Primary Sjogren syndrome with diffuse alveolar hemorrhage, cryoglobulinaemia and thrombotic microangiopathy

Sjogren syndrome is an autoimmune exocrinopathy with various extra glandular manifestations. Cryoglobulinemia, small vessel vasculitis has been reported in 5-10% of patients with primary Sjogren syndrome. It may be associated with a higher prevalence of extra glandular disease in Sjogren patients. Thrombotic microangiopathy (TMA), a potentially lethal condition characterized by microvascular thrombosis due to endothelial injury, is rare in SS. Its most characteristic histological finding is intravascular and intraglomerular fibrin thrombi. The association of Sjogren syndrome, cryoglobulinemia and thrombotic microangiopathy has not been previously reported. We report the first case of Sjogren syndrome with these three manifestations, occurring in combination.