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Eur Heart J Case Rep 2019 Dec 26;3(4):1-6. Epub 2019 Oct 26.

Cardiology Department, Royal Free Hospital, Pond Street, London NW3 2QG, UK.

Background: Acute ST elevation myocardial infarction (STEMI) is a medical emergency and is most commonly due to atherosclerotic plaque rupture and occlusion of coronary vessels. This case demonstrates that eosinophilic granulomatosis with polyangiitis (EGPA) myocarditis can mimic acute STEMI.

Case Summary: A 44-year-old woman presented with acute chest pain, shortness of breath, and collapse with ST elevation on electrocardiography. Read More

Neuroophthalmology 2021 25;45(2):109-116. Epub 2020 Jun 25.

Birmingham Neuro-Ophthalmology, Queen Elizabeth Hospital, Birmingham, UK.

Eosinophilic granulomatosis with polyangiitis (eGPA) is a rare vasculitis of small-medium sized vessels that can cause both anterior and posterior ischaemic optic neuropathies. Herein, the authors present a rare case of eGPA presenting initially as an acute unilateral anterior ischaemic optic neuropathy from short posterior ciliary artery vasculitis. The diagnosis presented a challenge as clinical and histopathological evidence suggested allergic rhinosinusitis, and no invasive fungal sinusitis was found. Read More

Pol Arch Intern Med 2021 Jun 8. Epub 2021 Jun 8.

Introduction: Granulomatosis with polyangiitis (GPA) patients show increased tendency to thromboembolic phenomena in the active phase of their disease.

Objectives: To evaluate thrombin generation potential and fibrinolytic plasma activity in GPA patients, both in the active phase and in GPA remission.

Patients And Methods: Thirty-eight GPA patients were studied; 18 with active GPA and 20 in remission. Read More

World J Gastroenterol 2021 May;27(20):2657-2663

Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Sendai 9808574, Japan.

Background: Although cyclophosphamide (CPA) is the key drug for the treatment of autoimmune diseases including vasculitides, it has some well-known adverse effects, such as myelosuppression, hemorrhagic cystitis, infertility, and infection. However, CPA-associated severe enteritis is a rare adverse effect, and only one case with a lethal clinical course has been reported. Therefore, the appropriate management of patients with CPA-associated severe enteritis is unclear. Read More

Intern Med 2021 Jun 5. Epub 2021 Jun 5.

Department of Internal Medicine, Teikyo University School of Medicine, Japan.

A 39-year-old man presented with peripheral eosinophilia, pulmonary eosinophilic infiltrate, and renal failure due to acute tubulointerstitial nephritis (TIN). He had experienced childhood asthma and was negative for anti-neutrophil cytoplasmic antibody (ANCA). He was tentatively diagnosed with ANCA-negative eosinophilic granulomatous polyangiitis (EGPA) or idiopathic hypereosinophilic syndrome (HES). Read More

Intern Med 2021 Jun 5. Epub 2021 Jun 5.

Department of Respiratory Medicine, Allergy and Clinical Immunology, Nagoya City University Graduate School of Medical Sciences, Japan.

Eosinophilic granulomatosis with polyangiitis (EGPA) is an anti-neutrophilic cytoplasm antibody (ANCA)-associated vasculitis characterized by asthma and eosinophilia. Although EGPA involves multiple organs, ocular involvement is infrequent and often carries a poor visual prognosis. We herein report a rare case of EGPA presenting with central retinal artery occlusion (CRAO) in which visual loss developed during treatment with anti-interleukin (IL)-5 receptor monoclonal antibody, and improvement in visual outcomes was attained after treatment combining high-dose oral corticosteroids, cyclophosphamide and an anticoagulant. Read More

Rheumatol Int 2021 Jun 6. Epub 2021 Jun 6.

Division of Pulmonary and Critical Care Medicine, Mayo Clinic Arizona, Phoenix, AZ, USA.

