Thrombotic Thrombocytopenic Purpura Complicating Secondary Sjögren's Syndrome: A Rare and Serious Association from Africa

Abstract

Thrombotic Thrombocytopenic Purpura is a potentially fatal disease that can be life-threatening. The association with Sjogren’s syndrome is rare entity that deserves special attention. To this end, we report the case of a Thrombotic thrombocytopenic purpura complicating secondary Sjögren's syndrome. This is a 49-year-old patient with a history of pure nephrotic syndrome with minimal glomerular lesions (LGM), who developed Sjögren's syndrome two years later. The diagnosis of Sjögren's syndrome was retained with an etiological investigation suggesting SS secondary to SLE systemic lupus erythematosus. Six months after the diagnosis of secondary SS, the patient was admitted with headache, ecchymotic and petechial lesions on the upper limbs. The laboratory analysis revealed severe thrombocytopenia, haemolytic anemia with a schizocyte count at 6% supporting the diagnosis of thrombotic microangiopathy. The ADAMTS 13 activity assay was less than 5% and the anti-ADAMTS 13 antibody test was positive, attesting thus, the diagnosis of acquired TTP complicating SS secondary to SLE. The patient received an emergency blood transfusion of fresh frozen plasma combined with corticosteroid therapy and mycophenolate mofetil. The clinico-biological outcome, at 6 month and 1 year, was favorable with complete remission. To the best of our knowledge, this is the first case report of TTP complicating Sjögren’s syndrome in Africa. It highlights the rarity of association between autoimmune disease particulary Sjögren’s syndrome and TTP.