Vasculite necrosante de pequenos vasos caracterizada pela associação de inflamação da parede do vaso e granulomatose peri e extravascular.
Introdução
O que você precisa saber de cara
Granulomatose com poliangiite (GPA) é uma condição que causa inflamação que afeta principalmente o trato respiratório (incluindo os pulmões e as vias aéreas) e os rins. Esse distúrbio era anteriormente conhecido como granulomatose de Wegener. Uma característica da GPA é a inflamação dos vasos sanguíneos (vasculite), particularmente dos vasos sanguíneos pequenos e de tamanho médio nos pulmões, nariz, seios nasais, traqueia e rins, embora vasos em qualquer órgão possam estar envolvidos. Poliangiite refere‑se à inflamação de múltiplos tipos de vasos, como pequenas artérias e veias. A vasculite causa cicatrização e morte do tecido nos vasos e impede o fluxo sanguíneo para tecidos e órgãos.
Outra característica da GPA é a formação de granulomas, que são pequenas áreas de inflamação compostas por células do sistema imunológico que auxiliam na reação inflamatória. Os granulomas geralmente ocorrem nos pulmões ou nas vias aéreas de pessoas com esta condição, embora possam aparecer nos olhos ou em outros órgãos. À medida que os granulomas crescem, podem invadir áreas circundantes, causando dano ao tecido.
Os sinais e sintomas da GPA variam de acordo com os tecidos e órgãos afetados pela vasculite. Muitas pessoas com a condição experimentam uma sensação vaga de desconforto (mal‑estar), febre, perda de peso ou outros sintomas gerais da reação imunológica do corpo. Na maioria das pessoas com GPA, a inflamação começa nos vasos do trato respiratório, levando a congestão nasal, sangramentos nasais frequentes, falta de ar ou tosse. Inflamação grave no nariz pode levar a um orifício no tecido que separa as duas narinas (perfuração do septo nasal) ou ao colapso do septo, causando um nariz com ponte afundada (nariz em sela).
Os rins são comumente afetados em pessoas com GPA. O dano ao tecido causado pela vasculite nos rins pode levar a diminuição da função renal, o que pode causar aumento da pressão arterial ou sangue na urina, e insuficiência renal que ameaça a vida. A inflamação também pode ocorrer em outras regiões do corpo, incluindo os olhos, estruturas do ouvido médio e interno, pele, articulações, nervos, coração e cérebro. Dependendo dos sistemas envolvidos, sintomas adicionais podem incluir erupções cutâneas, dor no ouvido interno, articulações inchadas e doloridas, e formigamento ou dormência nos membros.
A GPA é mais comum em adultos de meia‑idade, embora possa ocorrer em qualquer idade. Se não tratada, a condição costuma ser fatal dentro de 2 anos após o diagnóstico. Mesmo após o tratamento, a vasculite pode retornar.
Fonte: MedlinePlus Genetics (NLM/NIH), traduzidoVasculite necrosante de pequenos vasos caracterizada pela associação de inflamação da parede do vaso e granulomatose peri e extravascular.
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 56 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 129 características clínicas mais associadas, ordenadas por frequência.
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5 genes identificados com associação a esta condição. Padrão de herança: Not applicable.
Systemic lupus erythematosus
A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
Systemic lupus erythematosus
A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
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Publicações mais relevantes
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[Successful early use of mepolizumab in eosinophilic granulomatosis with polyangiitis presenting with peripheral neuropathy and gastrointestinal bleeding].
