9,604 results match your criteria Wegener Granulomatosis


Bilateral Dacryocystitis Resulting from Granulomatosis with Polyangiitis.

Am J Ophthalmol 2022 Jun 22. Epub 2022 Jun 22.

Vanderbilt Eye Institute, Nashville, TN.

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MPO-ANCA-positive conversion and microscopic polyangiitis development in idiopathic interstitial pneumonia: a case report.

CEN Case Rep 2022 Jun 24. Epub 2022 Jun 24.

Division of Nephrology and Endocrinology, Faculty of Medicine, Tohoku Medical and Pharmaceutical University, 1-15-1 Fukumuro, Miyagino-ku, Miyagi, Sendai, 983-8536, Japan.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a systemic autoimmune disease characterized by necrotizing inflammation of the small blood vessels. ANCA-associated vasculitis is subclassified into three variants: granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis (MPA). Myeloperoxidase (MPO) ANCA is a marker antibody for MPA. Read More

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Exacerbation of Eosinophilic Granulomatosis With Polyangiitis After Administering Dupilumab for Severe Asthma and Eosinophilic Rhinosinusitis With Nasal Polyposis.

Cureus 2022 May 22;14(5):e25218. Epub 2022 May 22.

Respiratory Medicine, Japanese Red Cross Society Kyoto Daiichi Hospital, Kyoto, JPN.

Eosinophilic granulomatosis with polyangiitis (EGPA) refers to systemic vasculitis in patients with bronchial asthma and eosinophilic rhinosinusitis. Dupilumab has been approved for the treatment of asthma, eosinophilic rhinosinusitis, and atopic dermatitis. A man in his 50s with a history of asthma and eosinophilic rhinosinusitis with nasal polyposis developed high fever and dyspnea while undergoing dupilumab treatment. Read More

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Recurrent Optic Perineuritis With Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease Complicated With Granulomatous Polyangiitis.

Cureus 2022 May 23;14(5):e25239. Epub 2022 May 23.

Division of Hematology and Rheumatology, Teikyo University Chiba Medical Center, Ichihara, JPN.

Optic perineuritis (OPN) is an intraorbital inflammatory disease that targets the optic nerve sheath, which can cause severe vision loss. OPN has been recently reported to be sometimes caused by myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD). MOGAD is rarely reported to be complicated with other autoimmune diseases. Read More

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Gut Microbiota and Associated Mucosal Immune Response in Eosinophilic Granulomatosis with Polyangiitis (EGPA).

Biomedicines 2022 May 24;10(6). Epub 2022 May 24.

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

Eosinophilic granulomatosis with polyangiitis (EGPA) is an anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. A genome-wide association study showed a correlation between ANCA-negative EGPA and variants of genes encoding proteins with intestinal barrier functions, suggesting that modifications of the mucosal layer and consequent gut dysbiosis might be involved in EGPA pathogenesis. Here, we characterized the gut microbiota (GM) composition and the intestinal immune response in a cohort of EGPA patients. Read More

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Coexistence of Mucormycosis and Granulomatosis With Polyangiitis: A Diagnostic and Therapeutic Challenge.

Cureus 2022 May 18;14(5):e25124. Epub 2022 May 18.

Infectious Diseases, East Tennessee State University - Quillen College of Medicine, Johnson City, USA.

Mucormycosis is a destructive, necrotizing, and potentially fatal fungal disease that usually affects immunocompromised or diabetic patients. Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis is a rare, aseptic necrotizing, granulomatous vasculitis affecting small- to medium-sized vessels, resulting in systemic manifestations. Here, we present a case of a 46-year-old gentleman with overlapping features of mucormycosis and GPA, that was successfully treated with isavuconazole monotherapy. Read More

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A case of cerebral paragonimiasis misdiagnosed as eosinophilic granulomatosis with polyangiitis.

Neuropathology 2022 Jun 20. Epub 2022 Jun 20.

Department of Neurorogical Surgery, Nihon University School of Medicine, Tokyo, Japan.

Paragonimiasis is a parasitic disease caused by Paragonimus westermani infection, and migration to the brain results in cerebral paragonimiasis. Cerebral paragonimiasis is now extremely rare, but a few cases are still reported. A 48-year-old Japanese woman presented with right-hand convulsion, right-hand numbness, sputum, and fatigue. Read More

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Diagnostic and Therapeutic Approach in ANCA-Associated Glomerulonephritis: A Review on Management Strategies.

Front Med (Lausanne) 2022 3;9:884188. Epub 2022 Jun 3.

Department of Internal Medicine and Oncology, Semmelweis University, Budapest, Hungary.

Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis is a destructive small vessel vasculitis affecting multiple organs. Renal involvement often leads to end-stage renal disease and increases mortality. Prompt diagnosis and initiation of adequate immunosuppressive therapy are critical for the best patient and kidney outcomes. Read More

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Radiological significance of mediastinal lymphadenopathy in eosinophilic granulomatosis with polyangiitis.

