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Cureus 2022 Apr 21;14(4):e24342. Epub 2022 Apr 21.

Department of Surgery, University Hospital Galway, Galway, IRL.

Eosinophilic granulomatosis with polyangiitis (EGPA) or Churg-Strauss syndrome (CSS) is a rare, autoimmune vasculitis usually affecting small and medium-sized blood vessels in its later phases. It is a diffuse, systemic, multisystem disease that is reported to present with gastrointestinal manifestations but very rarely as an acute abdomen secondary to eosinophilic peritonitis. A 28-year-old relatively healthy male with a pre-existing diagnosis of inactive pulmonary sarcoidosis presented to the emergency department with an acute abdomen. Read More

Brain Nerve 2022 May;74(5):531-536

Toyota Kosei Hospital.

Eosinophilic granulomatosis with polyangiitis (EGPA), an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, is a systemic vasculitis syndrome involving inflammatory damage of predominantly small vessels. Initial treatment is extremely important because the peripheral nervous system is a major target organ that depends on long-term clinical outcomes. Moreover, detailed neurological observations are necessary during the remission period. Read More

Brain Nerve 2022 May;74(5):525-530

Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine.

Recently, given the availability of mepolizumab as a novel treatment for eosinophilic polyangiitis granulomatosis (EGPA), several studies on remission-induction/maintenance therapies are in progress. However, there is little evidence regarding the treatment of EGPA neuropathy. In this article, we clarify the characteristics of steroid-resistant EGPA neuropathy by presenting actual cases and explaining the selection of remission-induction/maintenance therapies based on the characteristics. Read More

Ther Umsch 2022 Jun;79(5):229-237

Klinik für Rheumatologie, Universitätsspital Basel.

ANCA-Associated Vasculitides The according to their immunoserological markers (anti-neutrophil cytoplasmic antibodies - ANCA) named ANCA-associated vasculitides (AAV) are classified following the Chapel Hill nomenclature (2012). Microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic granulomatosis with polyangiitis (EGPA) are categorized according to clinical, histological, and imaging findings. GPA and EGPA mainly differ from MPA in the presence of granulomatous inflammation within the airways. Read More

Clin Kidney J 2022 May 9;15(5):855-864. Epub 2021 Dec 9.

Translational and Clinical Research Institute, Faculty of Medical Sciences, Newcastle University, Central Parkway, Newcastle upon Tyne, UK.

Acute kidney injury (AKI) is a common medical problem with a multitude of aetiologies. Prompt diagnosis and management is key in the prevention of complications. Cutaneous signs can often give diagnostic clues of underlying systemic diseases causing AKI. Read More

BMJ Case Rep 2022 Apr 29;15(4). Epub 2022 Apr 29.

Internal Medicine, Texas Health Presbyterian Hospital Dallas, Dallas, Texas, USA.

A middle age man with a history of diabetes mellitus type 2, hypertension, migraine and eosinophilic granulomatosis with polyangiitis (EGPA) with polyneuropathy in remission presented with paresthesia and motor weakness soon after receiving the Pfizer-BioNTech COVID-19 messanger RNA (mRNA) vaccine. The patient had polyneuropathy 10 years ago secondary to EGPA, which had resolved. EGPA was diagnosed on the basis of typical symptoms and positive sural nerve biopsy. Read More

Clin Exp Rheumatol 2022 May 27;40(4):720-726. Epub 2022 Apr 27.

Centre for Intensive Care and Perioperative Medicine, Jagiellonian University Medical College, Kraków, Poland.

Objectives: The study aimed to characterise the Polish population of (ANCA)-associated vasculitides (AAV) with respiratory involvement (RI), in comparison to the subgroup without lung manifestations and the other cohorts.

Methods: Retrospective analysis of the Polish population of AAV with RI was conducted, based on data from the POLVAS registry. Standard descriptive statistics, χ2 test, and Mann-Whitney U test were used to perform comparisons. Read More

Autoimmun Rev 2022 Jun 19;21(6):103100. Epub 2022 Apr 19.

ARUP Laboratories, Salt Lake City, UT, United States of America; Department of Pathology, University of Utah, Salt Lake City, UT, United States of America. Electronic address:

Objective: To determine the impact of myeloperoxidase (MPO) and proteinase 3 (PR3) antigen-specific immunoassays in the stratification of patients at-risk for anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV) at diagnosis.