We report a case of a 55-year-old male with eosinophilic granulomatosis with polyangiitis (EGPA) presenting with peripheral neuropathy and gastrointestinal (GI) bleeding. Despite initial treatment with high-dose intravenous methylprednisolone and intravenous immunoglobulin, the patient's symptoms persisted, and GI bleeding worsened. Capsule endoscopy revealed multiple ulcers and erosions throughout the small intestine, and biopsies from the ileum, cecum, and skin showed marked eosinophilic infiltration. Although peripheral blood eosinophil counts were normalized, tissue eosinophilia remained evident. Early administration of mepolizumab, an anti-IL-5 monoclonal antibody, led to rapid improvement in both GI bleeding and neuropathic symptoms. This case highlights the potential role of tissue-infiltrating eosinophils in EGPA pathogenesis and suggests that mepolizumab may be effective even when blood eosinophil levels are not elevated. Early intervention with mepolizumab may be beneficial in severe or refractory EGPA cases with GI involvement, which are associated with poor prognosis and high relapse rates. Careful monitoring and timely therapeutic decisions are essential for improving outcomes in such patients.
Clinical and Pathological Features of Vasculitic Neuropathy: A Single-Center Study in China.
Vasculitic neuropathy (VN) is a disease in which vessel inflammation happens and injures peripheral nerves. Despite increasing awareness, features of VN in mainland China are still understudied. To characterize the clinical, pathological features and outcomes of VN in mainland China, and evaluate clinicopathological correlations: METHODS: We retrospectively reviewed records of VN patients diagnosed pathologically between June 1999 and December 2024, including demographic data, clinical manifestations, biopsy features, and outcomes. 112 patients were totally included. All presented with axonal sensorimotor neuropathy, most commonly involving the tibial (90.63%) and peroneal (87.50%) nerves. Systemic VN (SVN) showed more frequent transmural inflammatory cell infiltration (ICI) than non-systemic VN (NSVN) (p = 0.046). Eosinophilic granulomatosis with polyangiitis (EGPA) was more common in VN without ICI than VN with ICI (p = 0.008). In SVN, the ICI-positive exhibited more severe distal upper limb weakness (p = 0.042) and higher thrombosis rates (p = 0.001) than ICI-negative. Of the 63 patients followed, 62 received glucocorticoids with or without immunosuppressants. Thirteen died from multi-organ complications (12 SVN and 1 NSVN), while others achieved remission. The 5-year all-survival rate was 80.23% (95% CI 66.83%- 88.66%). This first large cohort of VN in mainland China delineates its clinical-pathological features. EGPA showed a lower diagnostic yield on biopsy, suggesting diverse mechanisms of vascular injury. Nerve biopsy remains the diagnostic gold standard. The overall prognosis of VN is relatively favorable, emphasizing the need for early recognition and treatment.
Leukotriene receptor antagonists and eosinophilic granulomatosis with polyangiitis: a disproportionality analysis from FAERS, JADER, CVAR databases integrated with network pharmacology.
The relationship between leukotriene receptor antagonists (LTRAs) usage and the subsequent occurrence of eosinophilic granulomatosis with polyangiitis (EGPA) remained highly polarizing and controversial in previous studies. We aimed to investigate the risk of EGPA caused by LTRAs and the potential toxicological mechanisms of LTRAs-related EGPA. In this real-world pharmacovigilance study, we collected adverse event (AE) reports of EGPA associated with LTRAs use from the U.S. FDA Adverse Event Reporting System (FAERS), Japanese Adverse Drug Event Reporting (JADER), and Canadian Vigilance Adverse Reaction (CVAR) databases. The reporting odds ratio (ROR), proportional reporting ratio (PRR), information component (IC), and empirical Bayesian geometric mean (EBGM) were calculated to quantify the strength of the association between LTRAs and EGPA. The Weibull shape parameter (WSP) test was applied to analyze time-to-onset profiles of EGPA toxicity. Network pharmacology analysis was subsequently performed to identify the central genes to determine the potential mechanisms underlying LTRAs-induced EGPA. LTRAs, including montelukast, zafirlukast, and pranlukast, exhibited a strong association with EGPA in three databases (the lower limit of 95% confidence interval (CI) for ROR > 1, PRR > 2 with χ2 values ≥4, EBGM05 > 2, and IC025 > 0). After excluding corticosteroids as concomitant medication, montelukast remained significantly associated with EGPA in the FAERS database. The median time-to-onset of EGPA associated with LTRAs was 233 (range: 76-660) days, and the WSP test indicated LTRAs had early failure-type profiles. We isolated 81 interactive target genes linking LTRAs to EGPA. Several central genes, including SRC, PTGS2, EDN1, HMOX1, KDR, and OCLN, were revealed via protein-protein interactions analysis and molecular complex detection (MCODE) algorithm. Our study revealed LTRAs could increase the risk of EGPA, and initially explored potential genes and mechanisms of LTRAs-induced EGPA. It is helpful for clinicians to be alerted to the risk of EGPA during LTRAs administration.
