Rheumatology: Current Research
Open Access

Abstract

Pulmonary Manifestations of Rheumatologic Diseases in Pediatric

Ingrid Herta Rotstein Grein, Christina Feitosa Pelajo, Thais Cugler Meneghetti, Loris Lady Janz Junior and Marcia Bandeira

Objectives: To describe the pulmonary manifestations in rheumatic diseases in pediatrics - systemic lupus erythematosus (SLE), juvenile dermatomyositis (JDM), systemic scleroderma (SSc), linear scleroderma (LS), and mixed connective tissue disease (MCTD)-and to correlate these findings with clinical manifestations, imaging and pulmonary function tests. Methods: A retrospective cross-sectional analysis was performed. We evaluated patients with rheumatic disorders followed at the Rheumatology Department of Hospital Pequeno Príncipe from January 2000 to July 2012. All patients diagnosed with SSc, as well as patients with SLE, JDM, MCTD and LS who had pulmonary symptoms or worsening of their underlying disease, were submitted to chest radiography (CXR), chest high resolution computed tomography (HRCT) and pulmonary function tests (PFTs). Results: A sample of 193 patients was obtained for this study. Thirty-eight percent of them were submitted to CXR and chest HRCT, with or without PFTs. Within all patients included in this study, 37% had some degree of pulmonary involvement, verified in at least one test. The groups who presented higher rates of pulmonary involvement were the SSc (100%) and the MCTD (46%). Patients with SLE and JDM had similar results, presenting pulmonary involvement in 35% of cases, and LS had the lowest rate of respiratory system involvement, with 25% of patients affected. In one quarter of the cases, the abnormalities found were due to infections. In the remaining three quarters pulmonary involvement was due to the underlying disease, and a large spectrum of manifestations was detected. Conclusions: Pulmonary involvement often occurs in systemic rheumatic diseases. There is a variety of manifestations in all rheumatic disorders, and there are no pathognomonic signs of pulmonary involvement in each disease. Health professionals should be aware of the possibility of pulmonary involvement in rheumatic disorders, in order to detect it early and initiate adequate treatment precociously, to improve the long term prognosis.