الفهرس 
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Abstract
Pulmonary Manifestations in Systemic Lupus Erythematosus
Background: The respiratory system is commonly involved in systemic lupus erythematosus (SLE). Lung disorders are classified as primary (due to lupus) and secondary to other conditions. Pleuritis and pulmonary infections are the most prevalent respiratory manifestations of each type. Other infrequent manifestations include interstitial lung disease, acute lupus pneumonitis, diffuse alveolar haemorrhage, pulmonary arterial hypertension, acute reversible hypoxaemia and shrinking lung syndrome. In terms of lung involvement, only few studies are available, suggesting that some serum markers can be elevated in blood and exhaled breath condensate of SLE patients with pulmonary manifestations. The role of chemokine ligand 21 (CCL21) and interferon gamma induced protein 10 (IP-10) and their association to pulmonary involvement in SLE were not widely investigated. Even when current diagnostic tests contribute to an earlier diagnosis, the treatment of these manifestations is still based on clinical experience and small series.
Aim of the work: The specific objectives of this study are evaluating the frequency of lung involvement, diagnosed clinically and by imaging techniques, among both symptomatic and asymptomatic SLE patients, correlating SLE disease parameters with different pulmonary manifestations and assessing the levels of CCL21 and IP-10 and evaluating them as specific biomarkers for pulmonary involvement in SLE and assessing their impact on the quality of life (Qol).
Methods: Sixty patients (56 females, 4 males) who fulfilled the 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria were included in the present study. Thirty healthy age and sex matched individuals served as a control group. All patients were subjected to full history, clinical examination, laboratory investigations including CCL21 and IP10, pulmonary function tests and imaging techniques (Chest X ray (CXR) and High resolution computed tomography (HRCT)). Patients were classified according to pulmonary involvement (clinical and/or pulmonary function tests (PFTs), HRCT) and compared as regards demographic, clinical and serum biomarkers levels. Disease activity and Lupus Qol. were also assessed and correlated to demographic and clinical data.
Results: Pleuro-pulmonary involvement was reported in 43 (71.7%) of the SLE patients, among them, 38 (63.3%) were diagnosed as chronic interstitial pulmonary fibrosis, pleural involvement was found in 34 (56.7%) with pleural effusion in 5 (8.3%) of them, pulmonary hypertension was found in 16 (26.7) patients, 4 (6.7%) were diagnosed as acute lupus pneumonitis and 1 (1.7%) was diagnosed as shrinking lung syndrome. Patients with SLE and pulmonary involvement had higher serum CCL21 and IP10 levels compared to those without pulmonary involvement who in turn had higher levels than the controls with statistically significant differences (p<0.001). There were strong significant negative correlations between CCL21 and IP10 and FEV1st, FVC, DLCO with (p<0.001). Concerning HRCT score, there were strong significant positive correlations between it and CCL21 and IP10 with (p<0.001) for both. Modified medical research council (MMRC) dyspnea scale could be reported as the best determinant of pulmonary involvement as detected by HRCT in SLE patients and also was found to be the best predictor for worse Qol.
Conclusion: About two thirds of SLE patients develop pleuro-pulmonary manifestations at some point during the disease course. CCL21 and IP10 can be identified as specific serum biomarkers to detect pulmonary involvement in SLE with high sensitivity and specificity. Both are significantly associated with worse PFTs and imaging scores on HRCT. MMRC dyspnea scale can predict pulmonary involvement in SLE and a worse Qol.
Key words: Systemic lupus erythematosus, pleuropulmonary involvement, pulmonary vascular involvement, respiratory infection, pulmonary fibrosis, CCL21, IP10, quality of life.