/10.4168/aair.2021.13.2.245DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7840880PMC
March 2021

Characteristics of Specialist-Diagnosed Asthma-COPD Overlap in Severe Asthma: Observations from the Korean Severe Asthma Registry (KoSAR).

Allergy 2021 01 28;76(1):223-232. Epub 2020 Jul 28.

Department of Internal Medicine, Hanyang University College of Medicine, Seoul, Korea.

Background: While the clinical characteristics and outcomes of asthma-chronic obstructive pulmonary disease (COPD) overlap (ACO) have been frequently compared with those of COPD or asthma, the prevalence and features of ACO in patients with severe asthma are unclear.

Objectives: Evaluation of the prevalence and clinical features of ACO using the Korean severe asthma registry.

Methods: At the time of registration, ACO was determined in patients with severe asthma by attending specialists. Patients were classified into ACO and non-ACO groups, and the demographic and clinical characteristics of these two groups were compared.

Results: Of 482 patients with severe asthma, 23.7% had ACO. Patients in the ACO group were more likely to be male (P < .001), older (P < .001), and ex- or current smokers (P < .001) compared with those in the non-ACO group. Patients in the ACO group had lower mean forced expiratory volume in 1 second (P < .001) and blood eosinophil percentage (P = .006), but higher blood neutrophil percentage (P = .027) than those in the non-ACO group. The ACO group used more inhaled long-acting muscarinic antagonist (P < .001), methylxanthine (P = .001), or sustained systemic corticosteroid (P = .002). In addition, unscheduled emergency department visits due to exacerbation were more frequent in the ACO group (P = .006).

Conclusion: Among patients with severe asthma, those with ACO were older, predominantly male, and were more likely to have a smoking history than those with asthma only. Patients with ACO used more systemic corticosteroid and had more frequent exacerbations related to emergency department visits than those with severe asthma only.
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http://dx.doi.org/10.1111/all.14483DOI Listing
January 2021

A Nationwide Study of Severe Cutaneous Adverse Reactions Based on the Multicenter Registry in Korea.

J Allergy Clin Immunol Pract 2021 02 19;9(2):929-936.e7. Epub 2020 Sep 19.

Department of Internal Medicine, Gyeongsang National University School of Medicine, Jinju, Korea.

Background: Because severe cutaneous adverse reactions (SCARs), such as Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and drug reaction with eosinophilia and systemic symptoms (DRESS) rarely occur, clinical data based on large-scale studies are still lacking.

Objective: To provide information on culprit drugs and clinical characteristics, including morbidity and mortality of SCARs based on a nationwide registry.

Methods: SCAR cases that occurred from 2010 to 2015 were recruited to the Korean SCAR registry from 34 tertiary referral hospitals. Demographics, causative drugs, causality, and clinical outcomes were collected by reviewing the medical record.

Results: A total of 745 SCAR cases (384 SJS/TEN cases and 361 DRESS cases) due to 149 drugs were registered. The main causative drugs were allopurinol (14.0%), carbamazepine (9.5%), vancomycin (4.7%), and antituberculous agents (6.3%). A strong preference for SJS/TEN was observed in carbonic anhydrase inhibitors (100%), nonsteroidal anti-inflammatory drugs (84%), and acetaminophen (83%), whereas dapsone (100%), antituberculous agents (81%), and glycopeptide antibacterials (78%) were more likely to cause DRESS. The mortality rate was 6.6% (SJS/TEN 8.9% and DRESS 4.2%). The median time to death was 19 days and 29 days in SJS/TEN and DRESS respectively, and 89.8% of deaths occurred within 60 days after the onset of the skin symptoms.

Conclusion: Allopurinol, carbamazepine, vancomycin, and antituberculous agents were the leading causes of SCARs in Korea. Some drugs preferentially caused a specific phenotype. The mortality rate of SCARs was 6.6%, and most of the deaths occurred within 2 months.
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http://dx.doi.org/10.1016/j.jaip.2020.09.011DOI Listing
February 2021

Evaluation and Management of Difficult-to-Treat and Severe Asthma: An Expert Opinion From the Korean Academy of Asthma, Allergy and Clinical Immunology, the Working Group on Severe Asthma.

Allergy Asthma Immunol Res 2020 Nov;12(6):910-933

Department of Internal Medicine, Soonchunhyang University Bucheon Hospital, Bucheon, Korea.

Severe asthma (SA) presents in about 3%-5% of adult asthmatics and is responsible for over 60% of asthma-related medical expenses, posing a heavy socioeconomic burden. However, to date, a precise definition of or clear diagnostic criteria for SA have not been established, and therefore, it has been challenging for clinicians to diagnose and treat this disease. Currently, novel biologics targeting several molecules, such as immunoglobulin E, interleukin (IL)5, and IL4/IL13, have emerged, and many new drugs are under development. These have brought a paradigm shift in understanding the mechanism of SA and have also provided new treatment options. However, we need to agree on a precise definition of and its diagnostic criteria for SA. Additionally, it is necessary to explain the diagnostic criteria and to summarize current standard and additional treatment options. This review is an experts' opinion on SA from the Korean Academy of Asthma, Allergy, and Clinical Immunology, the Working Group on Severe Asthma, and aims to provide a definition of and diagnostic criteria for SA, and propose future direction for SA diagnosis and management in Korea.
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http://dx.doi.org/10.4168/aair.2020.12.6.910DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7492516PMC
November 2020

The first case of tegoprazan-induced urticaria.