A 77-year-old man with past medical history of granulomatosis with polyangiitis (GPA) on rituximab and prednisone, presented to the hospital with worsening cough and shortness of breath. He had tested positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection by nasal swab polymerase chain reaction (PCR) while asymptomatic, 6 weeks earlier. He started with cough and shortness of breath 2 weeks after his initial positive test. Read More

Pediatr Rheumatol Online J 2021 Jun 5;19(1):81. Epub 2021 Jun 5.

Section of Pediatric Rheumatology, University of Chicago Medical Center, Chicago, IL, USA.

Background: Granulomatosis with polyangiitis (GPA) is an anti-neutrophilic cytoplasmic antibody-associated vasculitis affecting small to medium-sized vessels and involves most commonly the kidneys and the respiratory tract. Skin involvement can be seen in up to 50% of children with GPA and is the initial presenting symptom in 7.7%. Read More

Surv Ophthalmol 2021 May 31. Epub 2021 May 31.

Department of Ophthalmology, Royal Adelaide Hospital, Adelaide, South Australia, Australia.

Dacryoadenitis is an inflammation of the lacrimal gland that may have various etiologies with similar presentations. Despite more recent elucidation of specific causes, the management has remained largely unchanged. Hence, the condition remains under biopsied with the rationale that empirical treatment with corticosteroids is effective for many of the causes. Read More

Clin Exp Allergy 2021 Jun 3. Epub 2021 Jun 3.

Department of Respiratory Sciences, College of Life Sciences, Institute for Lung Health, NIHR Leicester Biomedical Research Centre (Respiratory theme), University of Leicester and Respiratory and Allergy Services, University Hospitals of Leicester NHS Trust, Leicester, UK.

Background: A peripheral blood eosinophilia of greater than 1.0 × 10 /L is relatively unusual and offers a clue to the underlying diagnosis. In 2003, we established a specialist service to diagnose unexplained eosinophilia. Read More

Eur J Intern Med 2021 May 29. Epub 2021 May 29.

Medical University of Warsaw, Stepinska str 19/25, Warsaw, Poland; Central Clinical Hospital of Ministry of MSWiA, Warsaw, Poland.

Introduction: Granulomatosis with polyangiitis (GPA) is one of antineutrophil cytoplasmic autoantibody (ANCA) - associated systemic vasculitis and is characterised by inflammation of blood vessels. Systemic vasculitis exhibits an enhanced cardiovascular morbidity and cardiovascular disease (CVD) has become a leading cause of death in this group of patients.

Objectives: The aim of the present study was to assess the prevalence of clinical manifestation of atherosclerosis and its relation with classic risk factors for atherosclerosis, echocardiographic parameters and laboratory findings in GPA patients. Read More

Clin Pract 2021 May 14;11(2):293-302. Epub 2021 May 14.

Department of Nephrology, Rigshospitalet, University of Copenhagen, 2100 Copenhagen, Denmark.

Diagnosis of anomalous intrathoracic lesions may be challenging and require a multidisciplinary approach. We present a case of granulomatosis with polyangiitis (GPA) clinically and radiologically mimicking metastatic lung cancer with a bilateral pulmonary mass, mediastinal and cervical lymph node involvement, and pleural effusion. Surgical biopsy of the thoracic lesion revealed necrotic granulomatous inflammation, and the final diagnosis was subsequently confirmed by kidney biopsy and biochemical parameters. Read More

Cureus 2021 May 24;13(5):e15206. Epub 2021 May 24.

Pathology, Giresun University Faculty of Medicine, Giresun, TUR.

Since idiopathic granulomatous lobular mastitis, also known as idiopathic granulomatous mastitis or granulomatous lobulitis, was first described by Kessler and Wolloch in 1972, no consensus on the ideal and definitive treatment for this phenomenon has been reached thus far. Idiopathic granulomatous lobular mastitis mostly frequently observed in women of childbearing age within a few years of pregnancy with a higher incidence in patients of Hispanic, Native American, Middle Eastern, and African descent. This entity, , is a rare, benign, chronic inflammatory breast condition of unknown aetiology mimicking two common breast disorders. Read More

Cureus 2021 Apr 23;13(4):e14649. Epub 2021 Apr 23.

