Pleuropulmonary Manifestations in Juvenile Systemic Lupus Erythematosus; A Review and Descriptive Study in 64 Cases

Objective: Juvenile systemic lupus erythematosus (JSLE) is a multisystemic autoimmune disease that can involve multiple organs such as: skin, kidney, musculoskeletal system, brain, and others as well as lung. Pulmonary manifestations may be an initial and/or life-threatening complication of SLE in children. The aim of this report is to describe the first pleuropulmonary manifestation of childhood lupus erythematosus.Material Methods: We studied retrospectively 64 children with JSLE, diagnosed as JSLE at the Children s Medical Center Hospital between 1995 and 2005. All met the American College of Rheumatology (formerly American Rheumatism Association, ARA) revised criteria for SLE. They were evaluated for evidence of pleuro-pulmonary involvement.Findings: During the 10-yr study period, 64 patients were diagnosed as childhood-onset JSLE, who had the disease at or before the age of 16 (3-16 years). Fifty five patients (86%) were females and 9 patients (14%) were males (female: male ratio=6/1). Mean age of this group at the onset of the disease was 10 years (range 3-16). Eighteen cases (28%) had pulmonary involvement. Pulmonary complications include: infectious pneumonia in 38%, pleuritis in 33%, pulmonary vasculitis in 11%, and acute lupus pneumonitis, chronic interstitial pneumonitis and pulmonary embolism (so-called lupus anticoagulant) each one in 5.5%.Conclusion: The prevalence of pulmonary involvement in patients with JSLE varies according to the method used, but clinically significant pulmonary involvement in our series occurs in approximately 28%. There is few data regarding the treatment for most of the immune mediated pulmonary manifestations of JSLE.