A 25-year-old female had complaints of low grade fever and back pain and her chest X ray film revealed the bilateral hilar lymphoadenopathy (BHL). She was diagnosed as sarcoidosis from the histrogical findings of biopsied paraaortic lymph nodes.
The serum IgA level was less than 5 mg/d
l, but serum IgG and IgM slightly increased and secretory component in salivary and duodenal juice was normal.
The percentage of ERFC of her peripheral blood lymphocytes (PBL) was 67.7%, and membrane immuno-fluoresence technique revealed 29.8% s-IgG bearing cells, 26.1% s-IgA bearing cells and 20.6% s-IgM bearing cells. In her bone marrow smear and duodenal mucosa specimen, IgA containing cells were not found. Antinuclear antibodies, circulating immune complex and anti-lymphocytic antibody were negative.
Amounts of IgA synthesized by PWM-stimulated PBL from this patient were measured by radioimmunoassay, and the level of IgA was about a half of normal synthesis.
In this patient, we suppose that the hyperfuction of the class-specific suppressor cells or the defect in T helper function may cause the abnormality preventing the differentiation of IgA bearing B lymphocytes into IgA secreting plasma cells.
This patient was administered prednisolone (PSL). About 6 months later her BHL disappered, and serum IgA was begining to increase a year after of begining treatment with PSL. So, this suggests that selective IgA deficiency had closely related with sarcoidosis in this patient.
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