Publications by authors named "Paola Parronchi"

62 Publications

Orofacial Granulomatosis: Clinical and Therapeutic Features in an Italian Cohort And Review of the Literature.

Allergy 2021 Feb 28. Epub 2021 Feb 28.

Center for Basic and Clinical Immunology Research (CISI), WAO Center of Excellence, Department of Translational Medical Sciences, Allergy and Clinical Immunology, University of Naples Federico II, Naples, Italy.

Background: Orofacial granulomatosis (OFG) is characterized by granulomatous inflammation of the soft tissues of maxillofacial region. We explored OFG patients from 10different Italian Centers and summarized the most recent literature data.

Methods: a review ofpatients with OFG was carried out. An extensive online literature search was performed to identify studies reporting diagnosis and management of OFG.

Results: Thirty-nine patients were recruited between January 2018 and February 2020. Most of them (97.4%) displayed involvement of the lipsand 28.2% suffered from Melkersson-Rosenthal Syndrome. Two patients received diagnosis of CD and one patient of sarcoidosis, suggesting secondary OFG.Oral aphthosis and cervical lymphadenopathy were also described.The mean diagnostic delay was 3.4 years.Histological evaluation was performed in 34/39 patients (87.2%); non-caseating granulomas were found in 73.5% of them.Neurological symptoms (28.2%), gastrointestinal symptoms in absence of overt inflammatory bowel disease (IBD) (20.5%) and atopy (35.9%) were also identified. Therapeutic approaches varied among the centers. Steroids (51.3%) were used with good or partial results.Anti-TNF-α and anti-IgE monoclonal antibodieswere used in 6(15.4%) and 1(2.6%) patients, respectively, with variable results. Surgery was the choice for 2 patients with good response.

Conclusions: OFG is a rare and neglected disease showing multiple clinical phenotypes. While early diagnosis is crucial,managementis difficult and highly dependent on the expertise of clinicians due to the lack of international guidelines. There is a need to establishregistry databases and address challenges of long-term management.
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http://dx.doi.org/10.1111/all.14799DOI Listing
February 2021

Epidemiology of systemic sclerosis: a multi-database population-based study in Tuscany (Italy).

Orphanet J Rare Dis 2021 Feb 17;16(1):90. Epub 2021 Feb 17.

Institute of Clinical Physiology, National Research Council, Via Moruzzi 1, Pisa, Italy.

Background: Systemic Sclerosis (SSc) is a chronic autoimmune disease with a complex pathogenesis that includes vascular injury, abnormal immune activation, and tissue fibrosis. We provided a complete epidemiological characterization of SSc in the Tuscany region (Italy), considering prevalence and incidence, survival, comorbidities and drug prescriptions, by using a multi-database population-based approach. Cases of SSc diagnosed between 1st January 2003 and 31st December 2017 among residents in Tuscany were collected from the population-based Rare Diseases Registry of Tuscany. All cases were linked to regional health and demographic databases to obtain information about vital statistics, principal causes of hospitalization, complications and comorbidities, and drug prescriptions.

Results: The prevalence of SSc in Tuscany population resulted to be 22.2 per 100,000, with the highest prevalence observed for the cases aged ≥ 65 years (33.2 per 100,000, CI 95% 29.6-37.3). In females, SSc was predominant (86.7% on the total) with an overall sex ratio F/M of 6.5. Nevertheless, males presented a more severe disease, with a lower survival and significant differences in respiratory complications and metabolic comorbidities. Complications and comorbidities such as pulmonary involvement (HR = 1.66, CI 95% 1.17-2.35), congestive heart failure (HR = 2.76, CI 95% 1.80-4.25), subarachnoid and intracerebral haemorrhage (HR = 2.33, CI 95% 1.21-4.48) and malignant neoplasms (HR = 1.63, CI 95% 1.06-2.52), were significantly associated to a lower survival, also after adjustment for age, sex and other SSc-related complications. Disease-modifying antirheumatic drugs, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors were the drugs with the more increasing prevalence of use in the 2008-2017 period.