Methods: A Medline search was conducted to identify diagnostic accuracy studies using PR3-ANCA or MPO-ANCA for the evaluation of granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). Studies estimates were pooled using the bivariate method. Read More

BMJ Case Rep 2022 Apr 21;15(4). Epub 2022 Apr 21.

Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, Minnesota, USA

We present a case of a woman who had progressive shortness of breath and wheezing with a mild restrictive pulmonary function pattern. She was initially diagnosed with eosinophilic granulomatosis with polyangiitis on the basis of peripheral eosinophilia, bronchoalveolar lavage eosinophilia (47%) and surgical lung biopsy findings. Six months following her diagnosis, the patient returned because of persistent symptoms, and a second review of the lung biopsy revealed thrombotic lesions in the pulmonary vessels with polarisable foreign body materials, associated giant cell reactions and numerous eosinophil infiltrates, consistent with intravenous drug abuse. Read More

Curr Opin Rheumatol 2022 05 18;34(3):158-164. Epub 2022 Apr 18.

University of Michigan, Department of Internal Medicine, Division of Rheumatology, Ann Arbor, Michigan, USA.

Purpose Of Review: In recent years, therapeutic advances in eosinophilic granulomatosis with polyangiitis (EGPA) have changed our treatment paradigm. This review will summarize and discuss updates in management of EGPA, with a particular focus on biologic therapies.

Recent Findings: The anti-interleukin (IL)-5 agent mepolizumab (the first FDA-approved drug specifically for EGPA) is effective in induction and maintenance of remission particularly in patients with predominantly asthma and allergic manifestations, though efficacy in ANCA-positive, vasculitic disease is unclear; additional anti-IL-5 agents are under study. Read More

Neuropathology 2022 Jun 11;42(3):239-244. Epub 2022 Apr 11.

Department of Pathology, San-in Rosai Hospital, Yonago, Japan.

The definite diagnosis of central nervous system vasculitis requires pathological verification by biopsy or surgical resection of the lesion, which may not always be feasible. A 74-year-old woman with a history of allergic rhinitis, but not asthma, presented with slowly progressive left hemiparesis. Magnetic resonance imaging of the head revealed a heterogeneously enhancing mass involving the right internal capsule and corona radiata. Read More

Tuberk Toraks 2022 Mar;70(1):93-101

Division of Immunology and Allergy, Department of Chest Diseases, Ankara University Faculty of Medicine, Ankara, Turkey.

Eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome) is a rare systemic necrotizing granulomatous vasculitis in the spectrum of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Nevertheless, EGPA has specific clinical, biological and histological properties different from other AAVs [microscopic polyangiitis (MPA) and granulomatous polyangiitis (GPA)]. Recently, thanks to the studies conducted to understand the pathophysiology of EGPA, unlike neutrophils in other AAVs, the main cells involved in EGPA have been observed to be eosinophils. Read More

Curr Rheumatol Rev 2022 Mar 29. Epub 2022 Mar 29.

Professor and Chairman, Department of Internal Medicine, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh.

Background: Eosinophilic Granulomatosis with Polyangiitis (EGPA), also known as Churg Strauss Syndrome, is an uncommon vasculitis associated with antineutrophil cytoplasmic antibody (ANCA). The hallmarks of the disease are asthma, eosinophilia and systemic vasculitis with varying degrees of neurological, cutaneous, cardiac, gastrointestinal and renal involvement. Diagnosisis often difficult since the symptoms are diverse,and a number of differentials need to be excluded. Read More

Tohoku J Exp Med 2022 03;256(3):241-247

Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine.

Hypertrophic pachymeningitis (HP) presents with thickening of the dura mater in the cerebrum and spine, and its symptoms vary depending on the affected location. The association of HP with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis has been recognized, and most cases are complicated by granulomatosis with polyangiitis. We report the case of a 47-year-old man who presented with HP upon relapse of eosinophilic granulomatosis with polyangiitis (EGPA), with literature review. Read More

RMD Open 2022 03;8(1)

First Department of Internal Medicine, University of Occupational and Environmental Health, Japan, Kitakyushu, Fukuoka, Japan

Objective: To study the pathophysiological differences of EGPA and IgG-related disease (RD) by clarifying their clinical, pathological and immunological features.

