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–Schönlein 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–Schönlein 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.”
Similar content being viewed by others
References
Baart de la Faille-Kuyper EH, Kater L, Kooiker CJ, Dorhout Mees EJ: IgA-deposits in cutaneous blood-vessel walls and mesangium in Henoch-Schönlein syndrome. Lancet 1:892–893, 1973
Giangiacomo, J, Tsai CC: Dermal and glomerular deposition of IgA in anaphylactoid purpura. Am J Dis Child 131:981–983, 1977
Morichau-Beauchant M, Touchard G, Maire P: Jejunal IgA and C3 deposition in adult Henoch-Schönlein purpura with severe intestinal manifestations. Gastroenterology 82:1438–1442, 1982
Touchard G, Maire P, Beauchant M, Doeuvre P, Babin P, Pecheur H, Becq-Giraudon B, Matuchansky C: Vascular IgA and C3 deposition in gastrointestinal tract of patients with Henoch-Schönlein purpura. Lancet 1:771–772, 1983
Kato S, Shibuya H, Naganuma H, Nakagawa H: Gastrointestinal endoscopy in Henoch-Schönlein purpura. Eur J Pediatr 151:482–484, 1992
Kato S, Ebina K, Naganuma H, Sato S, Maisawa S, Nakagawa H: Intestinal IgA deposition in Henoch-Schönlein purpura with severe gastrointestinal manifestations. Eur J Pediatr 155:91–95, 1996
Banerjee B, Rashid S, Singh E, Moore J: Endoscopic findings in Henoch-Schönlein purpura. Gastrointest Endosc 37:569–571, 1996
Beck IT: Duodenitis. In Gastroenterology, 4th ed. WS Haubrich, MH Kalser, JLA Roth, F Schaffner (eds). Philadelphia, WB Saunders, 1985, pp 1403–1421
Suerbaum S, Michetti P: Helicobacter pylori infection. N Engl J Med 347:1175–1186, 1996
Gunasekaran TS, Berman J, Gonzalez M. Duodenojejunits: Is it idiopathic or is it Henoch-Schönlein purpura without the purpura. J Pediatr Gastroenterol Nutr 30:22–28, 2000
Fitzgerald JF: HSP—Without P? [Editorial]. J Pediatr Gastroenterol Nutr 30:5–7, 2000
Amano K, Tsukada K, Takeuchi T, Fukuda Y, Nagura H: IgA deposition in alcoholic liver disease. Am J Clin Pathol 89:728–734, 1988
Komatsu N, Nagura H, Watanabe K, Nomoto Y, Kobayashi K: Mesangial deposition of J chain-linked polymeric IgA in IgA nephropathy. Nephron 33:61–64, 1983
Lomax-Smith JD, Zabrowarny LA, Howarth GS, Seymour AE, Woodroffe AJ: The immunochemical characterization of mesangial IgA deposits. Am J Pathol 113:359–364, 1983
Conley ME, Cooper MD, Michael AF: Selective deposition of immunoglobulin A1 in immunoglobulin A nephropathy, anaphylactoid purpura nephritis, and systemic lupus erythematosus. J Clin Invest 66:1432–1436, 1980
Glasier CM, Siegel MJ, McAlister WH, Shackelford GD. Henoch-Schönlein syndrome in children: Gastrointestinal manifestations. AJR 136:1081–1085, 1980
Yoshiara S, Yoshikawa N, Matsuo T: Immunoelectron microscopic study of childhood IgA nephropathy and Henoch-Schönlein nephritis. Virchows Arch A 412:95–102, 1987
Tsai CC, Giangiacomo J, Zuckner J: Dermal IgA deposition in Henoch-Schönlein purpura and Berger’s nephritis. Lancet 1:342–343, 1975
Tomino Y, Endoh M, Nomoto Y, Sakai H: Immunoglobulin A1 in IgA nephropathy. N Engl J Med 305:1159–1160, 1981
Andre C, Berthoux FC, Andre F, Gillon J, Genin C, Sabatier JC: Prevalence of IgA2 deposits in IgA nephropathies. N Engl J Med 303:1343–1346, 1980
Walshe JJ, Brentjens JR, Costa GG, Andre GA, Venuto RC: Abdominal pain associated with IgA nephropathy. Am J Med 77:765–767, 1984
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Kato, S., Ozawa, K., Ando, N. et al. Immunoglobulin A Enteropathy: A Possible Variant of Henoch–Schönlein Purpura. Dig Dis Sci 49, 1777–1781 (2004). https://doi.org/10.1007/s10620-004-9569-0
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10620-004-9569-0