title: IgG4-Related Disease: A Multispecialty Condition.
abstract: 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.
authors.affiliation: Serviço de Clínica Médica do Hospital Geral Roberto Santos, Rua Direta do Saboeiro s/n, Cabula, 41180-900 Salvador, BA, Brazil.
authors.forename: Iuri
authors.identifier: 0000-0002-4002-7094
authors.initials: I
authors.lastname: Usêda Santana
authors.affiliation: Serviços Especializados em Reumatologia (SER) da Bahia, Rua Conde Filho 117, Graça, 40150-150 Salvador, BA, Brazil.
authors.forename: Emanuela Pimenta
authors.initials: EP
authors.lastname: da Fonseca
authors.affiliation: Serviços Especializados em Reumatologia (SER) da Bahia, Rua Conde Filho 117, Graça, 40150-150 Salvador, BA, Brazil.
authors.forename: Mittermayer Barreto
authors.initials: MB
authors.lastname: Santiago