Five cases of systemic lupus erythematosus are diagnosed by thrombopenia

Abstract

The prevalence of thrombocytopenia has been reported 7 to 30％ in patients with systemic lupus erythematosus（ SLE） and less than 15％ of patients have thrombocytopenia as a first symptom of SLE. The most common cause of secondary immune thrombocytopenic purpura （ITP） is SLE. Herein, we report 5 cases with childhood-onset SLE （cSLE） diagnosed following by thrombocytopenia. Four of the five cases were girls and the age at disease onset was 5-14 years （median : 11 years of age）. Skin rash on the lower leg was recognized as the first manifestation in 2 cases. Thrombocytopenia was accidentally noticed by examination for other diseases in the other 3 cases. All cases showed refractory thrombocytopenia and referred to our hospital. The platelet count at the first visit of our hospital was as low as 1,000 to 93,000/μL. All patients showed high anti-nuclear antibody titer and met the classification criteria for cSLE. The range of SLE disease activity index score was 7 to 14, which indicated high disease activity. Four cases showed positive for antiphospholipid antibody, and 2 met the diagnostic criteria for antiphospholipid syndrome （APS）. The intravenous glucocorticoid therapy or methylprednisolone pulse therapy was used for all patients, and platelet count and disease activity of SLE were gradually improved. We concluded that patients with refractory ITP should be performed an autoantibody testing for the possibility of SLE and APS.