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Ann Otol Rhinol Laryngol 2020 Jul 2:3489420938827. Epub 2020 Jul 2.

Department of Otorhinolaryngology-Head and Neck Surgery, IRCCS Ospedale Policlinico San Martino, University of Genoa, Genoa, Italy.

Objective: To report presentation, diagnostic process, management and outcome of a case of autoimmune inner ear disease (AIED) related with Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA), treated with cochlear implantation, and review of relevant literature.

Case Presentation And Management: A retrospective case report of AIED associated with EGPA treated with cochlear implantation was described. A multi-step approach for diagnosis and confirmation of AIED and hearing rehabilitation was conducted, eventually leading to left cochlear implantation. Read More

Cesk Patol 2020 ;56(2):68-73

The most common group of systemic vasculitides in adulthood are anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). AAV represent autoimmune systemic vasculitides and include 3 clinical phenotypes: Granulomatosis with polyangiitis (GPA, formerly Wegener granulomatosis), Microscopic polyangiitis (MPA) and Eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome). Histological features are similar to each other in all affected locations, and there are represented by necrotizing vascular inflammation of small and medium calibers, often venules, capillaries or arteriols, typically with fibrinoid vessel wall necrosis. Read More

Cesk Patol 2020 ;56(2):65-67

ANCA positive vasculitis (AAV) is a serious autoimmune disease mainly affecting small vessels in various organ systems, accompanied by the presence of ANCA antibodies in serum. AAV represents a group of the most common systemic vasculitis in adulthood, and based on clinical manifestations this disease entity includes 3 phenotypes, namely: granulomatosis with polyangiitis (formerly Wegeners granulomatosis), microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome). Similar to other autoimmune diseases, AAV develops in patients with a predisposing genetic background who have been exposed to causative environmental factors, such as infections. Read More

Praxis (Bern 1994) 2020 ;109(7):509-512

Medizinische Klinik, Zuger Kantonsspital, Baar.

Mononeuritis Multiplex: A Diagnostic Challenge Eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome) is a multifaceted disease. Due to the variability in vascular and organ involvement, EPGA can manifest itself very differently. We report a case of a 60-year-old patient with a known bronchial asthma, pansinusitis and newly blood eosinophilia with a rapid-onset mononeuritis multiplex. Read More

Clin Rheumatol 2020 May 4. Epub 2020 May 4.

Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea.

Objectives: Serum IgG4 may often increases in allergic diseases. Eosinophilic granulomatosis with polyangiitis (EGPA) has an allergic phase. For this reason, it was questionable whether IgG4-RD and EGPA may share some clinical and laboratory features. Read More

Ann Cardiol Angeiol (Paris) 2020 May 4;69(3):148-150. Epub 2020 Apr 4.

Service de médecine interne, hôpital Habib Thameur, 8, rue Ali Ben Ayed, 1008 Tunis, Tunisie; Faculté de médecine de Tunis, université de Tunis El Manar, Tunis, Tunisie.

Churg-Strauss syndrome (CSS) or eosinophilic granulomatosis with polyangiitis is a systemic vasculitis characterized by necrotizing arteritis, eosinophilic infiltration and extravascular granuloma; that may involve several organs. Cardiac involvement is the first cause of death in this vasculitis. These include myocarditis, pericarditis, coronary heart disease, dysrhythmias, and rarely valvular involvement. Read More

Pediatr Dermatol 2020 Mar 24. Epub 2020 Mar 24.

Dermatology Inc., Indianapolis, Indiana.

Background/objectives: This study seeks to better define the clinical presentation and histopathology of cutaneous manifestations in childhood eosinophilic granulomatosis with polyangiitis (cEGPA).

Methods: Case reports were collected from Ovid Medline Database and PubMed using keyword identifiers from 1946 to 2017. Adult patients ≥ 18 years and cases not diagnosed with EGPA by the author were excluded. Read More

Acta Clin Belg 2020 Mar 13:1-8. Epub 2020 Mar 13.

Department of Nephrology, AZ St. Elisabeth Hospital, Zottegem, Belgium.

