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.