J Clin Pharm Ther 2020 Oct 29;45(5):1187-1189. Epub 2020 Jun 29.

Division of Allergy, Asthma, and Clinical Immunology, Department of Internal Medicine, Chonnam National University Medical School, Gwangju, Korea.

What Is Known And Objective: Tegoprazan induces adverse drug reactions during clinical trials; however, tegoprazan-induced urticaria has not been reported. Here, we describe the first case of this.

Case Description: A 55-year-old woman presented with acute urticaria with pruritus after taking the gastro-oesophageal reflux disease medication, tegoprazan. Urticaria disappeared after tegoprazan discontinuation. In an oral provocation test, after taking 10% of tegoprazan, she developed pruritus, and after taking 30%, she developed urticaria on her back.

What Is New And Conclusion: This is the first case of urticaria induced by tegoprazan. Physicians should understand the possibility of a tegoprazan-induced hypersensitivity reactions.
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http://dx.doi.org/10.1111/jcpt.13150DOI Listing
October 2020

A Prospective Observation of Psychological Distress in Patients With Anaphylaxis.

Allergy Asthma Immunol Res 2020 May;12(3):496-506

Department of Allergy and Clinical Immunology, Ajou University School of Medicine, Suwon, Korea.

Purpose: Anaphylaxis is an immediate allergic reaction characterized by potentially life-threatening, severe, systemic manifestations. While studies have evaluated links between serious illness and posttraumatic stress disorder (PTSD), few have investigated PTSD after anaphylaxis in adults. We sought to investigate the psychosocial burden of recent anaphylaxis in Korean adults.

Methods: A total of 203 (mean age of 44 years, 120 females) patients with anaphylaxis were recruited from 15 university hospitals in Korea. Questionnaires, including the Impact of Event Scale-Revised-Korean version (IES-R-K), the Korean version of the Beck Anxiety Inventory (K-BAI), and the Korean version of the Beck Depression Inventory (K-BDI), were administered. Demographic characteristics, causes and clinical features of anaphylaxis, and serum inflammatory markers, including tryptase, platelet-activating factor, interleukin-6, tumor necrosis factor-α, and C-reactive protein, were evaluated.

Results: PTSD (IES-R-K ≥ 25) was noted in 84 (41.4%) patients with anaphylaxis. Of them, 56.0% had severe PTSD (IES-R-K ≥ 40). Additionally, 23.2% and 28.1% of the patients had anxiety (K-BAI ≥ 22) and depression (K-BDI ≥ 17), respectively. IES-R-K was significantly correlated with both K-BAI ( = 0.609, < 0.0001) and K-BDI ( = 0.550, < 0.0001). Among the inflammatory mediators, tryptase levels were lower in patients exhibiting PTSD; meanwhile, platelet-activating factor levels were lower in patients exhibiting anxiety and depression while recovering from anaphylaxis. In multivariate analysis, K-BAI and K-BDI were identified as major predictive variables of PTSD in patients with anaphylaxis.

Conclusions: In patients with anaphylaxis, we found a remarkably high prevalence of PTSD and associated psychological distresses, including anxiety and depression. Physicians ought to be aware of the potential for psychological distress in anaphylactic patients and to consider psychological evaluation.
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http://dx.doi.org/10.4168/aair.2020.12.3.496DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7061156PMC
May 2020

Clinical Manifestations and Risk Factors of Anaphylaxis in Pollen-Food Allergy Syndrome.

Yonsei Med J 2019 Oct;60(10):960-968

Allergy and Clinical Immunology Research Center, Hallym University College of Medicine, Chuncheon, Korea.

Purpose: Many studies have reported that pollen-food allergy syndrome (PFAS) can cause anaphylaxis. No comprehensive investigations into anaphylaxis in PFAS have been conducted, however. In this study, we investigated the clinical manifestations and risk factors for anaphylaxis in PFAS in Korean patients with pollinosis.

Materials And Methods: Data were obtained from a nationwide cross-sectional study that previously reported on PFAS in Korean patients with pollinosis. Data from 273 patients with PFAS were collected, including demographics, list of culprit fruits and vegetables, and clinical manifestations of food allergy. We analyzed 27 anaphylaxis patients and compared them with patients with PFAS with oropharyngeal symptoms only (n=130).

