Purpose
To demonstrate the clinical features and predisposing factors of pulmonary thrombotic events in children with rheumatic diseases.
Methods
Chart review, observational.
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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).
Table 1
Pt/Gender | 1/F | 2/F | 3/M | 4/F | 5/M | 6/M |
|---|---|---|---|---|---|---|
Dx | SLE | SLE | SLE | SLE | SSc | PAN |
thrombocytopenia | nephritis (IV) | nephritis (V) | nephritis (IV) | PAH | CVA | |
Age at Dx (yr) | 12.6 | 14.1 | 9.0 | 12.0 | 12.8 | 0.3 |
Age at PTE | 15.2 | 14.8 | 16.8 | 12.6 | 16 | 6/4 |
Symptoms | leg pain | chest pain | chest pain | chest pain | chest pain | leg pain |
dyspnea | dyspnea | dyspnea | dyspnea | |||
DVT | + | - | - | - | - | + |
Lupus AC | + | - | - | - | - | +/- |
Anticardio AB | - | - | - | - | - | + |
D-dimer (ng/ml) | 647 (nl <220) | 8770 | 1600 | >10,000 | n/a | >10,000 |
Albumin (g/dl) | 4.7 (nl 3.7-5.5) | 2.0 | 0.7 | 1.7 | 4.1 | 3.6 |
AT III (%) | 105 (nl 77-132) | 278 | 7/2 | 154 | n/a | 114 |
Fibrinogen (ng/dl) | 718 (nl 180-394) | 234 | n/a | 298 | n/a | 421 |
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 anti-coagulation therapy is important and potentially life-saving.
Disclosure
Larry B. Vogler: None; Sheila Angeles-Han: None; Sampath Prahalad: None; Egla C. Rabinovich: None.
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.