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Case Reports in Rheumatology
Volume 2014, Article ID 723493, 3 pages
Case Report

IgG4-Related Disease: A Multispecialty Condition

1Serviço de Clínica Médica do Hospital Geral Roberto Santos, Rua Direta do Saboeiro s/n, Cabula, 41180-900 Salvador, BA, Brazil
2Serviços Especializados em Reumatologia (SER) da Bahia, Rua Conde Filho 117, Graça, 40150-150 Salvador, BA, Brazil

Received 15 September 2014; Revised 13 November 2014; Accepted 14 November 2014; Published 24 November 2014

Academic Editor: Mario Salazar-Paramo

Copyright © 2014 Iuri Usêda Santana et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


IgG4-related disease (IgG4-RD) is a recently recognized group of conditions, characterized by tumor-like swelling of involved organs, lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, variable degrees of fibrosis, and elevated serum IgG4 concentrations. Currently IgG4-RD is recognized as a systemic condition that can affect several organs and tissues. Herein we report the case of a 34-year-old male patient who was admitted to our hospital with diffuse abdominal pain, weight loss, and painful stiffness in his neck. He had a history of tumoral mass of the left maxillary region, right palpebral ptosis with protrusion of the eyeball, and chronic dry cough for about 6 years. Laboratory tests revealed polyclonal hypergammaglobulinemia and increased serum IgG4 levels. Immunohistochemical staining of the maxillary biopsy was compatible with IgG4-RD. He had an excellent response to corticosteroid therapy. This case highlights that IgG4-RD should be included in the differential diagnosis with multisystem diseases.