Agranulocytosis in a patient with primary Sjögren's syndrome

Abstract

The authors describe a case of primary Sjögren's syndrome, which was complicated with severe autoimmune agranulocytosis quite sensitive to immunosuppressive therapy. Agranulocytosis is a very rare complication of this autoimmune rheumatic disease as opposed to leucopenia. A remarkable feature of the presented case is the fact that correct diagnosis of primary Sjögren's syndrome has not been settled for almost 25 years. The disease has manifested only in the form of arthropathy imitating rheumatoid arthritis.

Micofenolato mofetil na síndrome de Sjögren primária: uma opção para o tratamento da agranulocitose

“…1 Unexplained agranulocytosis has been rarely reported in patients with pSS. [2][3][4] Bone marrow neutrophil production may be affected, or neutrophils may be destroyed in the circulation, by both humoral and cellular immune-mediated mechanisms. The pSS associated agranulocytosis usually responds to steroids used alone or with immunosuppressive drugs.…”