Results: The most common cause of anaphylaxis in PFAS was peanut (33.3%), apple (22.2%), walnut (22.2%), pine nut (18.5%), peach (14.8%), and ginseng (14.8%). Anaphylaxis was significantly associated with the strength of sensitization to alder, hazel, willow, poplar, timothy, and ragweed (<0.05, respectively). Multivariable analysis revealed that the presence of atopic dermatitis [odds ratio (OR), 3.58; 95% confidence interval (CI), 1.25-10.23; =0.017]; sensitization to hazel (OR, 5.27; 95% CI, 1.79-15.53; =0.003), timothy (OR, 11.8; 95% CI, 2.70-51.64; =0.001), or ragweed (OR, 3.18; 95% CI, 1.03-9.87; =0.045); and the number of culprit foods (OR, 1.25; 95% CI, 1.15-1.37; <0.001) were related to the development of anaphylaxis in PFAS.

Conclusion: The most common culprit foods causing anaphylaxis in PFAS were peanut and apple. The presence of atopic dermatitis; sensitization to hazel, timothy, or ragweed; and a greater number of culprit foods were risk factors for anaphylaxis in PFAS.
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http://dx.doi.org/10.3349/ymj.2019.60.10.960DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6753338PMC
October 2019

Severe Cutaneous Adverse Reactions to Antiepileptic Drugs: A Nationwide Registry-Based Study in Korea.

Allergy Asthma Immunol Res 2019 Sep;11(5):709-722

Department of Pulmonology and Allergy, Hallym University Dongtan Sacred Heart Hospital, Hallym University College of Medicine, Hwaseong, Korea.

Purpose: Severe cutaneous adverse reactions (SCARs), including Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and drug reaction with eosinophilia and systemic symptoms (DRESS) to antiepileptic drug (AED), are rare, but result in significant morbidity and mortality. We investigated the major culprit drugs, clinical characteristics, and clinical course and outcomes of AED-induced SCARs using a nationwide registry in Korea.

Methods: A total of 161 patients with AED-induced SCARs from 28 referral hospitals were analyzed. The causative AEDs, clinical characteristics, organ involvements, details of treatment, and outcomes were evaluated. We compared the clinical and laboratory parameters between SJS/TEN and DRESS according to the leading causative drugs. We further determined risk factors for prolonged hospitalization in AED-induced SCARs.

Results: Carbamazepine and lamotrigine were the most common culprit drugs causing SCARs. Valproic acid and levetiracetam also emerged as the major causative agents. The disease duration and hospital stay in carbamazepine-induced SJS/TEN were shorter than those in other AEDs (< 0.05, respectively). In younger patients, lamotrigine caused higher incidences of DRESS than other drugs (= 0.045). Carbamazepine, the most common culprit drug for SCARs, was associated with a favorable outcome related with prolonged hospitalization in SJS (odds ratio, 0.12; 95% confidence interval, 0.02-0.63, = 0.12), and thrombocytopenia was found to be a risk factor for prolonged hospitalization in DRESS.

Conclusion: This was the first large-scale epidemiological study of AED-induced SCARs in Korea. Valproic acid and levetiracetam were the significant emerging AEDs causing SCARs in addition to the well-known offending AEDs such as carbamazepine and lamotrigine. Carbamazepine was associated with reduced hospitalization, but thrombocytopenia was a risk factor for prolonged hospitalization. Our results suggest that the clinical characteristics and clinical courses of AED-induced SCARs might vary according to the individual AEDs.
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http://dx.doi.org/10.4168/aair.2019.11.5.709DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6658409PMC
September 2019

Unique Clinical Characteristics and Prognosis of Allopurinol-Induced Severe Cutaneous Adverse Reactions.

J Allergy Clin Immunol Pract 2019 Nov - Dec;7(8):2739-2749.e3. Epub 2019 Jun 13.

Department of Internal Medicine, College of Medicine, Chung-Ang University, Seoul, Korea. Electronic address:

Background: Allopurinol is the most common cause of severe cutaneous adverse reactions (SCARs) in Korea due to the relatively high prevalence of the HLA-B*58:01 genotype (8%-13%).

Objective: We aimed to reveal the clinical characteristics and risk factors for death in allopurinol-induced SCARs in Korea.

Methods: We retrospectively reviewed the medical records of 106 subjects with allopurinol-induced SCARs and 639 subjects with other drug-induced SCARs who were enrolled in the Korean SCARs Registry (collected from 34 nationwide medical institutions) from January 2010 to December 2015.

Results: Subjects with allopurinol-induced Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) were older and had more comorbidities, longer latent periods, longer disease durations, more deranged laboratory findings, and increased disease severity resulting in a higher mortality rate (17.6% vs 7.6%; P = .020) compared with the subjects with other drug-induced SCARs. There was no significant difference in age or mortality in drug reaction with eosinophilia and systemic symptoms (DRESS). Subjects with allopurinol-induced SJS/TEN were older and had shorter latent periods and a higher mortality rate (17.6% vs 3.7%; P = .044) than those with allopurinol-induced DRESS. In allopurinol-induced SJS/TEN, significant risk factors for death included chronic renal insufficiency, intensive care unit (ICU) admission, increased blood urea nitrogen levels on admission day, serum peak eosinophil counts, baseline and peak creatinine levels, baseline and peak alanine aminotransferase levels, and decreased lowest platelet counts. In allopurinol-induced DRESS, significant risk factors for death included ICU admission and increased glucose levels on admission day.