Allergol Int 2022 Jun 15. Epub 2022 Jun 15.

Division of Infectious Diseases and Respiratory Medicine, Department of Internal Medicine, National Defense Medical College, Saitama, Japan.

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Increased risk of venous thromboembolism in patients with granulomatosis with polyangiitis: A population-based study.

PLoS One 2022 17;17(6):e0270142. Epub 2022 Jun 17.

Arthritis Research Canada, Vancouver, British Columbia, Canada.

We assessed the risk and time trends of venous thromboembolism (VTE) including pulmonary embolism (PE) and deep venous thrombosis (DVT) in new granulomatosis with polyangiitis (GPA) cases compared to the general population. Using a population-level database from the entire province of British Columbia, Canada, we conducted a matched cohort study of all patients with incident GPA with up to ten age-, sex-, and entry time-matched individuals randomly selected from the general population. We compared incidence rates of VTE, PE, and DVT between the two groups, and calculated hazard ratios (HR), adjusting for relevant confounders. Read More

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Lung cavitation in granulomatosis with polyangiitis: clinical implications.

Clin Rheumatol 2022 Jun 16. Epub 2022 Jun 16.

Department of Internal Medicine, Hospital Universitario de Fuenlabrada, Camino del Molino, 2, 28942, Madrid, Spain.

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Portal Vein Thrombosis as a Cause of Undetermined Thrombocytopenia with Liver Dysfunction in a Patient with Eosinophilic Granulomatosis with Polyangiitis.

Intern Med 2022 Jun 14. Epub 2022 Jun 14.

Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Japan.

We herein report a 20-year-old woman who developed eosinophilic granulomatosis with polyangiitis (EGPA) and portal vein thrombosis (PVT). EGPA was diagnosed based on the patient's history of asthma, hypereosinophilia, and mononeuritis complex. Thrombocytopenia and liver dysfunction were observed, necessitating contrast-enhanced computed tomography (CECT), which revealed PVT. Read More

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Extravascular Necrotizing Granuloma: A Diagnostic Clue for Eosinophilic Granulomatosis With Polyangiitis.

J Rheumatol 2022 Jun 15. Epub 2022 Jun 15.

Department of Rheumatic Diseases, Department of Neurology, Department of Dermatology, Tokyo Metropolitan Tama Medical Center, Tokyo, Japan. Address correspondence to Dr. N. Tanomogi, Department of Rheumatic Diseases, Tokyo Metropolitan Tama Medical Center, 2-8-29 Musashidai, Fuchu, Tokyo 183-8524, Japan. Email: The authors declare no conflicts of interest relevant to this article. Ethics approval was obtained from Tokyo Metropolitan Tama Medical Center Ethics Board (approval no. 3-165). The patient's written informed consent to publish the material was obtained.

Extravascular necrotizing granuloma is pathologically one of the most characteristic findings of eosinophilic granulomatosis with polyangiitis (EGPA). A 46-year-old man presented to the rheumatology clinic with a 2-week history of arthralgia and 4-day history of dysesthesia of the feet. Read More

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Anti-Proteinase 3 Antibodies as a Biomarker for Ulcerative Colitis and Primary Sclerosing Cholangitis in Children.

J Pediatr Gastroenterol Nutr 2022 Apr 24;74(4):463-470. Epub 2021 Nov 24.

Institute of Immunology, Technische Universität Dresden, Dresden, Germany.

Objectives: Anti-neutrophil cytoplasmic antibody (ANCA) directed against proteinase 3 (PR3) is a marker for granulomatosis with polyangiitis, but is also found in patients with inflammatory bowel disease (IBD), mainly ulcerative colitis (UC). The aim of our study was to investigate ANCA and PR3-ANCA in paediatric IBD.

Methods: We tested 326 paediatric IBD patients and 164 controls for anti-Saccharomyces cerevisiae antibodies (ASCA), ANCA (indirect immunofluorescence, IIF) and PR3-ANCA (chemiluminescence immunoassay). Read More

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Granulomatosis with polyangiitis in gingiva: A rare case of isolated presentation.

Intractable Rare Dis Res 2022 May;11(2):93-95

Department of Oral and Maxillofacial Pathology, Krishnadevaraya College of Dental Sciences and Hospital, Bengaluru, India.

Granulomatosis with polyangiitis (GPA) is a rare autoimmune disease characterized by necrotising granulomatous inflammation of upper and lower respiratory tract, vasculitis and glomerulonephritis. This ailment may present with cough, haemoptysis, sinusitis, nasal deformity, skin lesions, malaise, fever, anorexia, and weight loss. Oral manifestation includes strawberry gingivitis, which is a pathognomonic clinical presentation. Read More

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[Dilated cardiomyopathy in a patient with Eosinophilic Granulomatosis with Polyangiitis (Churg Strauss)].