Methods: Clinical and pathological findings were compared in patients with EGPA and IgG-RD. Peripheral blood mononuclear cells were used for comprehensive flow cytometric analysis. Read More

Br J Hosp Med (Lond) 2022 Feb 23;83(2):1-10. Epub 2022 Feb 23.

The management of anti-neutrophil cytoplasmic antibody-associated vasculitis has substantially improved in the last decade. For the induction of remission, rituximab is increasingly used in place of cyclophosphamide, particularly for patients with proteinase 3 (PR3)-associated vasculitis or relapsing disease, and those wishing to preserve their fertility. A lower dose regimen of glucocorticoids, with a more rapidly tapering schedule, is preferable and is as effective and safer than the standard-dose regimen. Read More

Can J Cardiol 2022 May 11;38(5):623-633. Epub 2022 Feb 11.

Vanderbilt University Medical Center, Nashville, Tennessee, USA. Electronic address:

Systemic vasculitis can be a challenge to differentiate from other forms of vasculopathy. Because treatment for systemic vasculitis is disparate from that for other forms of vasculopathy, clinicians should strive for high diagnostic certainty. This review article aims to highlight the clinical, radiographic, and histologic clues to distinguish systemic vasculitis from mimics. Read More

Arthritis Rheumatol 2022 03 2;74(3):386-392. Epub 2022 Feb 2.

University of Pennsylvania, Philadelphia.

Objective: To develop and validate revised classification criteria for eosinophilic granulomatosis with polyangiitis (EGPA).

Methods: Patients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in 5 phases: 1) identification of candidate criteria items using consensus methodology, 2) prospective collection of candidate items present at the time of diagnosis, 3) data-driven reduction of the number of candidate items, 4) expert panel review of cases to define the reference diagnosis, and 5) derivation of a points-based risk score for disease classification in a development set using least absolute shrinkage and selection operator logistic regression, with subsequent validation of performance characteristics in an independent set of cases and comparators. Read More

Rom J Ophthalmol 2021 Oct-Dec;65(4):411-418

"Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania.

Eosinophilic angiocentric fibrosis (EAF) is an infrequent and slowly progressive disease, represented by fibroinflammatory lesions of unknown origin, which mainly involves the sinonasal structures and upper respiratory tract. Occasionally, it can affect the orbit and ocular adnexa causing symptoms such as proptosis, globe displacement and periorbital edema. In very rare cases, ocular manifestation as an orbital inflammatory pseudotumor can be the primary localization of the disease. Read More

Rom J Ophthalmol 2021 Oct-Dec;65(4):386-390

Department of Rheumatology and Internal Medicine, "Sfânta Maria" Clinical Hospital, Bucharest, Romania.

Granulomatosis with polyangiitis (GPA) is a type of small-sized blood vessel vasculitis that predominantly affects the upper airways, lungs and kidneys and associates with the presence of anti-neutrophil cytoplasmic antibodies (ANCA). Nevertheless, any organ of the body can be affected by GPA, including the eye. Occasionally, ocular involvement can be the initial manifestation, thus representing an essential clue for the physician in the early diagnosis of the disease. Read More

Z Rheumatol 2022 May 27;81(4):270-279. Epub 2022 Jan 27.

Klinik für Rheumatologie und klinische Immunologie, Universität zu Lübeck, Ratzeburger Allee 160, 23538, Lübeck, Deutschland.

Small vessel vasculitis is characterized by a necrotizing inflammation of the vessel wall predominantly with involvement of small intraparenchymal arteries, arterioles, capillaries and venules. Medium-sized and occasionally large vessels can also be involved. Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis) are differentiated from immune complex vasculitides based on immunopathological and serological aspects. Read More

Eur Respir Rev 2022 Mar 25;31(163). Epub 2022 Jan 25.

Médecine Interne, Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium.

The functions ascribed to eosinophils have classically been limited to host defence against certain parasitic infections and potentially deleterious effects in the setting of specific diseases that are associated with elevated eosinophil counts in blood and/or tissue. The ability to induce eosinophil depletion either experimentally in animal models or through targeted therapies in humans has extended our understanding of the roles played by eosinophils in health and homeostasis as well as in disease pathogenesis. When associated with human disease aetiology, the eosinophil takes on a pathogenic rather than a protective role. Read More

Z Rheumatol 2022 May 24;81(4):286-299. Epub 2022 Jan 24.

