Background/Purpose: Gastrointestinal (GI) involvement during adult IgA vasculitis (IgAV) occurs in roughly half the cases. In many other systemic vasculitides, GI involvement represents a poor-prognosis factor, leading to the use of immunosuppressive agents combined glucocorticoids. This study was undertaken to describe the first manifestations and prognoses of GI involvement in adult IgAV patients.

Methods:

This nationwide, retrospective study analyzed the data from 260 adult IgAV patients to describe and compare the initial symptoms and outcomes of those with GI involvement (GI+) vs. those without it (GI–).

Results: Among the 260 patients analyzed, 137 (53%) were GI+ vs. 123 (47%) GI–. The initial GI manifestations included: abdominal pain for 135/137 (99%), intestinal bleeding for 43/137 (31%) and diarrhea for 36/137 (26%). Abdominal computed-tomography (CT) scans and/or ultrasonography were obtained for 65% of GI+ patients. The most frequent imaging finding was thickening of the intestinal wall (61%). Upper and/or lower GI endoscopies of 78 (57%) patients revealed abnormalities in 77%, including mucosal ulceration (53%), erythema (34%) and/or purpura (26%). In contrast, intestinal biopsies were obtained from only 8 (6%) patients. The most frequent histological finding was nonspecific inflammatory infiltrates seen in all biopsies, but specific vasculitis features were rarely described (e.g., fibrinoid necrosis seen in only 1). At diagnosis, GI+ patients vs. GI– patients, respectively, were younger (mean±SD age 46.3±1.5 vs. 54.5±1.7 years; P=0.0004), had more constitutional symptoms (43% vs. 23%; P=0.0005), more frequent joint manifestations (72% vs. 50%; P=0.0002) and higher C-reactive protein (3.7 vs. 1.9 mg/dL; P=0.001). Renal involvement frequency and severity were similar for the 2 groups. Finally, GI+ patients vs. GI– patients, respectively, had comparable overall clinical response rates (80% vs. 76%; P=0.53); minor (12% vs. 13%; P=0.91) or major relapse rates (6% vs. 9%, P=0.54); renal outcomes (1 transplanted vs. 1%, P=1.00; 4 on dialysis vs. 2%, P=0.42); and vasculitis-related mortality (2% vs. 3%; P=0.68). None of the cohort patients had life-threatening GI manifestations at diagnosis, but 2 patients developed GI perforations 1 and 3 months after starting glucocorticoids.

Conclusion: GI involvement is a frequent manifestation of adult IgAV but does not seem to negatively impact overall prognosis. Therapeutic management should not necessarily include immunosuppressive agents as first-line therapy, as for systemic necrotizing vasculitis, but more data are needed to confirm these findings.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/gastrointestinal-involvement-in-adult-iga-vasculitis-henoch-schonlein-purpura-initial-manifestations-and-outcomes/