Conclusions: Allopurinol-induced SCARs have unique characteristics and poor prognoses with important predictive factors of death.
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http://dx.doi.org/10.1016/j.jaip.2019.05.047DOI Listing
October 2020

A phase II trial of bendamustine, carboplatin, and dexamethasone for refractory or relapsed peripheral T-cell lymphoma (BENCART trial).

Leuk Lymphoma 2019 12 6;60(13):3251-3257. Epub 2019 Jun 6.

Department of Pathology, Korea Cancer Center Hospital, Korea Institute of Radiological and Medical Sciences, Seoul, Korea.

This trial was designed to investigate the efficacy and toxicity of bendamustine, carboplatin, and dexamethasone (BCD) for relapsed or refractory peripheral T-cell lymphomas (PTCLs), which would be expected to exhibit more promising clinical outcomes compared with bendamustine therapy alone. After treatments with BCD, eight patients exhibited a complete response (CR; 29%) and seven exhibited a partial response (PR; 25%). The overall response rate (ORR) was 54%. Five patients proceeded to ASCT and three patients finally achieved CR. The median progression-free survival (PFS) was 4.4 months (2.8-6.0, 95% CI). For a total of 85 cycles of BCD, grade 3 or 4 neutropenia, thrombocytopenia, and anemia occurred in 17.6, 38.8, and 16.5% of cycles, respectively. Only one patient experienced febrile neutropenia. BCD was a considerable salvage regimen for relapsed or refractory PTCLs with acceptable toxicity; AITL or ASCT eligible patients were more effective to BCD.NCT02424045.
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http://dx.doi.org/10.1080/10428194.2019.1622100DOI Listing
December 2019

Variation of clinical manifestations according to culprit drugs in DRESS syndrome.

Pharmacoepidemiol Drug Saf 2019 06 1;28(6):840-848. Epub 2019 May 1.

Division of Allergy, Asthma and Clinical Immunology, Department of Internal Medicine, Chonnam National University Medical School, Gwangju, Korea.

Purpose: Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare but serious condition that systematically damages various internal organs through T-cell-mediated immunological drug reactions. We aimed to investigate whether clinical manifestations of DRESS syndrome differ according to culprit drugs.

Methods: We retrospectively analyzed data from 123 patients with probable/definite DRESS syndrome based on the RegiSCAR criteria (January 2011 to July 2016). The data were obtained from the Korean Severe Cutaneous Adverse Reaction Registry. Causality was assessed using the World Health Organization-Uppsala Monitoring Centre criteria. The culprit drugs were categorized as allopurinol, carbamazepine, anti-tuberculosis drug, vancomycin, cephalosporins, dapsone, and nonsteroidal anti-inflammatory drugs.

Results: Differences were observed among culprit drugs regarding the frequencies of hepatitis (P < 0.01), renal dysfunction (P < 0.0001), lymphadenopathy (P < 0.01), and atypical lymphocyte (P < 0.01). Latency period differed among culprit drugs (P < 0.0001), being shorter in vancomycin and cephalosporin. In terms of clinical severity, admission duration (P < 0.01) and treatment duration (P < 0.05) differed among culprit drugs, being longer in vancomycin and anti-tuberculosis drugs, respectively.

Conclusions: Based on the findings, clinical manifestations, including latency period and clinical severity, may differ according to culprit drugs in DRESS syndrome.
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http://dx.doi.org/10.1002/pds.4774DOI Listing
June 2019

Erratum: Pollen-Food Allergy Syndrome in Korean Pollinosis Patients: A Nationwide Survey.

Allergy Asthma Immunol Res 2019 05;11(3):441-442

Department of Pulmonology and Allergy, Hallym University Dongtan Sacred Heart Hospital, Hallym University College of Medicine, Hwaseong, Korea.

This corrects the article on p. 648 in vol. 10, PMID: 30306747.
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http://dx.doi.org/10.4168/aair.2019.11.3.441DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6439189PMC
May 2019

Cutaneous adverse drug reactions in patients with peripheral blood eosinophilia during antituberculosis treatment.

Korean J Intern Med 2019 Sep 19;34(5):1050-1057. Epub 2019 Mar 19.

Department of Internal Medicine, Chonnam National University Hospital, Gwangju, Korea.

Background/aims: Peripheral eosinophilia during tuberculosis (TB) treatment is common, but has not been fully evaluated. The aim of this study was to determine the prevalence and clinical significance of peripheral blood eosinophilia in patients undergoing anti-TB treatment.