Tuberculosis or vasculitis? Granulomatous inflammation of the pancreatic tail as an atypical presentation of granulomatosis with polyangiitis.
Granulomatosis with polyangiitis (GPA), a subtype of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), is a systemic inflammatory disease that typically affects the respiratory tract and kidneys. However, atypical pancreatic manifestations have been reported, which may present clinically as acute pancreatitis or exocrine insufficiency, and radiologically as pancreatic enlargement or pseudotumour.A female in her 40s presented with nasal crusting and epistaxis. She subsequently developed fever, weight loss, haemoptysis, bilateral pulmonary nodules and a pancreatic tail mass. Pancreatic biopsy revealed caseating granulomatous inflammation, and antitubercular therapy was commenced.On developing haematuria and proteinuria, proteinase 3-ANCA testing and renal biopsy confirmed AAV. Treatment with rituximab and high-dose corticosteroids led to clinical improvement and radiological regression of both pulmonary and pancreatic lesions, consistent with systemic GPA involvement.This case highlights the importance of considering systemic inflammatory disease, alongside infective and malignant aetiologies, when evaluating pancreatic masses or focal pancreatitis with constitutional symptoms and multiorgan involvement.
ANCA-associated vasculitis following sotatercept initiation in a patient with heritable pulmonary arterial hypertension and previously silent eosinophilic granulomatosis with polyangiitis: a case report.
Pulmonary arterial hypertension (PAH) is a progressive disease characterized by increased pulmonary vascular resistance and right ventricular failure. Sotatercept, a novel activin receptor ligand trap, has demonstrated promising haemodynamic benefits in PAH treatment. Eosinophilic granulomatosis with polyangiitis (EGPA) is an extremely rare, Th2-driven, small-vessel vasculitis, and its overlap with PAH is scarcely reported. We report the case of a woman with heritable PAH initially stabilized with oral therapy. In 2024, after further clinical decline, subcutaneous treprostinil was initiated. Subsequently, sotatercept was added, resulting in brief clinical improvement. Within weeks, however, the patient developed severe eosinophilia and exhibited laboratory and histopathological evidence of p-ANCA-positive necrotizing vasculitis, accompanied by renal and hepatocellular dysfunction. This case suggests that sotatercept's modulation of the TGF-β pathway may unmask latent autoimmune diseases such as EGPA in predisposed individuals. Although the temporal relationship between sotatercept initiation and the onset of vasculitis is compelling, both causality and underlying molecular mechanisms remain to be elucidated. Further studies are necessary to understand the potential immunomodulatory mechanisms of sotatercept.
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Evaluation of the Long-Term Safety of Avacopan in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis in the Real World (AvacoStar): Protocol for a Noninterventional Prospective Cohort Study.
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CureusIsolated antineutrophil cytoplasmic antibody-associated interstitial lung disease: A systematic review of an emerging condition.
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Medicina clinica[Risk factors of unfavorable clinical outcome in orbital granulomatosis with polyangiitis].
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European journal of neurologyAdvances in the treatment of antineutrophil cytoplasmic antibody associated vasculitis: current recommendations, clinical trials and real word data.
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ERJ open researchEpidemiology of eosinophilic granulomatosis with polyangiitis in northern Spain: A population-based study (2000-2024) and literature review.