Conclusions: The multi-database approach is important in the investigation of rare diseases where it is often difficult to provide accurate epidemiological indicators. A population-based registry can be exploited in synergy with health databases, to provide evidence related to disease outcomes and therapies and to assess the burden of disease, relying on a large cohort of cases. Building an integrated archive of data from multiple databases linking a cohort of patients to their comorbidities, clinical outcomes and survival, is important both in terms of treatment and prevention.
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http://dx.doi.org/10.1186/s13023-021-01733-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7890847PMC
February 2021

Sex Differences in Severe Asthma: Results From Severe Asthma Network in Italy-SANI.

Allergy Asthma Immunol Res 2021 Mar;13(2):219-228

Department of Surgery, Medicine and Molecular Biology and Critical Care, Nuovo Ospedale Apuano, University of Pisa, Pisa, Italy.

Purpose: After adolescence, asthma is more frequent in females than in males due to different hormonal, immunologic, and occupational/environmental factors. The higher prevalence and severity of the disease in females have already been reported in international registries. The aim of this study was to explore the difference in terms of clinical, functional, and biological characteristics between male and female patients with severe asthma in a real-life, registry-based setting.

Methods: Baseline data from the Severe Asthma Network in Italy registry were analyzed in 1,123 patients with severe asthma, according to sex.

Results: Almost 2/3 of severe asthmatics were female. Late-onset asthma, obesity and gastro-esophageal reflux were more frequent in females than in males, while previous smoking habits and nasal polyposis were more frequent in males. Females had poor asthma control and a higher number of severe exacerbations leading to hospitalization, in comparison to males. Biomarkers of type 2 inflammation (blood eosinophil, exhaled nitric oxide, and serum immunoglobulin E levels) were significantly higher in males than in females. The type 2 profile (defined by a combination of these 3 biomarkers) was significantly more frequent in males than in females. In multivariate analysis, late-onset asthma and a normal body mass index were only independent variables associated with the type 2 profile, while male sex and age showed only a trend toward the association with the type 2 profile.

Conclusions: Significant differences may be observed between male and female patients with severe asthma, influencing the asthma pheno-endotyping in both sexes.
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http://dx.doi.org/10.4168/aair.2021.13.2.219DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7840868PMC
March 2021

Spectrum of Fibrotic Lung Diseases.

N Engl J Med 2020 12;383(25):2485

University of Florence, Florence, Italy

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http://dx.doi.org/10.1056/NEJMc2031135DOI Listing
December 2020

Pulmonary vascular improvement in severe COVID-19 patients treated with tocilizumab.

Immunol Lett 2020 12 5;228:122-128. Epub 2020 Nov 5.

Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy; Immunology and Cell Therapy Unit, Careggi University Hospital, Florence, Italy. Electronic address:

As of October 2020 management of Coronavirus disease 2019 (COVID-19) is based on supportive care and off-label or compassionate-use therapies. On March 2020 tocilizumab - an anti-IL-6 receptor monoclonal antibody - was suggested as immunomodulatory treatment in severe COVID-19 because hyperinflammatory syndrome occurs in many patients similarly to the cytokine release syndrome that develops after CAR-T cell therapy. In our retrospective observational study, 20 severe COVID-19 patients requiring intensive care were treated with tocilizumab in addition to standard-of-care therapy (SOC) and compared with 13 COVID-19 patients receiving only SOC. Clinical respiratory status, inflammatory markers and vascular radiologic score improved after one week from tocilizumab administration. On the contrary, these parameters were stable or worsened in patients receiving only SOC. Despite major study limitations, improvement of alveolar-arterial oxygen gradient as well as vascular radiologic score after one week may account for improved pulmonary vascular perfusion and could explain the more rapid recovery of COVID-19 patients receiving tocilizumab compared to controls.
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http://dx.doi.org/10.1016/j.imlet.2020.10.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7644186PMC
December 2020

Efficacy and Safety of High-Dose Immunoglobulin-Based Regimen in Statin-Associated Autoimmune Myopathy: A Multi-Center and Multi-Disciplinary Retrospective Study.