Rheumazentrum Schleswig-Holstein Mitte, Neumünster, Deutschland.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare chronic inflammatory systemic disease that occurs in patients with bronchial asthma and is associated with significant blood and tissue eosinophilia. Another characteristic is vasculitis of small and/or medium-sized vessels, which may be absent in prodromal stages of the disease and is therefore no longer an obligatory part of the disease definition. Antineutrophil cytoplasmic antibodies (ANCA) can be detected in approximately one third of patients. Read More

Neurol Ther 2022 Mar 19;11(1):21-38. Epub 2022 Jan 19.

Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, 466-8550, Japan.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a systemic disorder that frequently affects the peripheral nervous system and consists of three distinct conditions: microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA, previously Wegener's granulomatosis), and eosinophilic granulomatosis with polyangiitis (EGPA, previously Churg-Strauss syndrome). The neuropathic features associated with this condition usually include mononeuritis multiplex, which reflects the locality of lesions. Findings suggestive of vasculitis are usually found in the epineurium and occur diffusely throughout the nerve trunk. Read More

Internist (Berl) 2022 Feb 14;63(2):143-154. Epub 2022 Jan 14.

Zentrum für Interdisziplinäre Klinische Immunologie, Rheumatologie und Autoimmunerkrankungen (INDIRA), Universitätsklinikum Tübingen, Tübingen, Deutschland.

Despite therapy with glucocorticoids (GC) and conventional immunosuppressants, patients with connective tissue diseases and vasculitides often develop functionally relevant and prognostically unfavourable internal organ damage. Based on new pathogenetic insights, biologics and small molecules have recently been studied as targeted therapies for collagen vascular diseases and vasculitides. The B lymphocyte stimulator antagonist belimumab has been used for the treatment of systemic lupus erythematosus (SLE) for several years and has recently also been approved as an add-on therapy for lupus nephritis. Read More

World J Clin Cases 2021 Dec;9(34):10702-10707

Intensive Care Unit, Affiliated Dongyang Hospital of Wenzhou Medical University, Jinhua 322100, Zhejiang Province, China.

Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystem disease characterized by allergic rhinitis, asthma, and a significantly high eosinophil count in the peripheral blood. It mainly involves the arterioles and venules. When the coronary arteries are invaded, it can lead to acute myocardial infarction (AMI), acute heart failure, and other manifestations that often lead to death in the absence of timely treatment. Read More

Autoimmun Rev 2022 Apr 5;21(4):103037. Epub 2022 Jan 5.

Cardiology, Department of Cardiac Thoracic Vascular Sciences and Public Health, University of Padova, Padova, Italy. Electronic address:

Many systemic immune-mediated diseases (SIDs) may involve the heart and present as myocarditis with different histopathological pictures, i.e. lymphocytic, eosinophilic, granulomatous, and clinical features, ranging from a completely asymptomatic patient to life-threatening cardiogenic shock or arrhythmias. Read More

Pol Arch Intern Med 2022 03 5;132(3). Epub 2022 Jan 5.

Department of Internal Medicine, Rheumatology and Clinical Immunology, Medical University of Silesia, Katowice, Poland

Introduction: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is characterized by the presence of proteinase‑3 (PR3) or myeloperoxidase (MPO) ANCA. In over 90% of cases, PR3‑ANCA is associated with granulomatosis with polyangiitis (GPA). However, it is also rarely found in microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). Read More

Int J Cardiol 2022 Mar 31;351:84-88. Epub 2021 Dec 31.

Département de Médecine Interne, Hôpital Bichat, Université de Paris, Assistance Publique Hôpitaux de Paris, Paris, France; INSERM U1149, Paris, France. Electronic address:

Background: Acute myocarditis (AM) may be the heralding manifestation of autoimmune and inflammatory disorders (AIID). We aimed to describe the clinical presentation and outcome of patients with AM revealing AIID.

Methods: All consecutive adult patients with AM admitted in a department of Cardiology (Bichat Hospital, Paris, France) from January 2011 to January 2019 were included. Read More

Medicine (Baltimore) 2021 Dec;100(51):e28305

Rheumatology Division - Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo, Sao Paulo, SP, Brazil.

Abstract: The mortality rate of patients with anti-neutrophil cytoplasm antibody -associated vasculitis (AAV) is higher than the general population. To date, no studies have evaluated the factors associated with unfavorable outcomes in Brazilian patients, who represent a miscegenated population. Our objective was to identify clinical and laboratory features associated with mortality in Brazilian patients with AAV. Read More