: Guillain-Barré Syndrome usually presents with ascending symmetric polyneuropathy, typically preceded by a viral infection. Despite the low incidence, physicians will often include Guillain-Barré Syndrome in their differential diagnosis. However, another underlying cause of polyneuropathy known as ANCA-associated vasculitis (AAV) is even more rare than Guillain-Barré Syndrome and therefore is usually overlooked. Read More

Cardiovasc Pathol 2020 Jul - Aug;47:107193. Epub 2019 Dec 18.

Department of Cellular and Organ Pathology, Graduate School of Medicine, Akita University, Akita, Japan.

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly called Churg-Strauss syndrome, is an uncommon disease with pathological features consisting of systemic necrotizing vasculitis, eosinophilic infiltration, and granulomatous or nongranulomatous extravascular eosinophilic inflammation. EGPA preferentially affects certain organ systems, including the airways, peripheral nerves, heart, kidney, and gastrointestinal tract. Although gastrointestinal involvement, such as ulcerations, is common in EGPA, gastrointestinal perforation is relatively uncommon and is associated with a poor prognosis. Read More

Clin Otolaryngol 2020 May 20;45(3):316-326. Epub 2020 Mar 20.

West Suffolk Hospital, Bury St. Edmunds, Suffolk, UK.

ENT involvement is common in ANCA-associated vasculitis (AAV), particularly in GPA and EGPA. Early recognition and treatment is important for good outcomes, yet evidence suggests that UK ENT surgeons may not consistently recognise the early features of AAV, despite a similar incidence to vestibular schwannoma. AAV is a rapidly advancing field, with significant developments in the understanding of its pathogenesis, classification and treatment over the past decade. Read More

Expert Opin Drug Metab Toxicol 2020 Feb 24;16(2):111-123. Epub 2020 Feb 24.

Amsterdam UMC, Department of Gastroenterology and Hepatology, VU University Medical Center, AG&M Research Institute, Amsterdam, Netherlands.

: In the 1950s, thioguanine (TG), a thiopurine-derivative together with azathioprine (AZA) and mercaptopurine (MP), were developed for the treatment of childhood leukemia. Over the years, the use of TG was also explored for other, mainly immune-mediated and inflammatory, diseases such as in the field of dermatology and rheumatology (. psoriasis, systemic lupus erythematosus (SLE)) and gastroenterology and hepatology (. Read More

Autoimmun Rev 2020 Apr 15;19(4):102495. Epub 2020 Feb 15.

Norwich Medical School, University of East Anglia, Norwich, United Kingdom.

Objective: Renal involvement in anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis is associated with significant morbidity and higher mortality rates. This study examined clinical manifestations associated with renal involvement in ANCA-associated vasculitis within a large, international cross-sectional cohort.

Methods: Univariate and multivariate analyses were performed to identify clinical factors associated with renal disease, which was defined as i) a serum-creatinine >30% above normal and a fall in creatinine-clearance >25%; or ii) haematuria attributable to active vasculitis. Read More

Rheumatol Int 2020 Aug 3;40(8):1301-1307. Epub 2020 Feb 3.

Genetics, GlaxoSmithKline, Upper Providence, PA, USA.

Treatment of patients with the rare disease eosinophilic granulomatosis with polyangiitis (EGPA) with mepolizumab, a monoclonal antibody to interleukin-5 (IL-5) that reduces blood eosinophil counts, as an add-on therapy to glucocorticoid treatment, results in more accrued weeks in remission, reductions in glucocorticoid use and reductions in relapse rate. However, treatment response varies across a continuum. Therefore, to investigate if large genetic effects could identify responders, the impact of genetic variants on efficacy in EGPA subjects taking mepolizumab and glucocorticoids was assessed in this post hoc study. Read More

Eur J Intern Med 2020 Apr 29;74:18-28. Epub 2020 Jan 29.