Autoimmunity reviewsPredictors of glucocorticoid-free clinical remission in patients with newly diagnosed microscopic polyangiitis and granulomatosis with polyangiitis: a retrospective cohort study using a nationwide registry in Japan (J-CANVAS).
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The LaryngoscopeA Case of Eosinophilic Granulomatosis with Polyangiitis Emerging During Benralizumab Therapy: Successful Management Through a Switch to Mepolizumab Therapy.
Journal of asthma and allergyBurden of eosinophilic granulomatosis with polyangiitis by disease phase and steroid-sparing effects of biologics: a real-world retrospective study in Europe.
ERJ open researchA Breach in the Lung: Broncho-Pleural Fistula in the Setting of Granulomatosis With Polyangiitis.
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Vestnik otorinolaringologiiPET/CT Features of Granulomatosis With Polyangiitis Mimicking Left Lung and Tracheal Malignancy.
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American journal of respiratory and critical care medicinePeripheral ulcerative keratitis (PUK) in autoimmune diseases: a severe ocular manifestation not always associated with systemic disease activity.
European journal of internal medicineClinical outcomes of subglottic stenosis in granulomatosis with polyangiitis: results of an international multicenter observational study.
Rheumatology (Oxford, England)A pharmacovigilance study of rituximab-associated adverse events in immune-mediated kidney diseases and transplant-related kidney diseases.
International journal of surgery (London, England)ANCA-Negative Granulomatous Polyangiitis Presenting With Isolated Oral and Esophageal Involvement.
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Current opinion in nephrology and hypertensionEosinophilic granulomatosis with polyangiitis: recent therapeutic advances.
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Modern rheumatologyTuberculosis or vasculitis? Granulomatous inflammation of the pancreatic tail as an atypical presentation of granulomatosis with polyangiitis.
BMJ case reportsEffectiveness of Mepolizumab in Severe Uncontrolled Asthma Associated or Not with EGPA Based on the Exacto Scale and Separ-Remas Criteria.
Journal of asthma and allergyStructures of proteinase 3 and the CD177 receptor complex reveal a major autoantibody epitope.
EMBO reportsEosinophils ETosis as a novel contributor to the pathogenesis of granulomatosis with polyangiitis.
Clinical and experimental immunologyEarly Strawberry Gingivitis in Granulomatosis with Polyangiitis.
Journal of general internal medicineRole of biological therapies targeting eosinophils in eosinophilic granulomatosis with polyangiitis: current evidence and future perspectives.
Current medical research and opinion[When inflammation gets sneaky : unmasking granulomatosis with polyangiitis with [18F]FDG PET/CT].
Revue medicale de LiegeOvercoming challenges: magnetic resonance imaging in the detection and monitoring of rare prostate involvement in granulomatosis with polyangiitis.
Clinical and experimental rheumatologyFatal Granulomatosis with Polyangiitis Presenting as Medial Longitudinal Fasciculus Syndrome with Gastrointestinal Perforation: An Autopsy Case Report.
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International journal of rheumatic diseases[RECONSIDERATION OF EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (EGPA)].
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Polish archives of internal medicineUnmasking EGPA with near fatal diffuse alveolar haemorrhage in severe eosinophilic asthma under treatment with benralizumab: a case report.
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Journal of clinical medicineUpdate on antineutrophil cytoplasmic antibody vasculitis.
Current opinion in nephrology and hypertensionBenralizumab: Bringing winds of change to eosinophil-associated diseases.
The World Allergy Organization journalANCA-Negative Eosinophilic Granulomatosis With Polyangiitis Presenting With Duodenal Stenosis: A Case Report.
The American journal of case reportsImaging the Cardiac Infiltration: Granulomatosis with Polyangiitis.
European heart journal. Cardiovascular ImagingScleritis and associated systemic diseases: contribution of systemic examination, follow-up, and additional investigations.
