An Unusual Presentation of Granulomatosis with Polyangiitis
Authors
Abstract:
The granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, is a pauci-immune vasculitis involving small- and medium-sized blood vessels. Classically, the patients inflicted with this disease present with a triad of necrotizing granulomatous inflammation of the respiratory tract, cutaneous necrotizing vasculitis, and glomerulonephritis (GN). The antinuclear (ANA) and anti-neutrophil cytoplasmic antibodies (ANCA) are considered as relatively specific markers of the disease. Herein, we presented the case of a patient who initially presented with sepsis but did not respond to the conventional antibiotics and later diagnosed with GPA.The granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, is a pauci-immune vasculitis involving small- and medium-sized blood vessels. Classically, the patients inflicted with this disease present with a triad of necrotizing granulomatous inflammation of the respiratory tract, cutaneous necrotizing vasculitis, and glomerulonephritis (GN).The antinuclear (ANA) and anti-neutrophil cytoplasmic antibodies (ANCA) are considered as relatively specific markers of the disease. Herein, we presented the case of a patient who initially presented with sepsis but did not respond to the conventional antibiotics and later diagnosed with GPA.
similar resources
An Unusual Presentation of Limited Granulomatosis with Polyangiitis Involving Vagina and Urethra
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 dur...
full textAn unusual renal presentation of Wegener's granulomatosis.
A 63-year-old woman was admitted to our clinic with arthralgia, microhematuria and a pleural based solid mass in lower lobe basal segment of right lung seen in computerised tomogram of thorax. She was diagnosed as Wegener's granulomatosis by histopathological findings of right thoracotomy, wedge resection and decortication. The patient had positive antineutrophil cytoplasmic antibodies (c-ANCA)...
full textAcute laryngeal dyspnea as first presentation of granulomatosis with polyangiitis.
Granulomatosis with polyangiitis (GPA) is a multi-organ disease which mostly affects lungs, kidney, and head and neck region. We report a rare case of acute laryngeal dyspnea and rapidly progressive pulmonary changes as first manifestations of disease. A 53 year-old woman presented with symptoms of two-week dyspnea, which aggravated rapidly in the preceding hours. Laryngological examination rev...
full textA Presentation of Cerebritis Secondary to Granulomatosis with Polyangiitis (Wegener)
Neurological manifestations of GPA are common, most frequently as a peripheral neuropathy. Cerebritis as a principal presentation is extremely rare. We report a patient who presented with subacute progression of ataxia, confusion, and vacant episodes. An MRI of her brain showed bilateral signal abnormalities in the cingulate and superior sagittal gyrus while a staging CT revealed a mass in the ...
full textRetroperitoneal fibrosis with periaortitis: A case report of an unusual form of presentation of granulomatosis with polyangiitis
Granulomatosis with polyangiitis (GPA) is the name that has been used in recent years for Wegener's granulomatosis. This condition is a systemic inflammatory disease characterised by necrotizing vasculitis that affects small and medium-sized blood vessels (capillaries, arterioles, venules and arteries). The granulomatous inflammation affects the respiratory system; it also commonly affects the ...
full textGranulomatosis With Polyangiitis (Wegener’s)
To determine outcomes in relation to duration of maintenance therapy in patients with granulomatosis with polyangiitis (Wegener’s) (GPA), we conducted a retrospective chart review of patients with GPA seen at a single vasculitis center from 1992 to 2010. All patients achieved remission defined by a Birmingham Vasculitis Activity Score for Wegener Granulomatosis (BVAS/WG) of 0 with either cyclop...
full textMy Resources
Journal title
volume 5 issue 3
pages 204- 207
publication date 2017-09-01
By following a journal you will be notified via email when a new issue of this journal is published.
Hosted on Doprax cloud platform doprax.com
copyright © 2015-2023