A case of Sjögren's syndrome with marked lacrimal gland enlargement, atypical onset and IgA-M-proteinemia

Abstract

Since July, 1999, a 66 year-old man had been complaining of dry cough. He noticed submandibular swelling, lacrimal gland enlargement and dry eye. Keratoconjuctivitis sicca was detected by an ophthalmologist. Sjögren's syndrome was diagnosed based on microscopic findings of a labial salivary gland biopsy although anti-SS-A and anti-SS-B antibodies were negative. Hypergammaglobulinemia (IgG 3916 mg/dl) and IgA-M-proteinemia were pointed out. Swelling of mediastinal and abdominal lymph nodes was detected together with enlargement of salivary and lacrimal glands. We suspected the existence of malignant lymphoma, but a biopsy of lacrimal glands showed only lymphocytic and plasma cell infiltration and immunohistochemical analysis denied monoclonality of lymphoid line. An administration of corticosteroids caused rapid diminution in size of lacrimal and submandibular glands and lymph nodes. Clinical symptoms were also improved, but IgA-M proteinemia remains. The characteristics of our case were enlargement of lacrimal glands, the negativity of anti SS-A and SS-B antibodies, atypical onset and M-proteinemia. We discussed about these characteristics of Sjögren's syndrome in our case.