J Clin Med 2020 Oct 27;9(11). Epub 2020 Oct 27.

Department of Medicine, Rheumatology Clinic, University of Udine, ASUFC Udine, 33100 Udine, Italy.

Statin-associated autoimmune myopathy is a rare muscle disorder, characterized by autoantibodies against HMGCR. The anti-HMGCR myopathy persists after statin, and often requires immunosuppressive therapy. However, there is not a standardized therapeutic approach. The purpose of this study is to report the effectiveness of the immunosuppressive treatment employed in a multi-center and multi-disciplinary cohort of patients affected by anti-HMGCR myopathy, in which an immunoglobulin (IVIG)-based treatment strategy was applied. We collected 16 consecutive patients with a diagnosis of anti-HMGCR myopathy, between 2012 and 2019, and recorded data on clinical and laboratory presentation (i.e., muscle strength, serum CK levels, and anti-HMGCR antibody titer) and treatment strategies. Our results highlight the safety and efficacy of an induction therapy combining IVIG with GCs and/or methotrexate to achieve persistent remission of the disease and steroid-free maintenance. Under IVIG-based regimens, clinical improvement and CK normalization occurred in more than two thirds of patients by six months. Relapse rate was low (3/16) and 2/3 relapses occurred after treatment suspension. Nearly 90% of the patients who successfully discontinued GCs were treated with a triple immunosuppressive regimen. In conclusion, an IVIG-based regimen, which particularly includes high-dose immunoglobulin, GCs and methotrexate, can provide a fast remission achievement with GC saving.
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http://dx.doi.org/10.3390/jcm9113454DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7692610PMC
October 2020

A multicenter approach to evaluate omalizumab effectiveness in Samter's triad.

Monaldi Arch Chest Dis 2020 Aug 6;90(3). Epub 2020 Aug 6.

Respiratory Diseases Unit, Department of Medical and Surgical Sciences and Neurosciences, University of Siena.

Omalizumab proved to be very effective in improving control of severe atopic asthma. Many small-sized studies suggested a potential role for omalizumab in the management of aspirin-exacerbated respiratory disease. The aim of this study is to describe the effectiveness of omalizumab in a multicentre group of patients with Samter's triad. We retrospectively enrolled eight patients (5 females) with Samter's triad who underwent at least one year of omalizumab therapy. Clinical data, functional parameters and questionnaires for asthma and nasal polyposis control were collected at baseline and follow-up.  We observed a significant reduction of moderate-to-severe asthma exacerbations, together with an increase of FEV1 and a reduction of steroids intake. An improvement in asthma control and nasal symptoms was also reported. This multicenter study confirms the effectiveness of omalizumab in patients affected by Samter's triad. Omalizumab may represent a potential therapeutic option for the management of this disease.
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http://dx.doi.org/10.4081/monaldi.2020.1397DOI Listing
August 2020

Disseminated Mycobacterium xenopi in an Adult with IL-12Rβ1 Deficiency.

J Clin Immunol 2020 11 27;40(8):1166-1170. Epub 2020 Aug 27.

Department of Experimental and Clinical Medicine, University of Florence, Largo Brambilla 3, 50134, Florence, FI, Italy.

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http://dx.doi.org/10.1007/s10875-020-00848-wDOI Listing
November 2020

Clinical Features at Onset and Genetic Characterization of Pediatric and Adult Patients with TNF- Receptor-Associated Periodic Syndrome (TRAPS): A Series of 80 Cases from the AIDA Network.

Mediators Inflamm 2020 7;2020:8562485. Epub 2020 Aug 7.

Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy.