Methods: We retrospectively reviewed the clinical and laboratory data of patients who received anti-TB treatment and had peripheral blood eosinophilia (> 5% of the total white blood cell count) at the Chonnam National University Hospital between January 2010 and December 2014.

Results: Of all 2,234 patients with TB who received anti-TB treatment, 397 (17.8%) had peripheral blood eosinophilia. Of the 397 with eosinophilia, we reviewed the data of 262 (66%), and cutaneous adverse drug reactions (CADRs) were observed in 161 (61.5%). Of the 161 with CADRs, itching (47.2%) and skin rash (47.8%) were common. Older age, abnormal liver function, and higher peak blood eosinophil percentage were associated with CADRs in multivariate analysis. There was a significant relationship between increased peak eosinophil counts and the degree of severity of CADRs.

Conclusion: Peripheral blood eosinophilia is a relatively common occurrence during anti-TB treatment. Peripheral blood eosinophil counts were higher according to the degree of severity of CADRs.
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http://dx.doi.org/10.3904/kjim.2018.063DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6718751PMC
September 2019

A Fusion Protein of Derp2 Allergen and Flagellin Suppresses Experimental Allergic Asthma.

Allergy Asthma Immunol Res 2019 Mar;11(2):254-266

Clinical Vaccine R&D Center and Department of Microbiology, Chonnam National University Medical School, Gwangju, Korea.

Purpose: The house dust mite (HDM) is one of the most important sources of indoor allergens and a significant cause of allergic rhinitis and allergic asthma. Our previous studies demonstrated that flagellin B (FlaB) plus allergen as a co-treatment mixture improved lung function and inhibited eosinophilic airway inflammation through the Toll-like receptor 5 signaling pathway in an ovalbumin (OVA)- or HDM-induced mouse asthma model. In the present study, we fused the major mite allergen Derp2 to FlaB and compared the therapeutic effects of the Derp2-FlaB fusion protein with those of a mixture of Derp2 and FlaB in a Derp2-induced mouse asthma model.

Methods: BALB/c mice sensitized with Derp2 + HDM were treated with Derp2, a Derp2 plus FlaB (Derp2 + FlaB) mixture, or the Derp2-FlaB fusion protein 3 times at 1-week intervals. Seven days after the final treatment, the mice were challenged intranasally with Derp2, and airway responses and Derp2-specific immune responses were evaluated.

Results: The Derp2-FlaB fusion protein was significantly more efficacious in reducing airway hyperresponsiveness, lung eosinophil infiltration, and Derp2-specific IgE than the Derp2 + FlaB mixture.

Conclusions: The Derp2-FlaB fusion protein showed a strong anti-asthma immunomodulatory capacity, leading to the prevention of airway inflammatory responses in a murine disease model through the inhibition of Th2 responses. These findings suggest that the Derp2-FlaB fusion protein would be a promising vaccine candidate for HDM-mediated allergic asthma therapy.
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http://dx.doi.org/10.4168/aair.2019.11.2.254DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6340794PMC
March 2019

Severe Cutaneous Adverse Reactions in Korean Pediatric Patients: A Study From the Korea SCAR Registry.

Allergy Asthma Immunol Res 2019 Mar;11(2):241-253

Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea.

Purpose: Although severe cutaneous adverse drug reactions (SCARs) are rare, they are associated with high morbidity and mortality, and thus early diagnosis and treatment are critical for improving prognoses. However, few studies have reported the characteristics of SCARs in children. Thus, we aimed to evaluate the clinical characteristics, current management and prognosis of pediatric SCARs.

Methods: We analyzed pediatric data in the Korean SCARs registry, which was built retrospectively in 2016 with SCAR cases treated in 34 tertiary referral university hospitals during 2010-2015. Using these cases, we descriptively analyzed detailed data regarding etiology, clinical and laboratory features, treatment strategies, and prognosis.

Results: Forty-seven pediatric SCAR cases from 15 tertiary referral hospitals were included. The median patient age was 10 (interquartile range, 3-15.5) years and 68.1% (n = 32) were males. The culprit drug was identified in 95.7% (n = 45) of the patients; antibiotics (44.7%) and antiepileptic drugs (19.1%) were the most common and second most common culprits, respectively. Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) cases presented with the largest area of skin involvement without permanent sequelae. Stevens-Johnson syndrome (SJS) cases involved relatively small areas of skin but serious sequelae in two children. Of 4 patients with toxic epidermal necrolysis (TEN), 1 died. Of all patients assessed, 36 (76.6%) received systemic steroids and 21 (44.7%) received intravenous immunoglobulin (IVIG). Thirteen (27.7%) received both systemic steroids and IVIG. Cyclosporine was administered to only 1 patient along with a systemic steroid.

Conclusions: In patients with pediatric SCARs, including those with DRESS, SJS and TEN, clinical presentations were variable. Thus, there was no clear continuous disease spectrum. Although the mortality rate was low (2.1%), clinical suspicion may be the best tool for proactive SCAR management.
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http://dx.doi.org/10.4168/aair.2019.11.2.241DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6340806PMC
March 2019

Phenotypes of Severe Cutaneous Adverse Reactions Caused by Nonsteroidal Anti-inflammatory Drugs.