Journal of ophthalmic inflammation and infectionPyoderma gangrenosum associated with granulomatosis with polyangiitis: a case report.
Journal of medical case reportsMethotrexate in the Management of Laryngotracheal Stenosis: A Narrative Review.
Journal of voice : official journal of the Voice FoundationEosinophilic granulomatosis with polyangiitis diagnosed during tezepelumab treatment for severe asthma: A case report.
Allergology international : official journal of the Japanese Society of AllergologyAnti-Programmed Death-Ligand 1 Antibody-Associated Eosinophilic Granulomatosis with Polyangiitis in Non-Small Cell Lung Cancer: A Case Report.
Internal medicine (Tokyo, Japan)A case of antineutrophil cytoplasmic antibody-associated vasculitis and sclerosing mediastinitis.
Modern rheumatology case reportsShifting Sands or ANCA-ed in Place? The Epidemiology of ANCA-Associated Vasculitis over 2 Decades at a Large Tertiary Centre.
Glomerular diseasesGranulomatosis With Polyangiitis (Wegener Granulomatosis): Then and Now.
Archives of pathology & laboratory medicineSerum Metrnl as a potential biomarker for renal involvement in ANCA-associated vasculitis.
Clinical and experimental immunologyUpdated treatment approaches for eosinophilic granulomatosis with polyangiitis: A systematic scoping review.
Modern rheumatologyAnti-proteinase 3 Antibody (PR3)-Anti-neutrophil Cytoplasmic Antibody (ANCA)-Positive Vasculitis: A Rare Presentation in a 90-Year-Old Female.
CureusOverview of Biologics Targeting Type 2 Inflammation in Respiratory Disease.
The journal of allergy and clinical immunology. In practiceInside GEMA 5.5: Expert Insights on the Latest Changes in Asthma Management.
Archivos de bronconeumologiaCritical limb Ischaemia in granulomatosis with polyangiitis: a rare but severe complication.
Modern rheumatology case reportsVenous Thromboembolism May Be Increased in the Early Phases of ANCA-Associated Vasculitis and Could Be Associated With a High Body Mass Index: TR-VaS Experience.
International journal of rheumatic diseasesSclerosing mesenteritis as a manifestation of granulomatosis with polyangiitis: expanding the disease spectrum.
Rheumatology (Oxford, England)Eosinophilic Granulomatosis With Polyangiitis Presenting With Syphilitic-Like Palmar Erythema.
International journal of rheumatic diseasesPost-COVID-19 shifts in granulomatosis with polyangiitis: epidemiology, clinical features, and predictors in Iran.
Annals of medicine and surgery (2012)Nanomedicine-driven microRNA therapy: a precision approach for eosinophilic granulomatosis with polyangiitis.
Annals of medicine and surgery (2012)Bilateral Ischaemic Stroke in Eosinophilic Granulomatosis With Polyangiitis.
CureusOverlapping forms of granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis: Insights from a European multicenter study.
Journal of internal medicineA case report of eosinophilic granulomatosis with polyangiitis in children with cerebella infarction as the first symptom and literature review.
Frontiers in immunologyEffective Use of Mepolizumab in a Rare Case of Eosinophilic Granulomatosis With Polyangiitis Complicated by Pulmonary Tuberculosis.
CureusAngiolymphoid Hyperplasia With Eosinophilia Vs Kimura Disease: Continuum of the Same Disease or a Distinct Entity-A Case Report With Literature Review.
Case reports in medicineCirculating peripheral helper T cells are expanded and associate with disease activity in granulomatosis with polyangiitis.
Arthritis research & therapyDual pathogenesis and treatment approaches for eosinophilic granulomatosis with polyangiitis: a comprehensive review.
ReumatologiaRESER/NVAN: Spanish Registry of patients with ANCA- associated vasculitis: objectives and methodology.
Reumatologia clinicaAdjunctive dual biologic therapy for persistent nasal symptoms in eosinophilic granulomatosis with polyangiitis (EGPA).
