Background/Purpose: Thrombotic microangiopathy (TTP) is a rare disease involving multiple organ systems. It is caused by extensive platelet thrombosis in the terminal arterioles and anterior capillaries, leading to clinical manifestations of microvascular hemolysis, thrombocytopenia, and organ dysfunction. Syndrome. TTP may be secondary to infection, malignancy, drugs, pregnancy, and autoimmune diseases. When TTP occurs in SLE patients, similar clinical symptoms may appear in TTP and SLE, and the symptoms in the early stages of the onset are lack of specificity, which mask each other and lead to delayed diagnosis. Although the incidence of SLE with TTP is not high, but the disease is serious, rapid progress, the fatality rate as high as 33.9-62.5%. This study investigated the clinical and laboratory characteristics, diagnosis, treatment, and prognosis of children with systemic lupus erythematosus (SLE) complicated with thrombotic microangiopathy (TTP).

Methods: Twenty-five children with SLE combined with TTP (SLE-TTP) were selected and their clinical symptoms, laboratory tests, renal pathological features, SLE disease activity indicators and treatment outcomes were analyzed retrospectively.

Results: 25 children with SLE-TTP, including 8 males (32%) and 17 females (68%). The onset age was 9-18 years, with a median age of 14 years. Among them, 9 cases were SLE first (25%), 1 case was TTP first (4%), and 15 cases were concurrent (60%). The mean SLE disease activity score (SLEDAI) was 22.4 points. Of the 25 children with SLE-TTP, all had thrombocytopenia, microvascular anemia, systemic symptoms in 21 cases (84%), cutaneous symptoms in 10 cases (40%), and digestive symptoms in 10 cases (40%). Nine cases had edema (36%), neurological symptoms in 9 cases (36%), respiratory symptoms in 6 cases (24%), joint pain in 5 cases (20%), and renal impairment in 19 cases (76%). Ten of these children completed a renal biopsy and found 8 pathological lesions of the kidney. Which type IV and TMA each accounted for 20% of renal pathological lesions. The most common treatment for children with SLE-TTP is immunosuppressive therapy with glucocorticoid pulse therapy (13 cases, 52%), followed by plasma exchange combined with glucocorticoid pulse therapy and immunosuppressive agents (10 cases, 40%). A total of 11 patients had plasma exchange (44%) with an average of 8.0 times. More than 80% of patients used plasma exchange and glucocorticoid therapy, and all patients achieved remission. Among the 25 cases, one case died (4%), and the remaining 15 cases (60%) were followed up at regular clinics without relapse.

Conclusion: SLE-TTP is often the first or concurrent SLE event, accompanied by middle-to-severe lupus activity. The main clinical manifestations of systemic symptoms in children with SLE-TTP included neurological symptoms, edema, and gastrointestinal symptoms. Renal pathology was mainly wolf kidney type IV or IV+TMA (40%). Severe SLE-TTP infection is an important risk factor for SLE-TTP. More than 92% of the patients were treated with immunosuppressants and glucocorticoids, and 40% of them needed plasmapheresis for effective remission.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-characteristics-and-treatment-of-25-children-with-systemic-lupus-erythematosus-complicated-with-thrombotic-microangiopathy-in-china/