Allergy Asthma Immunol Res 2019 Mar;11(2):212-221

Department of Allergy and Clinical Immunology, Ajou University School of Medicine, Suwon, Korea.

Purpose: Nonsteroidal anti-inflammatory drugs (NSAIDs) are common cause of severe cutaneous adverse reactions (SCARs). The present study aimed to investigate the characteristics of SCARs induced by NSAIDs in the Korean SCAR registry.

Methods: A retrospective survey of NSAID-induced SCARs recorded between 2010 and 2015 at 27 university hospitals in Korea was conducted. Clinical phenotypes of SCARs were classified into Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), SJS-TEN overlap syndrome and drug reaction with eosinophilia and systemic symptoms (DRESS). Causative NSAIDs were classified into 7 groups according to their chemical properties: acetaminophen, and propionic, acetic, salicylic, fenamic and enolic acids.

Results: A total of 170 SCARs, consisting of 85 SJS, 32 TEN, 17 SJS-TEN overlap syndrome and 36 DRESS reactions, were induced by NSAIDs: propionic acids (n=68), acetaminophen (n=38), acetic acids (n=23), salicylic acids (n=16), coxibs (n=8), fenamic acids (n=7), enolic acids (n=5) and unclassified (n=5). Acetic acids (22%) and coxibs (14%) accounted for higher portions of DRESS than other SCARs. The phenotypes of SCARs induced by both propionic and salicylic acids were similar (SJS, TEN and DRESS, in order). Acetaminophen was primarily associated with SJS (27%) and was less involved in TEN (10%). DRESS occurred more readily among subjects experiencing coxib-induced SCARs than other NSAID-induced SCARs (62.5% vs. 19.7%, = 0.013). The mean time to symptom onset was longer in DRESS than in SJS or TEN (19.1 ± 4.1 vs. 6.8 ±1.5 vs. 12.1 ± 3.8 days). SCARs caused by propionic salicylic acids showed longer latency, whereas acetaminophen- and acetic acid-induced SCARs appeared within shorter intervals.

Conclusions: The present study indicates that the phenotypes of SCARs may differ according to the chemical classifications of NSAIDs. To establish the mechanisms and incidences of NSAID-induced SCARs, further prospective studies are needed.
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http://dx.doi.org/10.4168/aair.2019.11.2.212DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6340804PMC
March 2019

Differentiation of angioimmunoblastic T-cell lymphoma from DRESS syndrome.

J Allergy Clin Immunol Pract 2019 May - Jun;7(5):1684-1686.e1. Epub 2018 Dec 14.

Division of Allergy, Asthma, and Clinical Immunology, Department of Internal Medicine, Chonnam National University Medical School and Hospital, Gwangju, Korea. Electronic address:

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http://dx.doi.org/10.1016/j.jaip.2018.11.048DOI Listing
August 2020

Characteristics of Adult Severe Refractory Asthma in Korea Analyzed From the Severe Asthma Registry.

Allergy Asthma Immunol Res 2019 Jan;11(1):43-54

Division of Allergy and Clinical Immunology, Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Purpose: Although mild to moderate asthma is much more common, the morbidity and mortality of severe asthma are much higher. This study was performed to identify and analyze the clinical characteristics of severe asthma in Korea.

Methods: We registered patients with severe refractory asthma into the Severe Asthma Registry supported by the Severe Asthma Work Group of the Korean Academy of Asthma, Allergy and Clinical Immunology. Patients were enrolled since 2010 from the 15 university hospitals nationwide in Korea. Severe asthma was defined according to modified European Respiratory Society/American Thoracic Society criteria. Information on demographics, medical history, pulmonary function tests and skin prick tests was collected; the clinical characteristics of severe asthmatics were analyzed from the collected data.

Results: A total of 489 patients were enrolled with a mean age of 62.3; 45% are male. Sixty percent of patients received Global Initiative for Asthma step 4 treatment, and 30% received step 5 treatment. The most common comorbidities were allergic rhinitis (58.7%). Aspirin hypersensitivity was observed in 14.0%. Approximately half (53.9%) are non-smokers. Atopy was proven in 38.5% of the patients. Regarding asthma medications, inhaled corticosteroids and long-acting β-agonist combination inhalers were most commonly prescribed (96.5%), followed by leukotriene antagonists (71.0%). A recombinant anti-immunoglobulin E monoclonal antibody (omalizumab) has been used in 1.8% of the patients. The mean forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1) and FEV1/FVC were 78.7%, 67.5% and 67.9% of predicted values, respectively. The mean Asthma Control Test and quality of life questionnaire scores were 16.5 out of 25 and 59.5 out of 85, respectively.