Autoimmune Diseases Unit, Internal Medicine Department, Centro Hospitalar de Trás-os-Montes e Alto Douro, Avenida da Noruega, 5000-508 Vila Real, Portugal; NEDAI/SPMI Autoimmune Diseases Group/Portuguese Society of Internal Medicine, Portugal. Electronic address:

Vasculitis is characterized by inflammation of the vascular wall. It reaches vessels of different sizes and locations, conditioning multisystem and complex manifestations that require a holistic approach. Antineutrophil cytoplasmic antibody-associated vasculitis has an annual incidence rate of 20 per million inhabitants. Read More

Harefuah 2020 Jan;159(1):34-37

Department of Dermatology, Emek Medical Center, Afula, Israel.

Aims: This study aims to critically review the pros and cons of biological drugs as treatments and triggers of eosinophilic dermatoses.

Background: Eosinophilic dermatoses syndromes are rare diseases with a prominent eosinophilic infiltration mechanism. These syndromes have several known treatments with limited success. Read More

Clin Rheumatol 2020 May 2;39(5):1581-1590. Epub 2020 Jan 2.

Division of Pulmonary and Critical Care Medicine, Mayo Clinic, 200 First St SW, Rochester, MN, 55905, USA.

Objectives: Rituximab (RTX) treatment is used for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, but its benefits in eosinophilic granulomatosis with polyangiitis (EGPA) are unclear. Our aim was to characterize asthma control and glucocorticoid (GC) sparing after RTX treatment.

Methods: A retrospective, computer-assisted search was performed to identify patients with EGPA and GC-dependent asthma diagnosed between 2000 and 2017 who received RTX for remission induction. Read More

Clin Dermatol 2019 Sep - Oct;37(5):528-547. Epub 2019 Jul 17.

The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York, USA. Electronic address:

The color purple can be seen in several types of eruptions including inflammatory dermatoses like lichen planus, infectious dermatoses like ecthyma gangrenosum, neoplasms like Kaposi sarcoma, and vasculitis and vasculopathy. The current review focuses on the clinical appearance, pathophysiology, and treatment of several vasculitides and vasculopathies including capillaritis, cutaneous small-vessel vasculitis, immunoglobulin A (IgA) vasculitis, cryoglobulinemia, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis, polyarteritis nodosum, warfarin-induced skin necrosis, heparin-induced thrombocytopenia, purpura fulminans, antiphospholipid antibody syndrome, calciphylaxis, levamisole-induced vasculopathy, and thrombotic thrombocytopenic purpura. Dermatologists play a central role in treating patients with cutaneous vasculitis and vasculopathy and may have the opportunity to facilitate identification of systemic disease by diagnosing cutaneous vasculitis and vasculopathy. Read More

Rheumatology (Oxford) 2020 01;59(1):77-83

Department of Internal Medicine, Referral Center for Rare Systemic and Autoimmune Diseases, Hôpital Cochin, APHP, Université Paris-Descartes, Paris Cedex 14, France.

Objective: To identify the role of Staphylococcus aureus (SA) or its intracellular small-colony variant phenotype (SCV) with co-trimoxazole (CTX) or ANCA-associated vasculitis (AAV) activity.

Methods: All consecutive AAV patients with granulomatosis with polyangiitis (GPA), eosinophilic GPA or microscopic polyangiitis, followed at the French National Vasculitis Referral Center (09/2012-05/2013), and hospitalized non-AAV controls, exclusively for SA/SCV carriage comparisons, were enrolled. All had bilateral anterior nasal swab cultures. Read More

Int Forum Allergy Rhinol 2020 02 2;10(2):217-222. Epub 2019 Dec 2.

Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, MN.

Background: In this study we aim to describe presenting characteristics and identify prognostic factors for disease resolution in patients with chronic rhinosinusitis (CRS) in the setting of eosinophilic granulomatosis with polyangiitis (EGPA).

Methods: Patients evaluated at a tertiary care center with diagnoses of EGPA and CRS were identified. Descriptive statistics were obtained. Read More

Expert Rev Clin Immunol 2020 Jan 17;16(1):51-61. Epub 2020 Jan 17.

INSERM UMR1227, Lymphocytes B et Autoimmunité, Université de Bretagne Occidentale, CHU de Brest, Brest, France.

