Sjögren's syndrome associated with multiple myeloma.

There have been very few reported cases of multiple myeloma (MM) which had Sjögren syndrome (SS) as the first presentation. We report a 63-year-old Moroccan woman with IgA-lambda-type MM presenting as SS and who responded to anti-myeloma treatment. The patient, treated for SS, was admitted to our department for persistent and increasing thoracic pain. Clinical examination was normal. Laboratory investigations showed haemoglobin of 10 g/dL. Erythrocyte sedimentation rate was 80 mm/hr. Monoclonal spike was found in the betaglobulin region of the serum protein electrophoresis. Immunofixation identified it as IgA lambda and the level was 3.7 g/dL. The bone marrow contained 35 percent plasma cells, with atypical features. Radiographs showed diffuse lytic lesions. Treatment with vincristine, adriamycin and dexamethasone (VAD) was started and bisphosphonate was administered regularly. After three cycles of VAD therapy, the MM regressed without any evidence of SS symptoms. The development of MM in the setting of SS is unusual and the aetiopathogenic mechanism still unknown. However, some elements orient toward a common pathway for these two diseases, like the clinical remission of SS after treatment of the MM, such as described in our patient.