Immune-mediated thrombotic thrombocytopenic purpura with systemic lupus erythematosus: clinical features and outcome.

objectiveThe association of immune-mediated thrombotic thrombocytopenic purpura (iTTP) and SLE was previously described, but patients with iTTP with coexistent SLE remain poorly characterised.

methodsWe compared the clinical presentation and the outcome of patients with iTTP with coexistent SLE (SLE-iTTP) to an age-sex matched cohort of patients with idiopathic iTTP without SLE.

resultsDuring the study period, 1409 patients with iTTP were recruited in our registry. Of these, 79 (6%) had a prior or concurrent diagnosis of SLE at the time of iTTP diagnosis, and 437 (31%) had detectable ANAs without other clinical features of SLE. When compared with idiopathic iTTP, patients with SLE-iTTP had more severe renal involvement, and cardiac involvement was more prevalent, whereas central nervous system involvement was less common. Patients with SLE-iTTP received more immunosuppressive agents. There was no difference in response categories during the acute phase. During follow-up, SLE-iTTP had superior ADAMTS13 relapse-free survival than idiopathic iTTP. Among patients with ANAs without other clinical features of SLE, 33 (8%) were diagnosed with SLE 27 months (IQR: 7-65 months) following iTTP diagnosis; 32 additional patients (7%) developed another systemic autoimmune disease. No patient from the idiopathic iTTP group developed clinical SLE during follow-up.

conclusionPatients with iTTP are prone to develop autoimmune features, and patients with SLE-iTTP have distinct clinical features and outcome. Relapse-free survival seems better in patients with SLE-iTTP, underscoring the need for tailored management strategies in this population, including a specific follow-up to assess early features suggestive of SLE. TRIAL REGISTRATION NUMBER: NCT00426686.