This study explores demographic, clinical, and therapeutic features of tumor necrosis factor receptor-associated periodic syndrome (TRAPS) in a cohort of 80 patients recruited from 19 Italian referral Centers. Patients' data were collected retrospectively and then analyzed according to age groups (disease onset before or after 16 years) and genotype (high penetrance (HP) and low penetrance (LP) gene variants). Pediatric- and adult-onset were reported, respectively, in 44 and 36 patients; HP and LP variants were found, respectively, in 32 and 44 cases. A positive family history for recurrent fever was reported more frequently in the pediatric group than in the adult group ( < 0.05). With reference to clinical features during attacks, pericarditis and myalgia were reported more frequently in the context of adult-onset disease than in the pediatric age (with < 0.01 and < 0.05, respectively), while abdominal pain was present in 84% of children and in 25% of adults ( < 0.01). Abdominal pain was significantly associated also to the presence of HP mutations ( < 0.01), while oral aphthosis was more frequently found in the LP variant group ( < 0.05). Systemic amyloidosis occurred in 25% of subjects carrying HP variants. As concerns laboratory features, HP mutations were significantly associated to higher ESR values ( < 0.01) and to the persistence of steadily elevated inflammatory markers during asymptomatic periods ( < 0.05). The presence of mutations involving a cysteine residue, abdominal pain, and lymphadenopathy during flares significantly correlated with the risk of developing amyloidosis and renal impairment. Conversely, the administration of colchicine negatively correlated to the development of pathologic proteinuria ( < 0.05). Both NSAIDs and colchicine were used as monotherapy more frequently in the LP group compared to the HP group ( < 0.01). Biologic agents were prescribed to 49 (61%) patients; R92Q subjects were more frequently on NSAIDs monotherapy than other patients ( < 0.01); nevertheless, they required biologic therapy in 53.1% of cases. At disease onset, the latest classification criteria for TRAPS were fulfilled by 64/80 (80%) patients (clinical plus genetic items) and 46/80 (57.5%) patients (clinical items only). No statistically significant differences were found in the sensitivity of the classification criteria according to age at onset and according to genotype ( < 0.05). This study describes one of the widest cohorts of TRAPS patients in the literature, suggesting that the clinical expression of this syndrome is more influenced by the penetrance of the mutation rather than by the age at onset itself. Given the high phenotypic heterogeneity of the disease, a definite diagnosis should rely on both accurate working clinical assessment and complementary genotype.
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http://dx.doi.org/10.1155/2020/8562485DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7428902PMC
August 2020

A gendered magnifying glass on COVID-19.

Clin Mol Allergy 2020 4;18:14. Epub 2020 Aug 4.

Department of Experimental and Clinical Medicine, University of Florence, Largo Brambilla 3, 50134 Florence, Italy.

COVID-19 pandemia is affecting Countries worldwide with a gendered death excess as being a male represents, especially in the 50-69 years age group, an unfavourable factor. Females are constitutionally prone to defend themselves against pathogens with a stronger efficiency than males. As a fact, several genes involved into the regulation of the innate and adaptive immune response are strategically placed on the X-chromosome and, among them, pathogen-related receptors (PRRs), such as Toll-like receptor 7, suitable to recognize ssRNAs and trigger a gendered successful anti-viral fight. On the other hand, a more regulated IL-6 production and a more contained inflammation after the encounter of a pathogen supply score points in favour of the female sex in the view that an abnormal and exaggerated cytokine release does represent the hallmark of the deathful SARS-CoV-2 infection. The sex-prevalent expression of the attachment and permissive molecules ACE2 and TMPRSS2 further supports the concept of a male-oriented vulnerability. In this review, the possible role of biological and immunological sex differences into the higher morbidity and mortality of SARS-CoV-2 between females and males are discussed.
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http://dx.doi.org/10.1186/s12948-020-00129-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7402393PMC
August 2020

Low-Dose Mepolizumab Effectiveness in Patients Suffering From Eosinophilic Granulomatosis With Polyangiitis.