Rev Fac Cien Med Univ Nac Cordoba 2022 06 6;79(2):193-196. Epub 2022 Jun 6.

Sanatorio Allende, Servicio de Cardiología. Córdoba, Argentina..

Eosinophilic granulomatosis with polyangiitis, formerly known as Churg-Strauss Syndrome, is a rare pathology that belongs to the group of diseases characterized by necrotizing vasculitis of small and medium-sized systemic blood vessels. Symptomatic cardiovascular involvement occurs in 27% to 47% of Churg-Strauss cases, being one of the most serious manifestations. The diagnosis is usually confirmed with tissue biopsy showing eosinophil infiltration, but in selected cases with the recent inclusion of cardiac MRI, we can dispense with it. Read More

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Silicone Sphere Implant Extrusion From Orbital Granulomatosis With Polyangiitis: A Rare Complication in the Anophthalmic Socket.

Ophthalmic Plast Reconstr Surg 2022 Jun 14. Epub 2022 Jun 14.

Department of Ophthalmology, Indiana University School of Medicine, Indianapolis, IN, U.S.A.

Orbital implant extrusion is a known complication following evisceration and enucleation. In this case report, we present a 45-year-old woman who presented with a left silicone implant exposure and infection 2 years following evisceration with saddle nose on examination. CT of the maxillofacial bones without contrast showed bilateral soft tissue infiltration around the superior recti muscles, as well as a nasal septum perforation from extensive sinus disease. Read More

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The effect of achieving serological remission on subsequent risk of relapse, end-stage renal disease and mortality in ANCA-associated vasculitis: a target trial emulation study.

Ann Rheum Dis 2022 Jun 13. Epub 2022 Jun 13.

Clinical Epidemiology Program, Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Boston, Massachusetts, USA

Objective: To evaluate the effect of achieving a negative postinduction antineutrophil cytoplasmic antibody ANCA) assay on the risk of relapse, end-stage renal disease (ESRD) and death in ANCA-associated vasculitis (AAV).

Methods: We emulated a target trial using observational data from the Mass General Brigham AAV cohort comparing patients who achieved versus did not achieve serological remission (negative ANCA assay) within 180 days of induction. Outcomes were relapse, ESRD or death within 5 years, obtained from medical records, the US Renal Data System and the National Death Index. Read More

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Relapsing subarachnoid hemorrhage as a clinical manifestation in microscopic polyangiitis: a case report and literature review.

Clin Rheumatol 2022 Jun 11. Epub 2022 Jun 11.

Department of Rheumatology, Ren Ji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200001, China.

Microscopic polyangiitis (MPA) is a systemic small-vessel vasculitis associated with anti-neutrophil cytoplasmic antibody (ANCA) and predominantly causes kidney and pulmonary injuries. Subarachnoid hemorrhage, a life-threatening manifestation of the central nervous system (CNS), rarely occurs in patients with ANCA-associated vasculitis (AAV). We report the case of a young man with spontaneous SAH recurrence and active nephritis. Read More

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A case of cardiac calcified amorphous tumor complicated with acute myocardial infarction.

J Cardiol Cases 2022 Jun 31;25(6):396-399. Epub 2022 Jan 31.

Division of Cardiology, Department of Internal Medicine, The Jikei University School of Medicine, Tokyo, Japan.

An 80-year-old woman with a history of eosinophilic granulomatosis with polyangiitis, cardiac hypertrophy, and diabetes called for an ambulance after developing chest pain. She was diagnosed with acute myocardial infarction (AMI), and coronary angiography revealed occlusion of the right coronary artery. Coronary aspiration was performed, and coronary aspirate was white with calcified factor. Read More

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Clinical Images: Granulomatosis with Polyangiitis and ATTR Amyloidosis.

Arthritis Rheumatol 2022 Jun 6. Epub 2022 Jun 6.

Reha Rheinfelden, Research Department, 4310, Rheinfelden, Switzerland.

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Granulomatosis with polyangiitis presenting with unilateral exophthalmos: A case report.

Radiol Case Rep 2022 Aug 28;17(8):2626-2629. Epub 2022 May 28.

Radiology Department, Specialty hospital, Mohamed V University, Rabat, Morocco.

We report a case of a patient with no medical history, admitted for right exophthalmos. For whom imaging showed orbital masses without inflammatory signs pointing to a granulomatous origin. However, the histological and immunological workup revealed the diagnosis of Granulomatosis with polyangiitis. Read More

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Granulomatosis With Polyangiitis Presenting as Complete Hearing Loss.

Cureus 2022 May 3;14(5):e24711. Epub 2022 May 3.

