Gastrointestinal involvement in granulomatosis with polyangiitis and microscopic polyangiitis: histological features
Joerg Latus1, Ina Koetter, Peter Fritz
1Division of Nephrology, Department of Internal Medicine, Robert-Bosch Hospital, Stuttgart, Germany.
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 CD20, CD3 and CD34. 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 CD3 in this patient compared to the control group (P = 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.
Conclusions:
GI involvement in GPA and MPA is rare. Therapy directed at T cells might be an alternative treatment option.
Insights
Gastrointestinal (GI) involvement is rare in granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). T-cell targeted therapy may be a potential treatment option for these rare cases.
Area of Science:
- Rheumatology
- Gastroenterology
- Immunology
Background:
- Gastrointestinal (GI) involvement is an uncommon manifestation in patients diagnosed with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA).
- Understanding the clinical presentation and underlying pathology of GI involvement is crucial for effective management.
Purpose of the Study:
- To investigate the clinical characteristics, outcomes, and diagnostic findings of GI involvement in GPA and MPA.
- To analyze the cellular composition of inflammatory infiltrates in GI tissues to identify potential therapeutic targets.
Main Methods:
- Retrospective review of medical charts for seven patients with GPA/MPA and GI involvement.
- Immunohistochemical analysis of GI tissue samples (ileum, colon, rectum, duodenum) for CD20, CD3, and CD34 expression.
- Comparison of patient samples with a healthy control group (n=15).
Main Results:
- GI symptoms were present in 71% of patients at diagnosis, often with high disease activity (mean BVAS 26.3).
- Abdominal pain was universal; 57% had acute kidney injury, and 71% required surgery.
- Histological analysis revealed significantly higher CD3 expression in a fatal case, suggesting T-cell involvement.
Conclusions:
- Gastrointestinal involvement in GPA and MPA is rare but can be severe.
- Therapeutic strategies targeting T-cells may represent a promising treatment avenue for GI manifestations in these vasculitides.
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