Allergy Asthma Immunol Res 2020 Sep;12(5):885-893

Immunoallergology Unit, Careggi University Hospital, Florence, Italy.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a vasculitis characterized by multisystemic manifestations including asthma. Mepolizumab (300 mg/4 weeks) has recently been approved for EGPA. However, real-life data are scarce and report experiences with high doses of mepolizumab intravenously administered (750 mg/4 weeks). The aim of our study was to investigate in a real-life setting whether mepolizumab in EGPA patients at low doses would enable us 1) to control asthma symptoms, 2) to obtain oral corticosteroids (OCS) and/or immunosuppressors tapering and 3) to maintain clinical remission and avoid disease relapses. Mepolizumab (100 mg/4 weeks) was subcutaneously administered for 12 months in 18 EGPA patients with uncontrolled severe asthma. Symptoms, annual asthma exacerbation rates, OCS-sparing effects, lung function and eosinophil activation markers were monitored. The proportion of patients with clinical remission or relapse was also evaluated in month 12. A significant decrease in the annual rate of asthma exacerbations in association with significant changes in asthma control were observed. Specifically, 66.6% of the patients experienced no exacerbations during the mepolizumab treatment. Most patients (77.7%) were able to reduce the daily OCS dose by at least 50%. Four patients also stopped cyclosporine A during the study period. No EGPA relapse was observed and a large majority of the patients achieved clinical remission (94.3%). Clinical benefits were paralleled by reduction in blood eosinophils and serum levels of eosinophil activation markers. Low-dose mepolizumab showed clinically relevant benefits in exacerbation rates, asthma symptoms, OCS and immunosuppressive use in EGPA patients. These effects occurred without any EGPA relapse for extrapulmonary manifestations.
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http://dx.doi.org/10.4168/aair.2020.12.5.885DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7346991PMC
September 2020

Prompt Predicting of Early Clinical Deterioration of Moderate-to-Severe COVID-19 Patients: Usefulness of a Combined Score Using IL-6 in a Preliminary Study.

J Allergy Clin Immunol Pract 2020 Sep 19;8(8):2575-2581.e2. Epub 2020 Jun 19.

Immunoallergology Unit, Careggi University Hospital, Florence, Italy. Electronic address:

Background: The early identification of patients at risk of clinical deterioration is of interest considering the timeline of COVID-19 after the onset of symptoms.

Objective: The aim of our study was to evaluate the usefulness of testing serum IL-6 and other serological and clinical biomarkers, to predict a short-term negative clinical course of patients with noncritical COVID-19.

Methods: A total of 208 patients with noncritical COVID-19 pneumonia at admission were consecutively enrolled. Clinical and laboratory findings obtained on admission were analyzed by using survival analysis and stepwise logistic regression for variable selection. Three-day worsening as outcome in a logistic model to generate a prognostic score was used.

Results: Clinical worsening occurred in 63 patients (16 = died; 39 = transferred to intensive care unit; 8 worsening of respiratory failure). Forty-five of them worsened within 3 days after admission. The risk of clinical worsening was progressively enhanced along with increasing quartiles of IL-6 levels. Multivariate analysis showed that IL-6 (P = .005), C-reactive protein (CRP) (P = .003), and SaO/FiO (P = .014) were the best predictors for clinical deterioration in the first 3 days after admission. The combined score yielded an area under the curve = 0.88 (95% confidence interval: 0.83-0.93). A nomogram predicting the probability of 3-day worsening was generated. The score also showed good performance for 7-day and 14- or 21-day worsening and in predicting death occurring during all the follow-up.

Conclusions: Combining IL-6, CRP, and SaO/FiO in a score may help clinicians to identify on admission those patients with COVID-19 who are at high risk for a further 3-day clinical deterioration.
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http://dx.doi.org/10.1016/j.jaip.2020.06.013DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7303032PMC
September 2020

Is asthma protective against COVID-19?

Allergy 2021 03 17;76(3):866-868. Epub 2020 Jun 17.

SOS Allergy and Clinical Immunology, USL Toscana Centro, Prato, Italy.

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http://dx.doi.org/10.1111/all.14426DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7300712PMC
March 2021

Impaired immune cell cytotoxicity in severe COVID-19 is IL-6 dependent.