Internal Medicine, University of Louisville, Louisville, USA.

Granulomatosis with Polyangiitis is a rare autoimmune vasculitis that is classically characterized by effects on the upper respiratory tract, lungs, and kidneys. Delay in diagnosis is often attributed to variable and sequential presentation of symptoms rather than concurrent symptomatology. It is important to recognize the wide range of initial presenting symptoms as early diagnosis and treatment is critical in preventing potentially irreversible damage resulting from delayed diagnosis. Read More

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Sclerokeratitis in Granulomatosis with Polyangiitis.

N Engl J Med 2022 06 4;386(23):2221. Epub 2022 Jun 4.

John A. Moran Eye Center, Salt Lake City, UT

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Meta-analysis confirmed genetic susceptibility conferred by multiple risk variants from CTLA4 and SERPINA1 in granulomatosis with polyangiitis.

Int J Rheum Dis 2022 Jun 3. Epub 2022 Jun 3.

Immunogenomics Laboratory, Department of Human Genetics & Molecular Medicine, School of Health Sciences, Central University of Punjab, Ghudda, Punjab, India.

Background: Granulomatosis with polyangiitis (GPA) is a rare systemic autoimmune disease. Smaller sample size and complex nature of the disease pathogenesis has made it challenging to perform well-powered genetic investigations. We performed a systematic review based meta-analysis in GPA to investigate the genetic susceptibility conferred by non-human leukocyte antigen (non-HLA) candidate genes. Read More

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Reversible Diffuse Bronchial Wall Thickening.

Intern Med 2022 May 31. Epub 2022 May 31.

Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Japan.

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MPO-ANCA-Positive Granulomatosis with Polyangiitis with Rapidly Progressive Glomerulonephritis and Saddle-Nose Deformity: A Case Report.

Antibodies (Basel) 2022 May 9;11(2). Epub 2022 May 9.

Department of Nephrology, National and Kapodistrian University of Athens, Attikon Hospital, 12462 Athens, Greece.

Early diagnosis and initiation of appropriate immunosuppressive treatment remain the cornerstone of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis at the cost of significant toxicity. In this report, we present a case of a 69-year-old female who presented with advanced renal insufficiency and evidence of pulmonary hemorrhage and was MPO-ANCA-positive with a clinical phenotype of granulomatosis with polyangiitis. Organ involvement included rapidly progressive glomerulonephritis (GN), along with extrarenal manifestations (skin, upper and lower respiratory system involvement, and onset of saddle-nose deformity). Read More

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[Eosinophilic granulomatosis with polyangiitis presenting with ischemic optic neuropathy].

Rinsho Shinkeigaku 2022 May 28. Epub 2022 May 28.

Department of Neurology, Kitano Hospital, Tazuke Kofukai Medical Research Institute.

A 60-year-old man with a history of bronchial asthma and nasal polyp presented with loss of vision in the right eye. His visual loss progressed within a single day, and he presented to our hospital 5 days after the onset of the symptom. Fundoscopy showed swelling and hemorrhage of the right optic disc. Read More

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Ophthalmic manifestations and visual outcomes of granulomatosis with polyangiitis: a retrospective multicentre study in Korea.

Eye (Lond) 2022 May 28. Epub 2022 May 28.

Department of Ophthalmology, Seoul National University College of Medicine, Seoul National University Hospital, Seoul, Korea.

Objectives: To analyse the ophthalmic manifestations and treatment outcomes of Korean patients with granulomatosis with polyangiitis (GPA).

Methods: One hundred twenty patients diagnosed with GPA by rheumatologists from January 1984 to March 2019 at three referral centres were retrospectively reviewed. Patients with ophthalmic symptoms were examined by ophthalmologists and underwent orbital imaging. Read More

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Effect of Dupilumab on Blood Eosinophil Counts in Patients With Asthma, Chronic Rhinosinusitis With Nasal Polyps, Atopic Dermatitis, or Eosinophilic Esophagitis.

J Allergy Clin Immunol Pract 2022 May 27. Epub 2022 May 27.

Regeneron Pharmaceuticals, Inc., Tarrytown, NY, USA.

Background: Transient increases in blood eosinophil counts have been observed in dupilumab clinical trials.

Objective: Assessment of eosinophil counts and eosinophilia-related treatment-emergent adverse events (TEAEs) across 11 dupilumab clinical trials, comparing adult and adolescent patients with asthma, and adult patients with chronic rhinosinusitis with nasal polyps (CRSwNP), atopic dermatitis (AD), and eosinophilic esophagitis (EoE).

Methods: Eosinophil counts, rates of eosinophilia-related TEAEs or treatment-emergent eosinophilia (>1500 cells/μL), discontinuations, clinical symptoms, and efficacy in patients with asthma or CRSwNP with treatment-emergent eosinophilia are presented. Read More

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