IgG4-related disease in autoimmune lymphoproliferative syndrome

Publication date: Available online 3 May 2017
Source:Clinical Immunology
Author(s): Annick A.J.M. van de Ven, Maximilian Seidl, Vanessa Drendel, Annette Schmitt-Graeff, Reinhard E. Voll, Anne Rensing-Ehl, Carsten Speckmann, Stephan Ehl, Klaus Warnatz, Florian Kollert
A patient with autoimmune lymphoproliferative disorder (ALPS) developed IgG4-related disease. In retrospect, he had high levels of serum IgG4 for several years prior to presenting with IgG4-related pancreatitis. These high IgG4 levels were masked by hypergammaglobulinemia, a common feature of ALPS. We next screened 18 ALPS patients; four of them displayed increased levels of IgG4. Hence, IgG4-related disease should be considered in ALPS patients, especially in those manifesting lymphocytic organ infiltration or excessive hypergammaglobulinaemia. Screening of IgG4-related disease patients for ALPS-associated mutations would provide further information on whether this disease could be a late-onset atypical presentation of ALPS.



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