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.

Pregnancy-associated thrombotic thrombocytopenic purpura complicated by Sjögren’s syndrome and non-neutralising antibodies to ADAMTS13: a case report

“…Given that there were no other predisposing factors for TTP in this patient, the cause of TTP was considered tobe closely related to her pregnancy and pSS. To the best of our knowledge, only three cases of aTTP combined with pSS have been reported in the past 20 years, and none were associated with pregnancy [ 24 – 26 ]. Our case emphasises the importance of screening for collagen disease during pregnancy, including pSS, even if the patient is asymptomatic.…”

Thrombotic thrombocytopenic purpura with Graves’ disease during pregnancy

“…Patients with an autoimmune disorder are at risk for additional autoimmune diseases, resulting in various combinations including immune-hemolytic anemia, 3 systemic lupus erythematosus, 4 Sj€ ogren syndrome, 5 systemic sclerosis, 6 and others. However, we found only six reported cases of concomitant TTP and GD (Table 1), [7][8][9][10][11][12] although several mechanisms have been proposed in which GD may be causally related to TTP.…”

Sjögren's syndrome presenting as thrombotic thrombocytopenic purpura in a male patient with previous Kikuchi-Fujimoto disease

“…Steroids were discontinued at 6 months, and after one year the patient is off medication, without signs of TTP or SS extraglandular activity. TTP can co-occur with autoimmune diseases (AID) (1). A review identified 18 cases of concomitant SS and TTP (16 in primary SS) (2).…”