Portal de Periódicos da CAPES

IgG4-Related Systemic Disease

2011; Wolters Kluwer; Volume: 91; Issue: 1 Linguagem: Inglês

10.1097/md.0b013e3182433d77

1536-5964

Mikaël Ebbo, Laurent Daniel, Michel Pavic, P. Sève, M. Hamidou, Emmanuel Andrès, Stéphane Burtey, L. Chiche, Jacques Serratrice, M. Longy‐Boursier, M. Ruivard, Julien Haroche, Bertrand Godeau, Anne-Bérengère Beucher, Jean‐Marie Berthelot, T. Papo, Jean‐Loup Pennaforte, A. Benyamine, N. Jourde, C. Landron, P. Roblot, Olivier Moranne, Christine Silvain, B. Granel, F. Bernard, V. Veit, K. Mazodier, E. Bernit, H Rousset, José Boucraut, Jean‐Jacques Boffa, Pierre‐Jean Weiller, Gilles Kaplanski, Pièrre Aucouturier, Jean‐Robert Harlé, N. Schleinitz,

Gastrointestinal disorders and treatments

IgG4-related systemic disease is now recognized as a systemic disease that may affect various organs. The diagnosis is usually made in patients who present with elevated IgG4 in serum and tissue infiltration of diseased organs by numerous IgG4+ plasma cells, in the absence of validated diagnosis criteria. We report the clinical, laboratory, and histologic characteristics of 25 patients from a French nationwide cohort. We also report the treatment outcome and show that despite the efficacy of corticosteroids, a second-line treatment is frequently necessary. The clinical findings in our patients are not different from the results of previous reports from Eastern countries. Our laboratory and histologic findings, however, suggest, at least in some patients, a more broad polyclonal B cell activation than the skewed IgG4 switch previously reported. These observations strongly suggest the implication of a T-cell dependent B-cell polyclonal activation in IgG4-related systemic disease, probably at least in part under the control of T helper follicular cells.