Thrombotic thrombocytopenic purpura with an autoantibody to ADAMTS13 complicating Sjögren’s syndrome: two cases and a literature review

Abstract

An association between thrombotic thrombocytopenic purpura (TTP) and Sjögren’s syndrome (SS) is rare. This is the first report of two patients with TTP who had inhibitory autoantibodies to ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type 1 repeats) complicating primary SS. A rapid diagnosis of TTP, which is a potentially lethal condition, made it possible to treat the two cases successfully. Only eight similar cases with TTP complicating SS have been reported in the literature. The possible presentation of primary SS without classic sicca symptoms, but with haematological abnormalities including TTP, should be recognised. Furthermore, it is important to measure ADAMTS13 activity and anti-ADAMTS13 antibodies, because TTP with SS seems to be a concurrent overlapping autoimmune disorder. We suggest that plasma exchanges in combination with corticosteroids should be administered as early as possible, since they appeared to be effective in treating TTP with SS, including in our cases.

Keywords::

• ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type 1 repeats)
• Glomerular nephritis
• Plasma exchanges
• Sjögren’s syndrome (SS)
• Thrombotic thrombocytopenic purpura (TTP)