J Clin Invest 2020 09;130(9):4694-4703

Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.

BACKGROUNDCoronavirus disease 19 (COVID-19) is an emerging infectious disease caused by SARS-CoV-2. Antiviral immune response is crucial to achieve pathogen clearance; however, in some patients an excessive and aberrant host immune response can lead to an acute respiratory distress syndrome. The comprehension of the mechanisms that regulate pathogen elimination, immunity, and pathology is essential to better characterize disease progression and widen the spectrum of therapeutic options.METHODSWe performed a flow cytometric characterization of immune cell subsets from 30 patients with COVID-19 and correlated these data with clinical outcomes.RESULTSPatients with COVID-19 showed decreased numbers of circulating T, B, and NK cells and exhibited a skewing of CD8+ T cells toward a terminally differentiated/senescent phenotype. In agreement, CD4+ T and CD8+ T, but also NK cells, displayed reduced antiviral cytokine production capability. Moreover, a reduced cytotoxic potential was identified in patients with COVID-19, particularly in those who required intensive care. The latter group of patients also showed increased serum IL-6 levels that inversely correlated to the frequency of granzyme A-expressing NK cells. Off-label treatment with tocilizumab restored the cytotoxic potential of NK cells.CONCLUSIONThe association between IL-6 serum levels and the impairment of cytotoxic activity suggests the possibility that targeting this cytokine may restore antiviral mechanisms.FUNDINGThis study was supported by funds from the Department of Experimental and Clinical Medicine of University of Florence (the ex-60% fund and the "Excellence Departments 2018-2022 Project") derived from Ministero dell'Istruzione, dell'Università e della Ricerca (Italy).
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http://dx.doi.org/10.1172/JCI138554DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7456250PMC
September 2020

Unexpected Clues From Positron Emission Tomography in a Case of Fever of Unknown Origin.

J Clin Rheumatol 2020 Feb 4. Epub 2020 Feb 4.

Internal Medicine Unit 3, Careggi University Hospital, Firenze, Italy.

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http://dx.doi.org/10.1097/RHU.0000000000001377DOI Listing
February 2020

Linear Immunoglobulin A Bullous Disease (LABD) Triggered by Amoxicillin Clavulanic Acid in a Child.

J Allergy Clin Immunol Pract 2020 04 23;8(4):1398-1399.e1. Epub 2019 Dec 23.

Department of Health Science, Division of Dermatology, University of Florence, Anna Meyer Children's University Hospital, Florence, Italy.

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http://dx.doi.org/10.1016/j.jaip.2019.11.037DOI Listing
April 2020

Aetiopathogenesis of severe cutaneous adverse reactions (SCARs) in children: A 9-year experience in a tertiary care paediatric hospital setting.

Clin Exp Allergy 2020 01 28;50(1):61-73. Epub 2019 Oct 28.

Allergy Unit, Department of Paediatrics, Anna Meyer Children's University Hospital, Florence, Italy.

Background: Severe cutaneous adverse reactions (SCARs) are delayed-type hypersensitivity reactions to drugs including as follows: Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS), Stevens-Johnson syndrome (SJS), Toxic Epidermal Necrolysis (TEN) and Acute Generalized Exanthematous Pustulosis (AGEP). Incidence, triggers and management of SCARs have not been investigated in large-scale epidemiological studies on children.

Objective: The aim of our study was to collect epidemiological, clinical and aetiological data from children with SCARs referred to our tertiary care paediatric hospital of Florence.

Methods: From 2010 to 2018 charts of children with diagnosis of SCAR were reviewed, and data collected during the acute phase and/or the subsequent allergy evaluation. Patients underwent patch tests, intradermal tests and lymphocyte transformation tests. All children were investigated for infectious diseases.