: Eosinophilic granulomatosis with polyangiitis (EGPA) usually occurs in patients with late-onset asthma and sustained peripheral blood eosinophilia and classically presents with a clinical multifaceted spectrum of manifestations, which may vary at the different stages of the natural history of the disease.: We reviewed EGPA clinical presentation, focusing on clinical manifestations at three different phases of the disease: 1/before the development of overt vasculitis, 2/at vasculitis diagnosis and 3/during the long-term follow-up. An update on current classification criteria and recent therapeutic advances has been provided as well. Read More

J Pediatr Ophthalmol Strabismus 2019 Nov;56(6):373-377

Purpose: To describe four pediatric cases in which isolated orbital pseudotumor preceded the development of a systemic inflammatory disease by months to years.

Methods: The medical records of all patients with the clinical diagnosis of orbital pseudotumor seen at the Ocular Oncology Service of Wills Eye Hospital and Northern Virginia Ophthalmology Associates from 2010 to 2015 were reviewed retrospectively, and those associated with systemic inflammatory disease were selected for further study. Data were retrospectively collected from medical record review regarding patient demographics and clinical features, time to development of systemic inflammatory disease, and medical management. Read More

BMC Genomics 2019 Oct 11;20(1):727. Epub 2019 Oct 11.

Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA.

Background: Heart disease (HD) is the major cause of morbidity and mortality in patients with hypereosinophilic diseases. Due to a lack of adequate animal models, our understanding of the pathophysiology of eosinophil-mediated diseases with heart complications is limited. We have discovered a mouse mutant, now maintained on an A/J inbred background, that spontaneously develops hypereosinophilia in multiple organs. Read More

Int J Rheum Dis 2019 Oct 30;22(10):1926-1932. Epub 2019 Sep 30.

Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Hacettepe University, Ankara, Turkey.

Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and immunoglobulin G4-related disease (IgG4-RD) have some common features. The co-occurrence/concurrence of AAV and IgG4-RD was recently published by the collaborative European Vasculitis Study Group. First, we aimed to investigate ANCA positivity of our IgG4-RD cohort. Read More

Neurol India 2019 Jul-Aug;67(4):1043-1047

Department of Internal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

Background: Neurological manifestations are an important cause of morbidity in antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV). It is not clear whether or not they are indicative of a severe disease course with multiple organ involvement and shortened survival.

Aims And Objectives: To characterize the neurological manifestations of AAV and analyze their relationship with other organ system and cumulative survival. Read More

Praxis (Bern 1994) 2019 Sep;108(11):733-736

Kantonsspital Münsterlingen, Abteilung Pneumologie, Münsterlingen.

Pulmonary Infiltrate - Not Always Due to Bacterial Infection A 65-year-old female patient with a history of controlled asthma was diagnosed with community-acquired pneumonia (CAP). After two weeks of treatment on co-amoxicillin, she failed to respond and was referred for further investigations. Clinical symptoms and laboratory results were suggestive for eosinophilic granulomatosis with polyangiitis (EGPA). Read More

Adv Ther 2019 10 31;36(10):2558-2566. Epub 2019 Aug 31.

Department of Internal Medicine, Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium.

This article, coauthored by a patient with eosinophilic granulomatosis with polyangiitis (EGPA) initially presenting as severe eosinophilic asthma and his physician-specialist, discusses the use and management of oral corticosteroid (OCS) treatment. It also considers the importance of early diagnosis of a rare disease and patient education. The patient describes his journey from progressive worsening of asthma and eventual diagnosis of EGPA to long-term OCS treatment and then participation in a clinical trial for this rare disease, involving the introduction of targeted biologic therapy with OCS tapering. Read More

Eur Heart J Case Rep 2019 Jun;3(2)

Department of Cardiology, Lister Hospital, East and North Hertfordshire NHS Trust, Stevenage, UK.