Critical Care Medicine, University of Pittsburgh Medical Center Mercy, Pittsburgh, USA.

Eosinophilic granulomatosis with polyangiitis (EGPA) is an exceptionally rare systemic necrotizing vasculitis. The disease is clinically characterized by asthma with concomitant blood and tissue eosinophilia, often progressing to eosinophilic vasculitis. From the onset of asthma, there is usually a three to nine year delay of EGPA diagnosis. Read More

Clin J Am Soc Nephrol 2021 May 26. Epub 2021 May 26.

G Montini, Department of Clinical Sciences and Community Health, University of Milan, Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico , Milan, Italy.

Background And Objectives: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is extremely rare in children. We report the clinico-pathological features, long-term outcomes, and prognostic factors of a large paediatric cohort of patients with ANCA-associated kidney vasculitis.

Design, Setting, Participants, And Measurements: This retrospective study included 85 consecutive patients with kidney biopsy-proven ANCA-associated vasculitis followed at tertiary referral centres in Italy and Canada. Read More

Rheumatol Int 2021 May 25. Epub 2021 May 25.

Division of Rheumatology, Department of Internal Medicine, Ankara City Hospital, Ankara, 06100, Turkey.

Coronavirus disease 2019 (COVID-19) and eosinophilic granulomatosis with polyangiitis (EGPA) share similarities in clinical, imaging findings and may present with respiratory distress. Differentiating a new-onset EGPA from COVID-19 during the current pandemic is a diagnostic challenge, particularly if other EGPA symptoms are overlooked. Here in this study we reviewed the literature regarding EGPA patients with COVID-19 and patients who diagnosed with EGPA or suffered an EGPA flare mimicking COVID-19. Read More

ACR Open Rheumatol 2021 May 25. Epub 2021 May 25.

Vasculitis Clinic, Mount Sinai Hospital, University of Toronto, Toronto, Ontario, Canada.

Objective: To describe clinical manifestations and outcomes in patients with eosinophilic granulomatosis with polyangiitis (EGPA) in North America.

Methods: Analysis of patients aged 18 years or older who fulfilled the 1990 American College of Rheumatology Classification Criteria for EGPA enrolled in the Vasculitis Clinical Research Consortium from 2003 to 2019. Main clinical characteristics, treatments, outcomes, and accumulated damage were studied. Read More

Adv Exp Med Biol 2021 May 26. Epub 2021 May 26.

Humanitas Clinical and Research Center - IRCCS, Milan, Italy.

Eosinophils are a subset of differentiated granulocytes which circulate in peripheral blood and home in several body tissues. Along with their traditional relevance in helminth immunity and allergy, eosinophils have been progressively attributed important roles in a number of homeostatic and pathologic situations. This review aims at summarizing available evidence about eosinophils functions in homeostasis, infections, allergic and autoimmune disorders, and solid and hematological cancers. Read More

Yonsei Med J 2021 Jun;62(6):494-502

Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.

Purpose: The present study compared the efficacy of mycophenolate mofetil (MMF) with that of azathioprine (AZA) in Korean patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA).

Materials And Methods: The medical records of 69 patients with MPA and GPA who received cyclophosphamide and subsequently received AZA or MMF for remission maintenance therapy were reviewed. All-cause mortality, relapse, end-stage renal disease (ESRD), cerebrovascular accident, and cardiovascular disease were evaluated as poor outcomes. Read More

Eur Heart J 2021 May 21. Epub 2021 May 21.

Hospital Porz am Rhein gGmbH, Clinic of Rheumatology, Urbacher Weg 19, 51149 Köln, Germany.

J Cutan Pathol 2021 May 21. Epub 2021 May 21.

