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Revista Brasileira de Reumatologia
Print version ISSN 0482-5004
BRAGA, Josefina Aparecida Pellegrini; HOKAZONO, Mary; TERRERI, Maria Teresa R. A. and HILARIO, Maria Odete E.. Idiopathic thrombocytopenic purpura as initial manifestation of juvenile systemic lupus erythematosus. Rev. Bras. Reumatol. [online]. 2003, vol.43, n.6, pp. 392-396. ISSN 0482-5004. http://dx.doi.org/10.1590/S0482-50042003000600013.
Patients with Idiopathic Thrombocytopenic Purpura (ITP) present a high trend to develop Systemic Lupus Erythematosus (SLE), especially those with chronic presentation. Some authors observed that female gender, older patients and familial history of autoimmune disease in patients with Idiopathic Thrombocytopenic Purpura are factors that lead to increased susceptibility for the development of Systemic Lupus Erythematosus. Based on these facts, we decided to study 5 children with chronic Idiopathic Thrombocytopenic Purpura and late Systemic Lupus Erythematosus. In this paper, we describe the clinical and laboratorial features of 5 female children with Idiopathic Thrombocytopenic Purpura that later developed Systemic Lupus Erythematosus. All patients were girls, 3 Caucasian, with Idiopathic Thrombocytopenic Purpura onset age ranged between 6 years and 3 months and 12 years and 1 month (mean - 9 years and 2 months). The age at Systemic Lupus Erythematosus diagnosis ranged between 8 years and 13 years and 8 months (mean - 10 years and 10 months). Though, the gap between Idiopathic Thrombocytopenic Purpura and Systemic Lupus Erythematosus diagnosis ranged between 11 months and 2 years and 9 months (mean - 1 year and 10 months). All patients presented chronic Idiopathic Thrombocytopenic Purpura (thrombocytopenia lasts longer than 6 months). The Systemic Lupus Erythematosus classification criteria were (in decreasing frequency): malar erythema and positivity of ANA in 5 patients; arthritis, hematological (thrombocytopenia) and immunological alterations (positivity of anti-DNA) in 4 patients; photosensitivity and positivity of anti-cardiolipin in 3 patients. Other manifestations included oral ulcers, renal involvement, leukopenia and auto-immune hemolytic anemia, serositis (pericarditis), neurological involvement and positivity of anti-Sm antibody. We would like to emphasize this form of presentation of Systemic Lupus Erythematosus, in which the Idiopathic Thrombocytopenic Purpura was the first manifestation and that the auto-antibody determination in every children with chronic form of this disease is very important.
Keywords : idiopathic thrombocytopenic purpura; systemic lupus erythematosus; thrombocytopenia; cardiolipin antibody; children.