血小板減少症とＩｇＡ腎症を合併した原発性シェーグレン症候群の１例

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  • Report of a patient of primary Sjoegren syndrome, IgA nephropathy and chronic idiopathic thrombocytopenic purpura.

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We describe the case of a 61-year-old woman diagnosed with primary Sjögren's syndrome (SS) after an 8-year history of IgA nephropathy and a 3-year history of recurrent purpuric rashes. Her two daughters had previously been diagnosed with other autoimmune diseases. One daughter had Graves'disease and the other had Hashimoto's disease and systemic lupus erythematosus. The diagnosis of SS was made based on dryness of mucous membranes, Shirmer test, and parotid sialography. Thrombocytopenia, high platelet-aggregated IgG (PA-IgG) level, and normal megakaryocytes count in bone marrow suggested that her recurrent purpuric rashes were due to idiopathic thrombocytopenic purpura (ITP). Patients with SS may develop other autoimmune diseases. This case aids understanding of the immune pathogenesis and genetic background of SS.

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