Immunoglobulin A Enteropathy: A Possible Variant of Henoch–Schönlein Purpura

Abstract

This study calls attention to a new syndrome presenting with gastrointestinal symptoms including abdominal pain, vomiting, and/or hematemesis and endoscopic multiple lesions predominantly in the descending duodenum, without the skin rash observed in Henoch-Schonlein purpura. We examined the gastrointestinal mucosa for IgA deposits in nine children and compared the results with those for three patients with Henoch-Schönlein purpura. In addition, gastroduodenal biopsy specimens of 11 patients with various diseases were studied as controls for IgA staining. Intestinal histology showed nonspecific mucosal inflammation without vasculitis. In six patients without rash (67%), IgA deposition was observed in the capillary wall with the same staining pattern as seen in two patients with Henoch-Schonlein purpura. Compared with the controls (9%), the positive rate of IgA deposition was significantly higher in nonrash patients (P < 0.01). Deposited IgA showed immunoreactivities of polymeric IgAl containing J chain. IgA deposits were ultrastructually seen along the plasma membranes of the endothelial cells. Overall, the data suggest that IgA deposition played a pathogenetic role in the gastrointestinal damage in this group of patients presenting primarily with gastrointestinal complaints. Further studies are needed to clarify whether this patient population has a variant of Henoch-Schönlein purpura or a distinct "IgA enteropathy."

A Case of IgA-related Enteropathy Complicated with Gastrointestinal Bleeding and Progressive IgA Nephropathy: A Possible Variant Henoch-Sch&ouml;nlein Purpura?

“…On this issue, we propose the clinical entity of systemic vasculitis caused by IgA deposition and that HSP may be a part of this clinical entity with skin lesions. Consistent with our proposal, Kato et al proposed a new syndrome characterized as IgA enteropathy including abdominal pain and multiple endoscopic lesions, predominantly in the descending duodenum without palpable purpura (8). They examined the gastroduodenal biopsy samples of nine patients without any purpura and found IgA deposition in the capillary wall of the intestinal mucosa in six pa- An g i o g r a p h y s h o we d t h e e x t r a v a s a t i o n i n t h e i l e u m b r a n c h o f t h e s u p e r i o r me s e n t e r i c a r t e r y ( a r r o w, A) a n d i t wa s ma r k e d b y t h e c o i l ( a r r o w, B ) .…”

Gastrointestinal Manifestations of Henoch-Schonlein Purpura

“…Several authors suggest that IgA nephropathy is in fact HSP without a rash. There may also exist an IgA enteropathy lacking the characteristic rash of HSP [5].…”

IgA Anticardiolipin in Patients with Gastroenteric Tumor

“…In a variant of Henoch-Schonlein purpura, characterized by abdominal pain, vomiting, hematemesis, and endoscopic multiple lesions, there is an IgA deposit along the plasma membranes of the enteric endothelial cells [24]. These data clearly indicate the importance of IgA in gastroenteric system.…”