Background: Myocardial infarction is most commonly caused by thrombosis occurring on a background of coronary atherosclerosis, resulting in reduced coronary flow. Less often, myocardial infarction can occur in the absence of coronary disease. The pathomechanism of myocardial infarction in such patients is heterogeneous and more challenging to diagnose and treat. Read More

Arerugi 2019 ;68(7):857-868

Department of Allergy, Hiratsuka City Hospital.

Background And Aims: It is difficult to evaluate neurological signs of multiple mononeuritis (MM) in patients with eosinophilic granulomatosis with polyangiitis (EGPA). We created a new questionnaire about motor and sensory disturbances in EGPA and investigated whether the questionnaire would be a useful tool in the management of MM in EGPA patients.

Methods: We classified 40 EGPA patients attending Hiratsuka City Hospital into two groups, namely 30 who were treated with intravenous immunoglobulin (IVIG) and 10 who achieved remission by conventional treatment without IVIG. Read More

Intern Med 2019 Dec 6;58(24):3583-3587. Epub 2019 Aug 6.

Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan.

We herein report the first pediatric case (a 13-year-old girl) of relapsing eosinophilic granulomatosis with polyangiitis (EGPA) successfully treated with mepolizumab (anti-interleukin-5). She was classified as having EGPA based on the presence of asthma, eosinophilia, pulmonary infiltrates, and extravascular eosinophil infiltration confirmed by a biopsy. She achieved remission after initial oral prednisolone (PSL) therapy, but EGPA relapsed during PSL tapering. Read More

Rheumatol Int 2020 Feb 5;40(2):303-311. Epub 2019 Aug 5.

Service of Rheumatology, Hospital Universitario de Salamanca, Salamanca, Spain.

The different sets of criteria for diagnosis or classification of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) lead to numerous overlapping and reclassified diagnoses in clinical practice. We designed this study to assess the difficulties in classifying patients with AAV. As a secondary objective, different variables were tested to predict prognosis. Read More

Am J Kidney Dis 2020 01 26;75(1):124-137. Epub 2019 Jul 26.

Division of Nephrology, University of Washington, Seattle, WA. Electronic address:

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of disorders characterized by inflammation and destruction of small- and medium-sized blood vessels and the presence of circulating ANCA. Clinical disease phenotypes include granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, and renal-limited vasculitis. Serologic classification of AAV into proteinase 3-ANCA disease and myeloperoxidase-ANCA disease correlates with a number of disease characteristics. Read More

J Assoc Physicians India 2018 Jul;66(7):13-17

Resident in Medicine,Lokmanya Tilak Municipal Medical College, Mumbai, Maharashtra.

Introduction: Pulmonary Renal Syndrome (PRS), is characterized by diffuse alveolar haemorrhage (DAH) and glomerulonephritis (GN), occurring simultaneously. It has high mortality and dialysis dependence at one year, if not timely diagnosed and aggressively treated.

Objectives: To study etiology and short term outcome of PRS in India. Read More

Clin Rheumatol 2019 Dec 17;38(12):3493-3499. Epub 2019 Jul 17.

Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan.

To investigate the relevance of RF in patients with EGPA, we reviewed consecutive patients who were newly diagnosed with EGPA from August 1998 to February 2019 in Keio University Hospital with RF titer at diagnosis available. We divided the patients according to the median level of RF titer of 75 IU/mL and compared clinical features between the two groups. Among 16 patients identified, 8 patients were in the RF high group and the other 8 patients were in the RF low group. Read More

Arerugi 2019 ;68(6):696-700

Department of Pediatrics, University Hospital, Kyoto Prefectural University of Medicine.

Atopic dermatitis and bronchial asthma are common diseases in children. We report the development of eosinophilic polyangiitis granulomatosis (EGPA) in a young girl being treated for both atopic dermatitis, diagnosed at 1 year of age, and bronchial asthma, diagnosed at 4 years of age. Her eruption did not result in lichenification and was not fully responsive to corticosteroid ointment. Read More

BMJ Case Rep 2019 Jul 10;12(7). Epub 2019 Jul 10.

