IgG4-Related Systemic Disease: Features and Treatment Response in a French Cohort: Results of a Multicenter Registry.
Ebbo, Mikael MD; Daniel, Laurent MD, PhD; Pavic, Michel MD; Seve, Pascal MD, PhD; Hamidou, Mohamed MD, PhD; Andres, Emmanuel MD; Burtey, Stephane MD, PhD; Chiche, Laurent MD; Serratrice, Jacques MD, PhD; Longy-Boursier, Maite MD, PhD; Ruivard, Marc MD, PhD; Haroche, Julien MD, PhD; Godeau, Bertrand MD; Beucher, Anne-Berengere MD; Berthelot, Jean-Marie MD; Papo, Thomas MD; Pennaforte, Jean-Loup MD; Benyamine, Audrey MD; Jourde, Noemie MD; Landron, Cedric MD; Roblot, Pascal MD, PhD; Moranne, Olivier MD, PhD; Silvain, Christine MD; Granel, Brigitte MD; Bernard, Fanny MD; Veit, Veronique MD; Mazodier, Karin MD; Bernit, Emmanuelle MD; Rousset, Hugues MD; Boucraut, Jose MD, PhD; Boffa, Jean-Jacques MD, PhD; Weiller, Pierre-Jean MD; Kaplanski, Gilles MD, PhD; Aucouturier, Pierre PhD; Harle, Jean-Robert MD; Schleinitz, Nicolas MD
91(1):49-56, January 2012.
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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.
(C) 2012 Lippincott Williams & Wilkins, Inc.