Introduction: Wegener's granulomatosis presenting as diffuse alveolar hemorrhage is uncommon. However, the recognition of multisystem disease involving joints, kidney, eye and lung is critical for diagnosing Wegener's vasculitis. This is not the first report of this kind in the literature.
Case Presentation: A 51-year-old Croatian woman presented to our Emergency Department with a history of progressively worsening productive cough and shortness of breath, epistaxis and two episodes of hemoptysis. She developed respiratory failure due to diffuse alveolar hemorrhage, which was successfully treated with high-dose steroids, cyclophosphamide and plasmapheresis. Her clinical course was complicated with methicillin-resistant Staphyloccocus aureus pneumonia, which has been associated with Wegener's granulomatosis flares.
Conclusion: The recognition of multisystem disease is critical for diagnosing Wegener's vasculitis. Diffuse alveolar hemorrhage can be a fulminant manifestation of Wegener's granulomatosis, in which case immediate and aggressive treatment with pulse steroids, high-dose cyclophosphamide and plasma exchange can be life-saving.
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http://dx.doi.org/10.1186/1752-1947-6-335 | DOI Listing |
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.
SAGE Open Med Case Rep
December 2024
Department of Pediatrics, Faculty of Medicine & Dentistry, University of Alberta, Edmonton, AB, Canada.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic necrotizing vasculitis marked by eosinophilia and extravascular granulomas, predominantly affecting the respiratory tract. This report details a unique EGPA case in a 6-year-old girl with extensive cardiac involvement, featuring an atypical intracardiac mass suggestive of endomyocardial fibrosis and a concomitant thrombus. The clinical course unfolded in three phases: an initial prodrome with asthma; subsequent peripheral hypereosinophilia; and ultimately systemic vasculitis.
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