Conclusions: The baseline characteristics of severe asthma patients in the Korea Severe Asthma Registry were analyzed and reported for the first time. With this cohort, further prospective studies should be performed to search for ways to improve management of severe refractory asthma.
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http://dx.doi.org/10.4168/aair.2019.11.1.43DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6267188PMC
January 2019

Complete response of hepatocellular carcinoma with right atrium and pulmonary metastases treated by combined treatments (a possible treatment effect of natural killer cell): A case report and literature review.

Medicine (Baltimore) 2018 Oct;97(42):e12866

Division of Gastroenterology.

Rationale: Hepatocellular carcinomas (HCCs) with metastases to the right atrium (RA) and lungs are rare, with a poor prognosis. Furthermore, the treatment outcomes in patients with advanced HCCs remain unsatisfactory.

Patient Concerns: A 46-year-old man presented to our hospital for dyspnea on exertion and abdominal pain.

Diagnoses: HCC and extra-hepatic metastases to the lung and RA.

Interventions: Multidisciplinary treatment including radiotherapy (RT), transarterial chemoembolization (TACE), and sorafenib. During a follow-up evaluation computed tomography, he experienced a radio-contrast-induced anaphylaxis. After the event, treatment such as RT, TACE, and sorafenib were continued.

Outcomes: His tumor burden decreased, finally leading to a complete response as per the modified Response Evaluation Criteria in Solid Tumors. The patient is still alive, 30 months after the episode. Subsequent blood tests showed increased natural killer (NK) cell activity, which was significantly higher than that seen in other age-matched HCC patients with an identical stage of the tumor, receiving sorafenib. This suggests that the increase in NK cells induced by anaphylaxis influenced the tumor burden.

Lessons: We report here a rare case of long-term survival of an HCC patient with multiple metastases treated with multidisciplinary modalities, in which high NK cell activity was observed after a radio-contrast-induced anaphylactic reaction during follow-up investigations.
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http://dx.doi.org/10.1097/MD.0000000000012866DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6211840PMC
October 2018

Pollen-Food Allergy Syndrome in Korean Pollinosis Patients: A Nationwide Survey.

Allergy Asthma Immunol Res 2018 Nov;10(6):648-661

Department of Pulmonology and Allergy, Hallym University Dongtan Sacred Heart Hospital, Hallym University College of Medicine, Hwaseong, Korea.

Purpose: Pollen-food allergy syndrome (PFAS) is an immunoglobulin E (IgE)-mediated allergy in pollinosis patients caused by raw fruits and vegetables and is the most common food allergy in adults. However, there has been no nationwide study on PFAS in Korea. In this study, we investigated the prevalence and clinical characteristics of PFAS in Korea.

Methods: Twenty-two investigators participated in this study, in which patients with allergic rhinoconjunctivitis and/or bronchial asthma with pollen allergy were enrolled. The questionnaires included demographic characteristics, a list of fruits and vegetables, and clinical manifestations of food allergy. Pollen allergy was diagnosed by skin prick test and/or measurement of the serum level of specific IgE.

Results: A total of 648 pollinosis patients were enrolled. The prevalence of PFAS was 41.7% (n = 270). PFAS patients exhibited cutaneous (43.0%), respiratory (20.0%), cardiovascular (3.7%) or neurologic symptoms (4.8%) in addition to oropharyngeal symptoms. Anaphylaxis was noted in 8.9% of the PFAS patients. Seventy types of foods were linked to PFAS; e.g., peach (48.5%), apple (46.7%), kiwi (30.4%), peanut (17.4%), plum (16.3%), chestnut (14.8%), pineapple (13.7%), walnut (14.1%), Korean melon (12.6%), tomato (11.9%), melon (11.5%) and apricot (10.7%). Korean foods such as taro/taro stem (8.9%), ginseong (8.2%), perilla leaf (4.4%), bellflower root (4.4%), crown daisy (3.0%), deodeok (3.3%), kudzu root (3.0%) and lotus root (2.6%) were also linked to PFAS.

Conclusions: This was the first nationwide study of PFAS in Korea. The prevalence of PFAS was 41.7%, and 8.9% of the PFAS patients had anaphylaxis. These results will provide clinically useful information to physicians.
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http://dx.doi.org/10.4168/aair.2018.10.6.648DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6182195PMC
November 2018

Clinical Features of Hereditary Angioedema in Korean Patients: A Nationwide Multicenter Study.

Int Arch Allergy Immunol 2018 25;176(3-4):272-279. Epub 2018 Apr 25.

Institute of Allergy and Clinical Immunology, Seoul National University Medical Research Center, Seoul, Republic of Korea.

Background: Hereditary angioedema (HAE) is a genetically heterogeneous autosomal dominant disorder characterized by recurrent episodes of nonpruritic, nonpitting edema increasing after puberty. It can be fatal due to laryngeal or gastrointestinal (GI) involvement with varied and changing frequency of mortality according to studies published from the Western countries. Epidemiological and clinical data of HAE in Asian countries are sparse. We sought to examine the clinical characteristics of HAE patients in Korea.