Rheumatology, Khoo Teck Puat Hospital, Singapore.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystemic small-vessel vasculitic disease that can present with positive MPO-P-ANCA (myeloperoxidase-perinuclear-anti-neutrophil cytoplasmic antibody). It is a rare condition that is difficult to diagnose. We present the case of a 64-year-old man with late-onset adult asthma and treated nasopharyngeal carcinoma who initially presented to us with proximal myopathy. Read More

Case Rep Med 2019 30;2019:4648720. Epub 2019 May 30.

Department of Pathology, School of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Necrotizing sarcoid granulomatosis (NSG) is a rare granulomatous pneumonitis which is composed of a background of sarcoidosis-like granulomas, granulomatous vasculitis, and variable amount of necrosis. We reported a case of a 38-year-old nonsmoking woman presented with left-sided chest pain and dyspnea for three days. Chest CT scan exhibited collapse consolidation of the left lower lobe with the presence of two separated small-sized cystic lesions within the collapsed segment. Read More

Allergol Int 2019 Oct 29;68(4):430-436. Epub 2019 Jun 29.

Department of Allergy and Clinical Immunology, Chiba University Hospital, Chiba, Japan.

Eosinophilic granulomatosis with polyangiitis (EGPA) (formerly Churg-Strauss syndrome) is a rare form of anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis characterized by eosinophil-rich granulomatous inflammation and small to medium-size vessel vasculitis associated with bronchial asthma and eosinophilia. Its rarity and unique features such as eosinophilic inflammation have delayed progress of research regarding EGPA for several years, compared to other forms of ANCA-associated vasculitis. However, recently, attention to EGPA as a research subject has been gradually increasing. Read More

Pediatr Rheumatol Online J 2019 Jun 26;17(1):31. Epub 2019 Jun 26.

Department of Pediatrics, Division of Rheumatology, Hospital for Sick Children and University of Toronto, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) refers to a complex group of systemic vasculitides that are characterized by primary small-to-medium sized blood vessel inflammation with the presence of autoantibodies known as ANCA. AAV diseases include Granulomatosis with Polyangiitis (GPA), Eosinophilic Granulomatosis with Polyangiitis (EGPA), and Microscopic Polyangiitis (MPA). AAVs are challenging conditions associated with high cumulative disease and treatment related morbidity and mortality. Read More

BMJ Case Rep 2019 Jun 18;12(6). Epub 2019 Jun 18.

Department of Medicine, Lagos State University Teaching Hospital, Lagos, Nigeria.

Eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg-Strauss Syndrome, is a systemic autoimmune disease that is usually associated with asthma and eosinophilia. It is a rare condition associated with antineutrophil cytoplasmic antibodies (ANCA). We report a case of a 52-year-old Nigerian woman who presented with bilateral leg swelling with multiple ulcers, background history of allergic rhinitis and chronic sinusitis since adolescence. Read More

Zhonghua Yi Xue Za Zhi 2019 Apr;99(16):1216-1220

Department of Respiratory and Critical Care Medicine, China-Japan Friendship Hospital, Beijing 100029, China (Zhang Mengyuan is working in the Department of Respiratory, Liaocheng People's Hospital, Shandong Province, Liaocheng 252000, China).

To improve the understanding of Eosinophilic granulomatosis with polyangiitis (EGPA). A total of 30 EGPA patients from the China-Japan Friendship Hospital between August 2005 and April 2017 were included, and a systematic retrospective study about clinical manifestations, laboratory findings, imaging features, pathology and so on was conducted. There were 9 males and 21 females. Read More

Rheumatology (Oxford) 2019 12;58(12):2107-2116

National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, APHP, Université Paris Descartes, Paris, France.

Objective: In a previous controlled trial, 1-year adjunction of AZA to glucocorticoids (GC) for patients with non-severe, newly diagnosed eosinophilic granulomatosis with polyangiitis (EGPA) failed to lower remission failure, vasculitis relapse and isolated asthma/rhinosinus exacerbation rates, or cumulative GC use at month (M) 24. The aim of this study was to analyse longer-term outcomes to determine whether subsequent vasculitis relapse or isolated asthma/rhinosinus exacerbation (IARE) rates differed.