Results: Incidence of SCARs in hospitalized children was 0.32% over a 9-year period. Fifty-four children were enrolled (31 M; 23 F; median age 6.5 years): 17 cases of DRESS, 30 SJS, 3 TEN, 2 AGEP, 1 linear immunoglobulin A bullous disease (LABD) and 1 pemphigus. Twenty-eight out of 54 patients underwent drug allergy investigations, and 50% of them resulted positive. Combining clinical history and results of allergy work-up, 74% SCARs seem to be caused by drugs, 18.6% by both drugs and infections, 3.7% by infections, and 3.7% remained idiopathic. No deaths occurred.

Conclusions: In this study, SCARs incidence is in line with literature data. Drugs were most commonly the leading cause. Management of SCARs requires cooperation among professional figures for an early diagnosis and a prompt treatment. Mortality rate seems to be lower in children.
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http://dx.doi.org/10.1111/cea.13513DOI Listing
January 2020

Autoantibodies to protein-arginine deiminase (PAD) 4 in rheumatoid arthritis: immunological and clinical significance, and potential for precision medicine.

Expert Rev Clin Immunol 2019 10 13;15(10):1073-1087. Epub 2019 Oct 13.

Research and Development, Inova Diagnostics , San Diego , CA , USA.

: The protein-arginine deiminase (PAD) 4 enzyme plays an important role in the pathogenesis of rheumatoid arthritis (RA) and also represents an antigenic target. Anti-PAD4 antibodies can be present in RA and are associated with specific clinical features. : This review aims to analyze the current knowledge and recent findings on anti-PAD4 antibodies in RA and their clinical and immunological significance. : Anti-PAD4 antibodies are not currently used in clinical practice for the management of RA. Nevertheless, there is growing evidence of their relevance in RA, and of their potential utility to improve diagnosis, patient stratification, and prognosis.
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http://dx.doi.org/10.1080/1744666X.2020.1668778DOI Listing
October 2019

Gender differences in anaphylaxis.

Curr Opin Allergy Clin Immunol 2019 10;19(5):417-424

Purpose Of Review: Is sexual dimorphism also true in anaphylaxis as described in other allergic diseases? Possible gender differences in the epidemiology, triggers, severity, outcomes of anaphylaxis as well as in the pathogenesis of the disease are discussed.

Recent Findings: Hormonal status and the X-chromosome-coded factors deeply involved in the regulation of T-cell and B-cell responses may influence the gender difference noticed in allergic diseases, such as asthma and rhinitis. Little is known if sex is also relevant for anaphylaxis, although the description of catamenial anaphylaxis is intriguing. However, epidemiologic bias, lack of reliable animal models for the human disease, differences into diagnostic codes and not harmonized clinical grading unfortunately represent hurdles to obtain meaningful information on this topic.

Summary: The female sex predisposes to a dysregulation of the immune response as suggested by the increased prevalence of autoimmunity and atopy. In anaphylaxis, pathomechanisms are not fully disclosed, triggers are numerous and IgE-dependent mast cell degranulation only represents a part of the story. Improvement into the definition of the disease including a more careful coding system and better investigations about triggers seem the only way to allow a more precise assessment of the possible different risk for women to develop anaphylaxis.
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http://dx.doi.org/10.1097/ACI.0000000000000568DOI Listing
October 2019

The multiple roles of mite allergens in allergic diseases.

Curr Opin Allergy Clin Immunol 2019 12;19(6):623-631

Purpose Of Review: Mites are the most worldwide spread allergens and relevant causative of respiratory allergies. Life cycle, component allergens, biological activity and immunogenicity are discussed in depth.

Recent Findings: It is now known that mite allergens are able to stimulate the innate immune system through different receptors, for example, TLRs and PARs. The activation of the cells in the airway mucosa is followed by type 2 polarizing cytokine production in predisposed individuals. This complex network plays a pivotal role into the promotion of Th2 differentiation.

Summary: This is a comprehensive review regarding all the mite allergens known so far, including their location within dust mites, composition, biological activities and binding receptors relevant to the fate of the immunological response.
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http://dx.doi.org/10.1097/ACI.0000000000000573DOI Listing
December 2019

Intravenous immunoglobulin therapy: a snapshot for the internist.

