IgG4-Related Disease: A Multispecialty Condition

Abstract EN

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.