Methods: Patients diagnosed with HAE at 15 tertiary hospitals across the country until 2016 were retrospectively reviewed.

Results: A total of 65 patients diagnosed with HAE by 2016 were identified. The prevalence of HAE was estimated at 1.3/1,000,000 in Korea. Of the 65 patients, 21 (32.3%) were males. A total of 90.8% patients had type I HAE, while the remaining 9.2% patients had type II HAE. The first symptom developed after 20 years in 73.8% of patients, with a mean age 28.4 ± 14.1 years. The age at diagnosis was 36.5 ± 15.8 years, with a mean time delay of 7.8 ± 10.5 years. While the face (82.3%) and extremities (upper 71.0%, lower 62.9%) were the most frequently involved, the GI tract was affected in 40.5% of Korean HAE patients. Prophylaxis was maintained in 62.5% of patients. There was no reported case of death from HAE so far.

Conclusions: The clinical manifestation and severity of HAE may vary according to ethnicity. HAE is more infrequent and GI involvement is less likely in Korea compared with Western countries.
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http://dx.doi.org/10.1159/000488350DOI Listing
July 2018

Unusual Clinical Manifestation of Laryngeal Edema in a Case of DRESS Syndrome.

J Allergy Clin Immunol Pract 2018 Nov - Dec;6(6):2108-2109. Epub 2018 Mar 21.

Division of Allergy, Asthma, and Clinical Immunology, Department of Internal Medicine, Chonnam National University Medical School and Chonnam National University Hospital, Gwangju, Korea.

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http://dx.doi.org/10.1016/j.jaip.2018.03.001DOI Listing
November 2019

A Case of Schnitzler's Syndrome without Monoclonal Gammopathy-Associated Chronic Urticaria Treated with Anakinra.

Yonsei Med J 2018 Jan;59(1):154-157

Department of Internal Medicine, Chonnam National University Medical School, Gwangju, Korea.

Chronic urticaria may often be associated with interleukin (IL)-1-mediated autoinflammatory disease, which should be suspected if systemic inflammation signs are present. Here, we report a case of Schnitzler's syndrome without monoclonal gammopathy treated successfully with the IL-1 receptor antagonist anakinra. A 69-year-old man suffered from a pruritic urticarial rash for 12 years. It became aggravated episodically and was accompanied by high fever, arthralgia, leukocytosis, and an elevated C-reactive protein and erythrocyte sedimentation rate. The episodes each lasted for over one week. Neutrophilic and eosinophilic inflammation was found on skin biopsy. However, serum and urine electrophoresis showed no evidence of monoclonal gammopathy. The cutaneous lesions were unresponsive to various kinds of anti-histamines, systemic glucocorticoids, colchicine, cyclosporine, dapsone, and methotrexate, which were administered over a span of 3 years immediately preceding successful treatment. A dramatic response, however, was observed after a daily administration of anakinra. This observation suggests that the correct diagnosis of this case is Schnitzler's syndrome without monoclonal gammopathy. For an adult patient with refractory chronic urticaria and systemic inflammation, Schnitzler's syndrome could be considered as a possible differential diagnosis. Although the typical form of Schnitzler's syndrome exhibits the presence of monoclonal gammopathy as a diagnostic criterion, monoclonal gammopathy may be absent in an atypical form. In such a situation, an IL-1 antagonist should be effective for the management of chronic urticaria.
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http://dx.doi.org/10.3349/ymj.2018.59.1.154DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5725354PMC
January 2018

Unmet Primary Physicians' Needs for Allergic Rhinitis Care in Korea.

Allergy Asthma Immunol Res 2017 May;9(3):265-271

Department of Internal Medicine, Chonnam National University Medical School & Hospital, Gwangju, Korea.

Allergic rhinitis (AR) is one of the most common chronic allergic respiratory diseases worldwide. Various practical guidelines for AR have been developed and updated to improve the care of AR patients; however, up to 40% patients remain symptomatic. The unmet need for AR care is one of the greatest public health problems in the world. The gaps between guideline and real-world practice, and differences according to the region, culture, and medical environments may be the causes of unmet needs for AR care. Because there is no evidence-based AR practical guideline reflecting the Korean particularity, various needs are increasing. The purpose of the study was to evaluate whether existing guidelines are sufficient for AR patient management in real practice and whether development of regional guidelines to reflect regional differences is needed in Korea. A total of 99 primary physicians comprising internists, pediatricians, and otolaryngologists (n=33 for each) were surveyed by a questionnaire relating to unmet needs for AR care between June 2 and June 16 of 2014. Among 39 question items, participants strongly agreed on 15 items that existing guidelines were highly insufficient and needed new guidelines. However, there was some disagreement according to specialties for another 24 items. In conclusion, the survey results demonstrated that many physicians did not agree with the current AR guideline, and a new guideline reflecting Korean particularity was needed.
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http://dx.doi.org/10.4168/aair.2017.9.3.265DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5352578PMC
May 2017
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