{"title":"Granulomatosis with polyangiitis presenting with isolated external genital and urethral manifestations: a case-based review.","authors":"Haruka Noda, Shuntaro Isogai, Taio Naniwa","doi":"10.1007/s00296-025-05837-z","DOIUrl":null,"url":null,"abstract":"<p><p>Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis primarily affecting the respiratory tract and kidneys, with external genital and urethral lesions (EGUL) being exceedingly rare. We present a case of a middle-aged man with relapsing proteinase 3-antineutrophil antibody-positive GPA who developed isolated granulomatous, ulcerative balanitis, and urethritis. His condition abruptly worsened after a prolonged indolent course, requiring treatment with glucocorticoids and rituximab, leading to successful remission. To better characterize EGUL in GPA, a systematic literature search was performed in PubMed, Scopus, and the NPO Japanese Society of Medical Abstracts databases using keywords related to GPA and EGUL. Cases meeting the American College of Rheumatology or Japanese Ministry of Health, Labor, and Welfare criteria for GPA and the 2012 Chapel Hill Consensus Conference definitions were included for analysis. Our review identified that EGUL often presents as an initial symptom and can be classified by the presence or absence of preceding urethritis. Cases with preceding urethritis had a higher risk of severe complications with extensive penile or urethral involvement. In contrast, penile lesions without preceding urethritis typically presented as characteristic mucosal lesions localized around the glans. In females, GPA-associated urethritis frequently led to periurethral mass formation, vaginal involvement, and significant sequelae. Given the potential for delayed diagnosis and serious complications, clinicians should be vigilant for EGUL in patients with suspected or diagnosed GPA. Future prospective studies with larger cohorts are needed to elucidate the prevalence, clinical spectrum, and optimal management of these rare but significant manifestations of GPA.</p>","PeriodicalId":21322,"journal":{"name":"Rheumatology International","volume":"45 4","pages":"89"},"PeriodicalIF":3.2000,"publicationDate":"2025-04-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Rheumatology International","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1007/s00296-025-05837-z","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"RHEUMATOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis primarily affecting the respiratory tract and kidneys, with external genital and urethral lesions (EGUL) being exceedingly rare. We present a case of a middle-aged man with relapsing proteinase 3-antineutrophil antibody-positive GPA who developed isolated granulomatous, ulcerative balanitis, and urethritis. His condition abruptly worsened after a prolonged indolent course, requiring treatment with glucocorticoids and rituximab, leading to successful remission. To better characterize EGUL in GPA, a systematic literature search was performed in PubMed, Scopus, and the NPO Japanese Society of Medical Abstracts databases using keywords related to GPA and EGUL. Cases meeting the American College of Rheumatology or Japanese Ministry of Health, Labor, and Welfare criteria for GPA and the 2012 Chapel Hill Consensus Conference definitions were included for analysis. Our review identified that EGUL often presents as an initial symptom and can be classified by the presence or absence of preceding urethritis. Cases with preceding urethritis had a higher risk of severe complications with extensive penile or urethral involvement. In contrast, penile lesions without preceding urethritis typically presented as characteristic mucosal lesions localized around the glans. In females, GPA-associated urethritis frequently led to periurethral mass formation, vaginal involvement, and significant sequelae. Given the potential for delayed diagnosis and serious complications, clinicians should be vigilant for EGUL in patients with suspected or diagnosed GPA. Future prospective studies with larger cohorts are needed to elucidate the prevalence, clinical spectrum, and optimal management of these rare but significant manifestations of GPA.
期刊介绍:
RHEUMATOLOGY INTERNATIONAL is an independent journal reflecting world-wide progress in the research, diagnosis and treatment of the various rheumatic diseases. It is designed to serve researchers and clinicians in the field of rheumatology.
RHEUMATOLOGY INTERNATIONAL will cover all modern trends in clinical research as well as in the management of rheumatic diseases. Special emphasis will be given to public health issues related to rheumatic diseases, applying rheumatology research to clinical practice, epidemiology of rheumatic diseases, diagnostic tests for rheumatic diseases, patient reported outcomes (PROs) in rheumatology and evidence on education of rheumatology. Contributions to these topics will appear in the form of original publications, short communications, editorials, and reviews. "Letters to the editor" will be welcome as an enhancement to discussion. Basic science research, including in vitro or animal studies, is discouraged to submit, as we will only review studies on humans with an epidemological or clinical perspective. Case reports without a proper review of the literatura (Case-based Reviews) will not be published. Every effort will be made to ensure speed of publication while maintaining a high standard of contents and production.
Manuscripts submitted for publication must contain a statement to the effect that all human studies have been reviewed by the appropriate ethics committee and have therefore been performed in accordance with the ethical standards laid down in an appropriate version of the 1964 Declaration of Helsinki. It should also be stated clearly in the text that all persons gave their informed consent prior to their inclusion in the study. Details that might disclose the identity of the subjects under study should be omitted.