Pathology Service, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts, USA.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, but severe systemic vasculitis that can affect skin and other organ systems. Diagnostic criteria have evolved, and many attempts have been made to classify the vasculitides based on clinical and/or histopathologic features, with an aim to develop standardized criteria. According to the EGPA Consensus Task Force recommendations, EGPA is a syndrome of asthma, eosinophilia, pulmonary infiltrates, and extrapulmonary vasculitis (such as cutaneous involvement with purpura). Read More

Clin Exp Rheumatol 2021 Mar-Apr;39 Suppl 129(2):107-113. Epub 2021 May 19.

Department of rheumatology, IRCCS Policlinico S. Matteo Fondazione, University of Pavia, Italy.

Objectives: It has been suggested that anti-neutrophil cytoplasmic antibody (ANCA) specificity, rather than clinical diagnosis influences the phenotype and course of ANCA-associated vasculitis (AAV). However, preliminary evidence suggests that further combined levels of categorisation might be of clinical relevance. The aim of this study was to investigate differences in clinical presentation at disease onset and outcomes based on clinical diagnosis and ANCA specificity. Read More

Brain Nerve 2021 May;73(5):503-510

Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University.

Granulomatosis with polyangiitis (GPA) is designated as an intractable disease by the Ministry of Health, Labor and Welfare, Japan, and is classified as an antineutrophil cytoplasmic antibody (ANCA)-related vasculitis syndrome. It is associated with upper respiratory tract symptoms (E; ear and nose), pulmonary symptoms (L; lung), renal symptoms (K; kidney), and systemic vasculitis symptoms, and often involves the central/peripheral nervous system. Patients with GPA can be easily diagnosed as they often show positive serum C (Proteinase 3)-ANCA findings. Read More

Pol Arch Intern Med 2021 May 18. Epub 2021 May 18.

Introduction: Granulomatosis with polyangiitis (GPA) as an autoimmune disease leads to necrotizing changes in the affected tissues. Computed tomography (CT) of paranasal sinuses reveals multiple changes in GPA: sinus opacification, bone/cartilaginous destruction and neoosteogenesis.

Objectives: To describe and compare CT changes in GPA with chronic rhinosinusitis (CRS) patients. Read More

Rheumatology (Oxford) 2021 May 17. Epub 2021 May 17.

Division of Rheumatology, Department of Medicine, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA.

Front Immunol 2021 28;12:670312. Epub 2021 Apr 28.

Department of Rheumatology Immunology & Allergy, The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, China.

Activated phosphoinositide 3-kinase δ syndrome (APDS) is an autosomal dominant primary immunodeficiency caused by gain-of-function (GOF) mutations in or genes. The phenotypes of APDS are highly variable, ranging from asymptomatic adults to profound immunodeficiency causing early death in childhood. Herein, we reported two pediatric patients with APDS presented with recurrent lung infections, sinusitis, hematuria, and positive anti-neutrophil cytoplasmic antibody (ANCA), previously diagnosed as granulomatosis with polyangiitis (GPA). Read More

Can J Neurol Sci 2021 May 14:1-6. Epub 2021 May 14.

Front Mol Biosci 2021 27;8:653461. Epub 2021 Apr 27.

Department of Pathophysiology, School of Basic Medical Sciences, Guizhou Medical University, Guiyang, China.

Eosinophilic granulomatosis with polyangiitis (EGPA) prognosis is generally favorable and is treated with combined corticosteroids/immunosuppressor(s) therapy. However, disease flares increase the number of clinical visits. Therefore, discovering new serum biomarkers for early identification of active EGPA is crucial. Read More

Eur J Case Rep Intern Med 2021 22;8(4):002448. Epub 2021 Apr 22.

Nephrology Department, Centro Hospitalar Universitário do Porto, Portugal.

Granulomatosis with polyangiitis (GPA) is the most common antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We describe the case of a 38-year-old woman with relapsing GPA who presented with intracranial hypertension, followed by the appearance of cavitated lung nodules despite treatment with azathioprine. Clinical improvement and ANCA titre reduction were observed after rituximab treatment. Read More

Neurology 2021 May 12. Epub 2021 May 12.

. Department of Neurology, Yale University School of Medicine, New Haven, CT, USA.