A male case of Sjögren's syndrome presenting with generalized lymphadenopathy and swelling of both lacrimal and salivary glands which responded remarkably well to an administration of corticosteroids

Abstract

A 68-year-old man noticed severe oral dryness and a submandibular swelling. Sjögren's syndrome (SS) was diagnosed based on microscopic findings of a labial salivary gland biopsy although both anti-SS-A and anti-SS-B antibodies were negative. In addition, hypergammaglobulinemia (IgG 7940 mg/dl) and hypocomplementemia were pointed out and he was admitted to our department. On admission cervical, mediastinal, and abdominal lymph nodes swelling were detected together with enlargement of lacrimal and salivary glands. Lymphoproliferative disorders associated with SS were highly suspected. Biopsied specimens of his lacrimal gland and cervical lymph node disclosed neither malignant cells nor monoclonal proliferation of lymphocytes. An administration of corticosteroids caused rapid diminution in size of lacrimal glands, salivary glands, and lymph nodes. Both lacrimation and salivation recovered, and hypergammaglobulinemia and hypocomplementemia returned to normal after treatment. The characteristics of this case were an atypical onset in an elderly man, the negativity of anti-SS-A and anti-SS-B antibodies, and reversibility of dryness by corticosteroid treatment. These findings suggest that the pathogenesis of this case may be different from typical SS.