Abstract

ARGUELLES ZAYA, Ana; INFANTE AMOROS, Adalberto; CHONG LOPEZ, Agustín  and  CHAO PEREIRA, Caridad. IgG4-related disease. Rev cubana med [online]. 2018, vol.57, n.3, pp. 38-42. ISSN 1561-302X.

IgG4 - related disease is characterized by inflammatory fibrosis accompanied by elevated IgG4 serum concentrations. Clinically it can show involvement of one or multiple organs. It has been considered that this disease is secondary to the interaction between Th2 cells and B cells, which results in high concentrations of IgG4 and inflammatory mediators subsequent to lymphoplasmacytic infiltrates and fibrosis. However, it is not the only mechanism involved. For the diagnosis, clinical, serological, histopathological and immunohistochemical criteria are required. Good response to steroid treatment has been observed. We report the case of a 30-year-old woman with recurrent pleural effusion, interstitial lung disease, impaired progressive renal function, and elevation of parathyroid hormone and calcium in the blood. The studies revealed high serum levels of IgG4, lung and kidney biopsy consistent with IgG4-related disease. The patient was treated with 40 mg prednisone a day with excellent response.

Keywords : IgG4; fibrosis; IgG4-related disease.

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