Methods: After M24, patients were followed prospectively, being treated based on physicians' best judgment. Read More

Wiad Lek 2019 ;72(4):723-726

Ukrainian Medical Stomatological Academy, Poltava, Ukraine.

Objective: Introduction: The article describes the clinical case devoted to the features of the clinical course of Churg-Strauss syndrome (eosinophilic granulomatous with systemic necrotizing vasculitis). Churg-Strauss syndrome is a disease that is rarely diagnosed because of presenting by high-variability clinical syndromes, that causes difficulties in diagnostic process, especially on the first visit of patient to the general practioner. The aim of the article is to show the clinical course feautures of the Churg-Strauss syndrome in the time of disease progression, as well as pecullarities and possibilities of in-time diagnosis of this disease. Read More

Case Rep Dermatol 2019 Jan-Apr;11(1):28-35. Epub 2019 Feb 13.

Clinic of Dermatology and Venereology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystemic vasculitis which was previously called Churg-Strauss syndrome or allergic granulomatosis. It has an unknown pathogenesis, possibly autoimmune in nature. As it has a low incidence, there is only scant published literature. Read More

Arch Iran Med 2019 03 1;22(3):161-163. Epub 2019 Mar 1.

Department of Pediatrics, Dr. Sami Ulus Maternity and Children's Health and Diseases Training and Research Hospital, Ankara, Turkey.

The aim of the presentation of this case is to discuss whether there is an association with eosinophilic granulomatosis with polyangiitis (EGPA) and the use of montelukast, and clarithromycin and to discuss a successful treatment course. A 4-year-old girl with a preceding history of asthma attacks and increased eosinophil counts was admitted. She had been using clarithromycin for five days and montelucast for a month. Read More

Eur Heart J Case Rep 2018 Sep 21;2(3):yty075. Epub 2018 Sep 21.

Department of Cardiology, King's College Hospital, Denmark Hill, London, UK.

Background: Eosinophilic granulomatosis with polyangiitis (EGPA, formerly known as Churg-Strauss syndrome) is a rare autoimmune condition characterized by inflammation of small- and medium-sized blood vessels, which usually presents with systemic vasculitis preceded by airway allergic hypersensitivity.

Case Summary: Here, we report a highly unusual case of acute ST-elevation myocardial infarction in a young and fit man with no cardiovascular risk factors. His emergency coronary angiography revealed disproportionately severe widespread coronary artery disease. Read More

Clin Rheumatol 2019 Sep 23;38(9):2553-2563. Epub 2019 Apr 23.

2nd Department of Internal Medicine, Faculty of Medicine, Jagiellonian University Medical College, ul. Skawińska 8, 31-066, Kraków, Poland.

Objective: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare small to medium-size vessel systemic diseases. As their clinical picture, organ involvement, and factors influencing outcome may differ between countries and geographical areas, we decided to describe a large cohort of Polish AAV patients coming from several referral centers-members of the Scientific Consortium of the Polish Vasculitis Registry (POLVAS).

Methods: We conducted a systematic multicenter retrospective study of adult patients diagnosed with AAV between Jan 1990 and Dec 2016 to analyze their clinical picture, organ involvement, and factors influencing outcome. Read More

Nephrol Ther 2019 04;15 Suppl 1:S7-S12

Service de médecine interne, centre de références des maladies auto-immunes et systémiques rares, hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), 24, rue du Faubourg-Saint-Jacques, 75014 Paris, France; Faculté de médecine, université Paris Descartes, 24, rue du Faubourg-Saint-Jacques, 75014 Paris, France. Electronic address:

Antineutrophil cytoplasm antibodies (ANCA)-associated vasculitides are necrotizing vasculitis affecting small blood vessels and include granulomatosis with polyangiitis (formerly Wegener's), microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome). Diagnosis of ANCA-associated vasculitis is based on the clinical presentation, ANCA positivity directed against proteinase 3 or myeloperoxidase, and when possible on histological evidence of vasculitis. Evolution of ANCA-associated vasculitides without treatment leads constantly to death. Read More