Pulmonary thromboembolism in children with rheumatic diseases

Abstract

Purpose: To demonstrate the clinical features and predisposing factors of pulmonary thrombotic events in children with rheumatic diseases. Methods: Chart review, observational. Results: Thrombotic events have been associated with antiphospholipid antibodies in autoimmune diseases, including systemic lupus erythematosus (SLE). However, pulmonary thromboembolism (PTE) from deep vein thromboses (DVT) or in situ pulmonary arterial thrombosis is uncommon in rheumatic diseases, especially in children. The diagnosis and treatment of PTE may be delayed due to a paucity of symptoms or to symptoms attributed to more common manifestations such as pleuritis or pneumonia. We report findings in 6 children with PTE secondary to SLE (4), Systemic Sclerosis (SSc) (1) and Polyarteritis Nodosa (PAN) (1). Conclusion: Although antiphospholipid antibodies are common in SLE, pulmonary arterial thrombosis is rare. These 4 cases of SLE represent only 1.7% of 234 pediatric lupus patients seen at Emory University over 18 years. Pulmonary thromboemboli may mimic pleuritis with effusion or pneumonia. Besides antiphospholipid antibodies, which were present in only 2 of these patients, other associated findings include nephrotic syndrome, elevated D-dimers and elevated fibrinogen levels. Recognition of PTE in pediatric patients with rheumatic diseases and prompt anticoagulation therapy is important and potentially life-saving.