Pulmonary involvement: A potential independent factor for quality of life in systemic lupus erythematosus.

BACKGROUND: Systemic lupus erythematosus (SLE) is a chronic multi-systemic autoimmune disease. SLE patients may experience a wide range of physical, psychological, and social perception of well-being influenced by the patient illness that are not always fully captured by descriptions of the disease's physiological consequences alone. Nowadays, patients with SLE have a better survival than decades ago, nevertheless still experience a low health related quality of life (HRQoL). Assessing disease activity in SLE is crucial to the physician as it forms the basis for treatment decisions, moreover careful evaluation for respiratory involvement should be routinely considered. More chronic lung disease related to SLE can have a significant negative effect on patient well-being and physical performance status and are detrimental to quality of life. OBJECTIVE: The aim of this study was to evaluate quality of life changes in SLE patients using Lupus QoL scale, assessing their correlation with different disease aspects particularly pulmonary manifestations and predictors for worse QoL. MATERIALS AND METHODS: Total of 60 SLE patients, who fulfilled the 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria, were enrolled in this study. Disease activity was measured by systemic lupus erythematosus disease activity index (SLEDAI) and quality of life was assessed by Lupus QoL. Pulmonary evaluation included pulmonary function tests parameters (PFTs), mMRC dyspnea scale, HRCT score, and pulmonary damage index. RESULTS: Lupus QoL had a strong significant correlations with PFTs FEV1, FVC, and DLCO (r = 0.79, 0.78, 0.76, p < .001), respectively}, while Lupus QoL had strong negative correlations with both mMRC dyspnea scale and HRCT score (r = -0.96, -0.85, p < .001), respectively, and moderate negative correlation with neuropsychiatric lupus (NPSLE) (r = -0.61, p < .001). Weak negative correlations were found between Lupus QoL, photosensitivity, alopecia, Raynaud's and renal affection (r = -0.29, -0.30, -0.30, 0.38, p = .03, .02, .02, .002), respectively. NPSLE and pulmonary involvement were the most consistent predictors of low HRQoL [contributing 36% and 18% of the variance of Lupus QoL], respectively. CONCLUSION: Lupus QoL is negatively correlated with different SLE clinical parameters particularly pulmonary manifestations. Neuropsychiatric, pulmonary, renal affection, and SLEDAI are the best determinants for worse Lupus QoL.

CCL21 and IP10 as serum biomarkers for pulmonary involvement in systemic lupus erythematosus.

BACKGROUND: Although the significance of inflammatory cytokines and chemokines in the pathogenesis of SLE is well established, the findings showed diversity and implied that combining different biomarkers could be useful in monitoring disease activity or organ involvement. Despite the potentially high prevalence of lung involvement in SLE, only a few studies have investigated for lung biomarkers. OBJECTIVE: The aim of this study was to assess the value of Chemokine Ligand 21 (CCL 21) and Interferon gamma-induced protein 10 (IP10) as serum biomarkers for pulmonary involvement in SLE and their correlation with disease activity, organ involvement, pulmonary function tests (PFTs), and chest CT findings. MATERIALS AND METHODS: Sixty SLE patients and 30 age- and sex-matched controls were enrolled into this study. All patients underwent serological tests, PFTs, and chest CT examination. The serum levels of CCL21 and IP10 were analyzed, and their correlations with PFTs and CT were explored. RESULTS: SLE patients with pulmonary involvement had higher serum CCL21 and IP10 levels compared to those without pulmonary involvement which in turn had higher levels than the controls. There were strong negative correlations between CCL21 and IP10 and FEV1, FVC, and DLCO. There were also strong correlations between both biomarkers and HRCT and pulmonary damage, but no correlation with other disease manifestations. Serum level of 2095 pg/mL for CCL21 and 7185 pg/mL for IP10 could detect pulmonary involvement in SLE with a sensitivity of 83.7% and a specificity of 94.1%. Both biomarkers performed equally well in detecting SLE pulmonary involvement with a strong agreement between them (κ = 0.86, p < .001), but CCL21 was better correlated with PFT abnormalities. CONCLUSION: Both CCL21 and IP10 are serum biomarkers to detect pulmonary involvement in SLE with high sensitivity and specificity. CCL21 correlates better with PFT abnormalities.

Asymptomatic pulmonary hypertension in systemic lupus erythematosus.

INTRODUCTION: Pulmonary arterial hypertension (PAH) is a serious and often fatal complication of systemic lupus erythematosus (SLE). Because the diagnosis of PAH often is made years after symptom onset, early diagnostic strategies are essential. Doppler echocardiography currently is considered the noninvasive screening test of choice for evaluating pulmonary hypertension. AIM: Screening for asymptomatic pulmonary hypertension in systemic lupus erythematosus patients using Doppler echocardiography, and correlating it with inflammatory parameters of the disease. PATIENTS AND METHODS: Doppler echocardiography was performed in 74 patients with systemic lupus erythematosus over one year (66 adult and 8 juvenile), adult SLE included 57 patients with adult-onset and 9 patients with childhood-onset. Pulmonary hypertension was diagnosed if the peak systolic pressure gradient at the tricuspid valve was more than 30 mmHg. All patients were subjected to full history taking, rheumatological examination, laboratory studies and chest x-ray. RESULTS: In seventy four SLE patients, the pulmonary hypertension was detected in 8 patients (10.8%), 7 adult-onset SLE patients (aged from 19 to 30 years) and 1 juvenile SLE (aged 12 years). The range of pulmonary artery systolic pressure was 34-61.2 mmHg (43.19 ± 9.28). No significant differences between patients with and those without pulmonary hypertension as regard clinical features. Significantly higher frequencies of rheumatoid factor and anti-cardiolipin antibodies were found in patients with pulmonary hypertension versus those without (P = 0.02, P = 0.008 respectively). Positive rheumatoid factor and ACL were significantly associated with occurrence of PAH in SLE (P = 0.007, P = 0.006 respectively). No significant correlations were found between pulmonary artery pressure, disease duration, SLE Disease Activity Index (SLEDAI), ESR, and anti-ds DNA. CONCLUSION: Patients with SLE have an increased risk of pulmonary arterial hypertension. Echocardiography should be used as a screening tool in patients at high risk for development of pulmonary hypertension. Positive anti-cardiolipin antibodies and rheumatoid factor were significant predictors of pulmonary hypertension in our study.