Pulmonary involvement in children with rheumatic diseases

Background: The data on prevalence and type of pulmonary involvement in children with rheumatic diseases is scarce.Lung injury may be caused by the disease itself or by the biologic/nonbiologic disease modifying anti-rheumatic drugs(DMARDs). Aims and objectives: Our aim was to assess pulmonary functional status in children with connective tissue diseases(CTDs) and to assess the effect of DMARDs in children with rheumatic diseases. Methods: Children with a diagnosis of CTD or receiving DMARDs for more than 2 years were included into this study.Medical history, physical examination, chest radiology and pulmonary function tests were evaluated. Results: There were 27 children with CTDs(19 with JDM, 1 polymyositis, 3 SLE, 3 systemic sclerosis, 1 mixed CTD).CO diffusion capacity was low in 2 patients and spirometry showed restrictive changes.HRCT was normal in 1 patient with polymyositis.The second patient(JDM) who was positive for anti-MDA5 autoantibody had features suggesting interstitial lung disease(ILD) in HRCT. There were 50 children who received biologic/nonbiologic DMARDs(23methotrexate, 13etanercept, 4adalimumab, 4anakinra, 3tocilizumab, 1canakinumab, 1abatacept, 1infliximab) for more than 2 years.During the routine evaluation for tuberculosis in 27 patients receiving biologics, 12(%44.5) received isoniazid prophylaxis for 9 months.However, no active tuberculosis was diagnosed.Only 2 patients had ILD who were using anakinra for ≥2 years for the treatment of systemic juvenile idiopathic arthritis. Conclusions: Pulmonary disease especially ILD have been associated with several rheumatic diseases; thus the routine follow-up of pulmonary functional status is crucial to diagnose and treat pulmonary disease in the early phases.