Gastrointestinal involvement in granulomatosis with polyangiitis and microscopic polyangiitis: histological features and outcome. (29th October 2013)

• Record Type: Journal Article
• Authors: Latus, Joerg; Koetter, Ina; Fritz, Peter; Kimmel, Martin; Biegger, Dagmar; Ott, German; Stange, Eduard F.; Amann, Kerstin; Alscher, Dominik M.; Braun, Niko
• Is Part Of: International journal of rheumatic diseases. Volume 17:Number 4(2014)
• Page Start: 412
• Page End: 419
• Publication Date: 2013-10-29
• Subjects: Rheumatology -- Periodicals; Rheumatology -- Asia -- Periodicals; Rheumatology -- Pacific Area -- Periodicals; Rheumatic Diseases -- Periodicals; Connective Tissue Diseases -- Periodicals; Immune System Diseases -- Periodicals; 616.723
• DOI: 10.1111/1756-185X.12203
• Languages: English
• ISSNs: 1756-1841

Abstract

<abstract abstract-type="main" id="apl12203-abs-0001"> <title>Abstract</title> <sec id="apl12203-sec-0001" sec-type="section"> <title>Aim</title> <p>Gastrointestinal (GI) involvement in patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) is rare.</p> </sec> <sec id="apl12203-sec-0002" sec-type="section"> <title>Method</title> <p>Medical charts of seven patients with GPA and MPA and GI involvement were reviewed regarding clinical presentation, outcome, diagnostic tools and therapy. Second, the cellular composition of the inflammatory infiltrate associated with the vascular lesions in histological samples (ileum, colon, rectum, duodenum) were investigated to identify possible treatment targets. Immunohistochemistry was done with antibodies against CD20, CD3 and CD34. Samples from a healthy control group (<italic>n</italic> = 15) were used for comparison.</p> </sec> <sec id="apl12203-sec-0003" sec-type="section"> <title>Results</title> <p>Mean age at onset of the first symptoms of vasculitis was 48 ± 21.3 years. At time of diagnosis GI symptoms were present in five out of seven patients (71%) and occurred during relapse of the vasculitis in two patients (29%). All patients had abdominal pain, four of seven (57%) had an acute kidney injury and three patients required renal replacement therapy. At the time of diagnosis five of seven patients (71%) required surgery and mean Birmingham Vasculitis Activity Score (BVAS) on admission was high (26.3 ± 7.7). Regarding outcome, one patient died due to gastrointestinal bleeding. Histological analysis showed significantly higher expression of CD3 in this patient compared to the control group (<italic>P</italic> = 0.02). Analysis of expression of CD20 and CD34 showed no statistically significant differences between patients with GPA and MPA with GI involvement compared to the control group.</p> </sec> <sec id="apl12203-sec-0004" sec-type="section"> <title>Conclusions</title> <p>GI involvement in GPA and MPA is rare. Therapy directed at T cells might be an alternative treatment option.</p> </sec> </abstract>