American journal of otolaryngologyTuberculosis or GPA? An Unusual, Multifocal Granulomatosis With Polyangiitis of the Head and Neck.
Ear, nose, & throat journalLupus vorax: A rare case of facial tuberculosis in a patient with Noonan syndrome.
IDCasesAcute Eosinophilic Myocarditis and Heart Failure As the First Manifestation of Eosinophilic Granulomatosis With Polyangiitis (EGPA) in an Asthmatic Patient: A Diagnostic Challenge.
CureusDermatomyositis and microscopic polyangiitis overlap: a case-based review.
Rheumatology internationalPrediction of relapses in patients with small vessel vasculitides: a multicenter cohort study on histopathological risk patterns.
Rheumatology internationalClinical profile and outcome of patients with ANCA-associated vasculitis with special reference to vasculitis damage index: a longitudinal observational study.
Rheumatology internationalAssociation of Individual or Family History of Autoimmune Disease With Future Development of Type 1 Diabetes.
Diabetes/metabolism research and reviewsGlucocorticoid-free remission with simultaneous mepolizumab and rituximab in life-threatening PR3-ANCA-positive eosinophilic granulomatosis with polyangiitis: a case report and literature review.
Clinical rheumatologyEosinophilic granulomatosis with polyangiitis mimicking giant cell arteritis.
Canadian journal of ophthalmology. Journal canadien d'ophtalmologieGranulomatosis With Polyangiitis Presenting as a Rapidly Enlarging Choroidal Mass: A Case Report.
CureusPrimary hypophysitis: Classification review.
Journal of neuropathology and experimental neurologyPrediction model to identify patients with hypereosinophilic syndrome using real-world data.
The journal of allergy and clinical immunology. GlobalHypereosinophilic syndrome with pulmonary and hepatic involvement mimicking autoimmune disease.
Oxford medical case reportsGranulomatosis With Polyangiitis (GPA) Presenting With Painless Scleritis and Ocular Hypertension: Case Report.
Case reports in immunologyMolecular Pathogenesis and Targeted Therapies in Eosinophilic Granulomatosis with Polyangiitis: An Updated Review.
International journal of molecular sciencesPR3-ANCA Positive Behçet's Disease with Severe Multisystem Involvement: A Diagnostic Challenge.
Diagnostics (Basel, Switzerland)Abdominal pain as the sole initial manifestation of ANCA-positive EGPA with myocarditis: a case report and literature review.
BMC cardiovascular disordersHow to choose a biologic agent considering comorbidities of bronchial asthma.
Respiratory investigationANCA-negative Pauci-immune Vasculitis, an Often-missed Clinical Entity: A Case Report.
The Journal of the Association of Physicians of IndiaEarly Diagnosis and Treatment in Patients With Eosinophilic Granulomatosis With Polyangiitis.
The journal of allergy and clinical immunology. In practiceSexual Function in Patients With Systemic Vasculitis: An Overlooked Aspect of Patient Care. Results From a Cross-sectional Study.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesDistinct predictors of peripheral neuropathy in antineutrophil cytoplasmic antibody-associated vasculitis: KEIO-vasculitis cohort.
Seminars in arthritis and rheumatismMosunetuzumab, a CD20xCD3 bispecific T-cell engager, in granulomatosis with polyangiitis.
Annals of the rheumatic diseasesCoronary vasospasms and other cardiac manifestations in Eosinophilic Granulomatosis with Polyangiitis: Clinical impact and frequency in a monocentre study of 103 patients.
RMD openEpidemiology of anti-neutrophil cytoplasmic antibody-associated vasculitis in Lithuania, 2012-2021: a retrospective cohort study.
BMJ openEstimation of Health Utility Values for Eosinophilic Granulomatosis With Polyangiitis.
PharmacoEconomics - openClinical, Biomarker, and Radiological Progression from Asthma to Systemic Eosinophilic Granulomatosis with Polyangiitis: A Retrospective Cohort Study.
