Gastrointestinal involvement in patients with vasculitis: IgA vasculitis and eosinophilic granulomatosis with polyangiitis

Abstract

Background and study aims Among vasculitides, IgA vasculitis (IgAV) and eosinophilic granulomatosis with polyangiitis (EGPA) frequently damage the gastrointestinal tract. However, only a few studies have investigated the entire gastrointestinal tract in patients with IgAV or EGPA by endoscopy. The aim of this study was to clarify endoscopic characteristics of patients with IgAV and those with EGPA. Patients and methods Clinicopathological and endoscopic findings were retrospectively compared between 33 patients with IgAV and 19 patients with EGPA. Results Gastrointestinal involvement was observed in 33 patients with IgAV (100 %) and in 8 patients with EPGA (42 %; P = 0.0001). Duodenal involvement was more frequent in patients with IgAV (75.8 %) than in those with EGPA (21.1 %, P = 0.0002). Jejunoileal involvement was frequent in both groups (IgAV 94.4 %; EGPA 77.8 %). Gastric mucosal erythema was more frequent in patients with IgAV (18.2 %) than in those with EGPA (0 %, P = 0.0481). Duodenal mucosal erythema (IgAV 54.6 %; EGPA 21.1 %, P = 0.0227), ulcer (IgAV 33.3 %; EGPA 0 %, P = 0.0041), and hematoma-like protrusion (IgAV 21.1 %; EGPA 0 %, P = 0.039) were more frequently observed in patients with IgAV than in those with EGPA. Conclusions Frequent duodenal involvement, gastric mucosal erythema, and duodenal lesions including erythema, ulcer, and hematoma-like protrusion are characteristic of patients with IgAV. Because jejunoileal involvement was frequent in both groups of patients, small-bowel endoscopies should be performed for diagnosis of small-bowel lesions in patients with IgAV and EGPA.

Upper gastrointestinal ischemia as a rare complication of paroxysmal nocturnal hemoglobinuria

“…It seems difficult to make a diagnosis of PNH by endoscopic biopsy; nonetheless, gastrointestinal biopsy may be beneficial in differentiating PNH from IgA vasculitis, which can present with both gastric patchy redness and duodenal ulcer. 19,20 Treatment with corticosteroids, proton-pump inhibitors, and diet control was successful in our patient. Various treatments have been reported for PNH-related thrombosis.…”

“…The patchy areas of gastric redness present in our patient have not been reported so far. It seems difficult to make a diagnosis of PNH by endoscopic biopsy; nonetheless, gastrointestinal biopsy may be beneficial in differentiating PNH from IgA vasculitis, which can present with both gastric patchy redness and duodenal ulcer 19,20 …”

Gastrointestinal involvement and its association with the risk for nephritis in IgA vasculitis

“…Second line treatments, such as the use of mycophenolate mofetil, intravenous cyclophosphamide or intravenous immunoglobulin, as well as endoscopic haemostasis, were not used since all patients with GI bleeding favourably responded to therapy with glucocorticoids, similar to many other studies. 41 Although glucocorticoids have been shown to be very successful in treating our patients with severe gastrointestinal journals.sagepub.com/home/tab 11 manifestations, they have not influenced the risk of developing late-onset IgAVN, and similar results have been shown in controlled studies, therefore prophylactic use of glucocorticoids in IgAVN prevention is not indicated. 40 In patients who were treated with glucocorticoids because of GI manifestations we have observed higher chance for early-onset IgAVN, probably due to the previously explained association between GI symptoms and renal disease, in that more pronounced inflammatory response results in more severe GI manifestations and, consequently, by still insufficiently elucidated mechanisms, it also leads to increased local inflammation in the kidneys.…”

Gastrointestinal Hemorrhage With Gastritis and Pancolitis as the Sole Presentation for Granulomatosis With Polyangiitis Flare

“…14 Although some studies have explored endoscopic findings of GI disease in other vasculitides, because of the rarity of GPA in the GI tract, most of the work in this area has focused on the widely varying GI manifestations of GPA. 15 Currently, neither the American College of Rheumatology nor the American College of Gastroenterology has guidelines on the endoscopic diagnosis or medical management of GI manifestations of GPA. 16 Our patient's young diagnosis age, ANCA negativity, and GI manifestations are unusual for GPA.…”