Intern Emerg Med 2019 10 15;14(7):1041-1049. Epub 2019 Jul 15.

Experimental and Clinical Medicine Department, University of Firenze, Largo Brambilla 3, 50100, Firenze, Italy.

Intravenous immunoglobulins are the cornerstone for the treatment of primary humoral immunodeficiencies and may be used for a great number of other autoimmune, neurological and hematological conditions as well. Given their wide application, the possibility of running across a patient who needs this kind of therapy is becoming increasingly common. Generally, intravenous immunoglobulins are well tolerated. However, numerous adverse reactions ranging from mild to severe have been reported and linked to patient- and product-related factors. For all these reasons, we present herein a comprehensive review of the on- and off-label applications of intravenous immunoglobulins and provide a guide for the internist how to minimize the risk of adverse reactions and manage them.
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http://dx.doi.org/10.1007/s11739-019-02150-zDOI Listing
October 2019

Belimumab reduces antiphospholipid antibodies in SLE patients independently of hydroxychloroquine treatment.

Autoimmun Rev 2019 03 11;18(3):312-314. Epub 2019 Jan 11.

Department of Experimental and Clinical Medicine, University of Firenze, Italy.

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http://dx.doi.org/10.1016/j.autrev.2018.11.002DOI Listing
March 2019

The percentage of patients achieving complete remission of urticaria increases with repeated courses of treatment.

J Allergy Clin Immunol Pract 2019 Jan 2;7(1):339-340. Epub 2018 Jul 2.

Immunoallergology Unit, Careggi University Hospital, Florence, Italy. Electronic address:

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http://dx.doi.org/10.1016/j.jaip.2018.06.011DOI Listing
January 2019

Amoxicillin/clavulanic acid-induced exfoliative dermatitis in a child.

Minerva Pediatr 2018 10;70(5):495-496

Allergy Unit, Department of Pediatrics, Anna Meyer Children's University Hospital, Florence, Italy.

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http://dx.doi.org/10.23736/S0026-4946.17.04845-9DOI Listing
October 2018

Omalizumab dampens type 2 inflammation in a group of long-term treated asthma patients and detaches IgE from FcεRI.

Eur J Immunol 2018 12 9;48(12):2005-2014. Epub 2018 Oct 9.

Department of Experimental and Clinical Medicine and DENOTHE Center, University of Florence, Florence, Italy.

Even if omalizumab is broadly used in the treatment of severe, allergic asthma, the immunological effects in long-term treated patients have not been fully elucidated. To this aim, a cohort of 15 allergic asthmatic patients treated with omalizumab for at least three years was compared with 12 allergic asthma patients treated with standard therapy. Omalizumab treated asthmatic patients showed lower frequencies of circulating plasmacytoid DCs, and lower CD154 expression on CD4 T-helper cells than the control group. Moreover, basophils and DCs from omalizumab-treated patients had lower surface expression of IgE compared to the control group. In a longitudinal evaluation of two patients that started omalizumab treatment, we show that FcεRI free of IgE were evident on basophils just after four weeks of drug administration. Finally, in vitro experiments with basophils obtained from healthy donors confirm that omalizumab is able to detach IgE from high affinity IgE receptors. Collectively these data indicate that long-term omalizumab treatment dampens type 2 inflammation acting on different cell types that play a pivotal role in the pathogenesis of allergic asthma. Moreover, we have identified a further mechanism of action of omalizumab, such as the ability to detach IgE from its receptor.
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http://dx.doi.org/10.1002/eji.201847668DOI Listing
December 2018

Sensitization to amoxicillin/clavulanic acid may underlie severe rashes in children treated for infectious mononucleosis.

J Allergy Clin Immunol Pract 2019 02 16;7(2):728-731.e1. Epub 2018 Aug 16.

Department of Experimental and Clinical Medicine, Internal Medicine Unit, University of Florence, Florence, Italy.

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http://dx.doi.org/10.1016/j.jaip.2018.06.022DOI Listing
February 2019