Journal of asthma and allergyUlcerative colitis complicated by granulomatosis with polyangiitis treated with tacrolimus.
Arab journal of gastroenterology : the official publication of the Pan-Arab Association of GastroenterologyCoronary Vasospasm in Eosinophilic Granulomatosis With Polyangiitis Presenting as Acute Coronary Syndrome Treated With Anti-IL-5.
JACC. Case reportsCardiac amyloidosis presenting as right ventricular failure: a case report.
QJM : monthly journal of the Association of PhysiciansCurrent definition of remission in eosinophilic granulomatosis with polyangiitis (EGPA) and future perspectives.
Allergology international : official journal of the Japanese Society of AllergologyModification of 2022 ACR/EULAR ANCA-associated vasculitis classification criteria in a Japanese cohort (J-CANVAS).
Clinical and experimental rheumatologyLoeffler endocarditis in hypereosinophilic syndrome, the paradox of absent peripheral eosinophilia: a case report.
European heart journal. Case reportsGranulomatosis with Polyangiitis (GPA) in a Polish Tertiary Centre (2010-2025): Sex-Stratified Phenotypes, Serology, and Evolving Treatment Patterns.
Journal of clinical medicinePhenotype, serotype, and data-driven clustering reveal complementary dimensions of heterogeneity in ANCA-associated vasculitis: a multicentre Japanese cohort (J-CANVAS).
Rheumatology internationalChronic rhinosinusitis is a risk factor for interstitial lung disease in Sjogren's syndrome.
BMC pulmonary medicineHistoplasmosis-Associated Hemophagocytic Lymphohistiocytosis in the Setting of Granulomatosis With Polyangiitis.
Journal of medical casesGranulomatosis With Polyangiitis Manifesting With Diabetes Insipidus and Cranial Nerve Involvement: A Case Report and Review of the Literature.
Clinical case reportsCase Report: Sequential use of tezepelumab and mepolizumab for eosinophilic otitis media in EGPA: a steroid-sparing strategy.
Frontiers in allergyCombined intracavitary, systemic antifungal and surgical treatment of Chronic Pulmonary Aspergillosis: experience from an immunocompromised patient.
Medical mycology case reportsBenralizumab for adults with rare and off-label eosinophilic disorders: a 52-week prospective, single-center study.
Frontiers in immunologySaddle Nose Deformity in Granulomatosis With Polyangiitis: Clinical and Radiologic Predictors From a Multicenter Cohort.
International forum of allergy & rhinologyAssociações
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Doença com base genética
Um médico geneticista pode ajudar no diagnóstico de Granulomatose com poliangeíte e no aconselhamento genético da família.
Doenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
Perguntas frequentes
O que as famílias mais perguntam sobre esta doença — cada resposta com a fonte de onde saiu
É uma vasculite rara que provoca inflamação destrutiva nos vasos de pequeno calibre e gera lesões granulomatosas. Seu envolvimento costuma ser mais acentuado nas vias aéreas e nos rins.
Referências
Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete
10 publicações do grafo RarasNet (PubMed) · 8 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Citar este verbete
Raras. (s.d.). Granulomatose com poliangeíte. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/granulomatose-com-poliangeite
Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
Granulomatose com poliangeíte
📋 Origem dos dados
Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.
- Doença rara (ontologia)
- fonte: Orphanet
- Identificador unificado
- fonte: MONDO
- Genética mendeliana
- fonte: OMIM
- Codificação WHO/SUS
- fonte: WHO ICD-10 / DATASUS
- CID-11 (futuro)
- fonte: WHO ICD-11
- NIH/GARD
- fonte: GARD (NIH)
- Indexação biomédica
- fonte: MeSH (NLM)
- Dado público estruturado
- fonte: Wikidata
- Medicamentos aprovados FDA
- fonte: FDA OpenFDA
- Ensaios clínicos
- fonte: ClinicalTrials.gov
