IgG4 related disease (IgG4-RD) is a rare and relatively new group of systemic inflammatory diseases characterized by inflammatory, fibrotic or sclerotic involvement of one or more organs accompanied by increased IgG4 plasma cells tissue infiltration and usually elevated serum IgG4 (IgG4 > 1.35g/l, normal range 0.08-1.40 g/l) level. Histopathological findings are crucial for the diagnostics of this disease.
The authors present a case report of a patient with IgG4 associated disease manifested by a rare combination of autoimmune hemolytic anemia and pulmonary involvement.