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Gastrointestinal involvement in granulomatosis with polyangiitis and microscopic polyangiitis: histological features and outcome
Author(s) -
Latus Joerg,
Koetter Ina,
Fritz Peter,
Kimmel Martin,
Biegger Dagmar,
Ott German,
Stange Eduard F.,
Amann Kerstin,
Alscher Dominik M.,
Braun Niko
Publication year - 2014
Publication title -
international journal of rheumatic diseases
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.795
H-Index - 41
eISSN - 1756-185X
pISSN - 1756-1841
DOI - 10.1111/1756-185x.12203
Subject(s) - medicine , microscopic polyangiitis , granulomatosis with polyangiitis , vasculitis , gastroenterology , duodenum , abdominal pain , immunohistochemistry , ileum , pathology , disease
Abstract Aim Gastrointestinal ( GI ) involvement in patients with granulomatosis with polyangiitis ( GPA ) or microscopic polyangiitis ( MPA ) is rare. Method 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 CD 20, CD 3 and CD 34. Samples from a healthy control group ( n = 15) were used for comparison. Results 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 CD 3 in this patient compared to the control group ( P = 0.02). Analysis of expression of CD 20 and CD 34 showed no statistically significant differences between patients with GPA and MPA with GI involvement compared to the control group. Conclusions GI involvement in GPA and MPA is rare. Therapy directed at T cells might be an alternative treatment option.