Pediatric patients with systemic lupus erythematosus (SLE) associated with thrombotic thrombocytopenic purpura (TTP) frequently have moderate to severe lupus disease activity, and combination therapy of glucocorticoids with an immunosuppressive agent can provide satisfactory clinical outcomes, according to results from a study published in the Italian Journal of Pediatrics.
Researchers retrospectively assessed 25 pediatric patients with TTP-SLE regarding disease activity, renal biopsy, clinical symptoms, and laboratory findings.
The median age of patients was 14 years. SLE as the first diagnosis occurred in 9 patients, and 15 patients were diagnosed concurrently with TTP and SLE. All patients had reduced platelet count and microangiopathic hemolytic anemia.
The primary clinical symptoms were rash, neurological symptoms, fever, and edema. Increased lactate dehydrogenase (LDH) and fragmentation of red blood cells on blood smear occurred in all patients.
More than three-quarters of patients (76%) had impaired renal function. Results from renal biopsy showed lupus nephritis class 4 in 20% of patients and thrombotic microangiopathy in 20% of patients.
Over half of patients (52%) received combination therapy comprising glucocorticoids with an immunosuppressive agent, and 40% of patients underwent plasma exchange with glucocorticoids plus an immunosuppressive agent.
One patient died from a lung infection. Some patients experienced remission. In the 15 patients that underwent regular follow-up, disease was stable.
Researchers stated that testing LDH levels and assessing blood smears should occur when renal and neurological symptoms occur in pediatric patients with SLE. Patients with refractory TTP-SLE will require plasma exchange therapy.
The authors noted that limitations of the study included the retrospective design, small sample size, and some missing laboratory and follow-up data. Because of these limitations, the authors explained that the results and conclusion might not be comprehensive but noted, “we believe the data reported in this study could provide useful reference for clinical practice.”
1. Li J, Jiang JJ, Wang CY, et al. Clinical features and prognosis of patients with thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus: a review of 25 cases [published online April 29, 2019]. Ital J Pediatr. doi:10.1186/s13052-019-0641