Granulomatosis with polyangiitis (GPA) is a systemic necrotizing granulomatosis vasculitis characterized by predilection to affect small- and medium-sized blood vessels and commonly affects the upper and lower respiratory tract and kidneys in most cases. Genital involvement is reported in <1% of cases in large cohorts and nearly all cases have been in the setting of multisystemic disease or during the course of the disease in patients already diagnosed as GPA. A case is presented of uncommon limited urogenital GPA in a 66-year-old woman with an irregular mass occupying urethra and vagina. The patient showed a good response after Corticoids and Methotrexate.
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http://dx.doi.org/10.1155/2017/9407675 | DOI Listing |
Purpose: This study investigates the capabilities of ultrasonography (US) in determing the stage of orbital inflammation in patients with granulomatosis with polyangiitis (GPA).
Material And Methods: The study included 24 patients (8 men and 16 women) with diffuse orbital tissue involvement in GPA. Group 1 (active stage) included nine patients, while group 2 (inactive stage) consisted of 18 patients.
Vestn Otorinolaringol
December 2024
Pavlov Ryazan State Medical University, Ryazan, Russia.
The article presents an analysis of 2 clinical cases of granulomatosis with polyangiitis from the practice of an otorhinolaryngologist. In the first case, the pathology manifested with lesions of the middle ear and nose. In the second case, in addition to manifestations from the nose and the ear, there was a lesion of the larynx.
View Article and Find Full Text PDFCureus
November 2024
Pulmonary & Critical Care, Indiana University Health Methodist Hospital, Indianapolis, USA.
Rituximab is an anti-CD20 monoclonal antibody medication used in treating various cancers like non-Hodgkin lymphomas as well as immunologic conditions like granulomatosis with polyangiitis. It disrupts and decreases the number of B-cells, which causes an immunosuppressive state. This can promote the growth of numerous rare and opportunistic pathogens, one of which is .
View Article and Find Full Text PDFRheumatol Int
December 2024
Department of Nephrology and Clinical Immunology, RWTH Aachen University Hospital, Pauwelsstraße 30, 52074, Aachen, Germany.
To explore disease characteristics, renal involvement and induction treatment strategies over the last decades and evaluate relapse rates and renal outcomes in ANCA-associated vasculitides (AAV). We retrospectively analyzed remission, relapse rates and the occurrence of the composite endpoint (comprising death and renal failure) in newly diagnosed AAV cases in four tertial referral centers in Germany and Switzerland diagnosed between 1999 and 2022. Hazard ratios were computed by Cox proportional hazard and Kaplan-Meier curves were plotted to compare therapeutic strategies after propensity-matching.
View Article and Find Full Text PDFMod Rheumatol
December 2024
Department of Nephrology and Rheumatology, Graduate School of Medical Science, Kanazawa University.
Objective: To assess the prevalence and outcomes among regimens of glucocorticoid tapering for microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) in real-world practice.
Methods: We retrospectively examined the Japan Collaborative Registry of ANCA-associated Vasculitis (J-CANVAS) registry, and evaluated the prevalence of glucocorticoid tapering regimens in the PEXIVAS trial. In patients with newly diagnosed MPA and GPA, we compared outcomes among standard and reduced pace regimens.
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