Abstract
Background Systemic lupus erythematosus (SLE) is a rare complex autoimmune disease with a multisystem involvement [1]. Although pulmonary involvement is relatively frequent in adult patients; it has been rarely reported in children with SLE. It may be an initial and/or life-threatening complication [2]. There is a scarcity of published literature on pulmonary function tests (PFTs) in patients with JSLE manifestations of pulmonary disease. Objectives To verify a subclinical pulmonary disease in juvenile onset SLE using laboratory parameters, pulmonary functions tests and multislice CT Methods Twenty five patients with JSLE were studied. All fulfilled updated American College of Rheumatology criteria for SLE [3]. All patients had the disease at or before the age of 16 years. Assessment of disease activity was done through SLE Disease Activity Index (SLEDAI) [4]. PFTs were done using (ZAN 100 Flow Handy II) pulmonary function apparatus. PFTs were done according to Grippi and Tino [5]. They included forced expiratory volume in 1 s as FEV1 (liter/min) and forced expiratory flow rate FEV1% of predicted FEV1, forced vital capacity FVC, residual volume RV, total lung capacity TLC, FEF 25-75%, FEF50% and FEF75%, and diffusion capacity of carbon monoxide DLCO. Abnormal PFTs were categorized into restrictive, obstructive, mixed restrictive, and obstructive, and small airway disease. Laboratory and immunological parameters were done for ANA, anti-dsDNA, ENA including Sm, and La, U1-snRNP, C3, C4, RF, Acl and LA. Plain radiography for chest and multislice CT were done. Results Twelve patients (48%) had abnormal results of PFTs, 5 (20%) had an isolated (DLCO) impairment and 6 (24%) had a restrictive pattern. Neurological lupus was significantly associated with decreased FEF25-75% (P<0.04) and FEF75% (P<0.001) respectively. Radiography was normal. Abnormal PFTs were not significantly associated with other parameters. Multislice CT examination of JSLE patients demonstrated a pleural effusion and thickening in 16%, also ground glass opacities found in 16% suggesting early interstitial lung disease. Conclusions A mandatory performance of pulmonary function tests have to be done early in JSLE, without findings of pulmonary involvement. References Torre O, Harari S. Pleural and pulmonary involvement in SLE Presse Med 2011;40 1 Pt 2):e19–29. Diane L, Kamen MD, Strange C. Pulmonary Manifestations of SLE Clin Chest Med 2010;31:479–88.2004; 43:587–91. Hochberg MC. Diagnostic and therapeutic criteria committee of the American college of rheumatology updating the American college of rheumatology revised criteria for the classification of SLE. Arthritis Rheum 1997; 40:1725. Bombardier C, Gladman DD, Urowitz MB, Caron D, Chang CH. Derivation of the SLEDAIA disease activity index for lupus patients. The committee on prognosis studies in SLE. ArthritisRheum 1992;35:630–40. Grippi MA, Tino G. Pulmonary function testing. In: Fishman AP, Elias JA, Fishman JA, Grippi MA, Senior RM, Pack AI, editors. Fishman’s pulmonary diseases and disorders. New York: McGraw-Hill Professional; 2008. p. 567–610, Chapter 34. Disclosure of Interest None Declared