Abstract
Patients with SSc have the presence of several autoantibodies, some of which are SSc-specific antibodies associated with the clinical features of SSc (9-13). The presence of anti-topoisomerase I (topo-I) antibody is associated with the development of skin fibrosis and progressive ILD in SSc. Anti-centromere antibody (ACA) is seen in patients with limited skin disease and is a minor risk factor for the development of ILD. However, it remains unclear whether SSc-specific antibodies are associated with pulmonary function and radiological findings of HRCT. We divided SSc patients into 5 clusters on the basis of SSc-specific autoantibodies present and retrospectively evaluated the clinical and radiological features of ILD among these 5 SSc patient clusters. Abstract Background: Patients with systemic sclerosis have the presence of several autoantibodies, some of which are systemic sclerosis-specific autoantibodies that are related to the clinical features of this disease. We retrospectively evaluated the clinical and radiological features of systemic sclerosis in patients with interstitial lung disease on the basis of these autoantibodies. Method: This study comprised 226 patients with systemic sclerosis. We divided patients with systemic sclerosis into 5 clusters based on the systemic sclerosis-specific autoantibodies present and compared the clinical and radiological features of interstitial lung disease among these 5 systemic sclerosis clusters. Results: Of 226 patients, interstitial lung disease was present in 73 (32.3%) patients. Clustering by Ward cluster analysis subsequently identified 5 major clusters of patients. Clusters of patients with anti-topoisomerase I antibody and without systemic sclerosis-specific autoantibodies had a higher incidence of interstitial lung disease compared to the other clusters (75.0% and 51.9%, respectively). Patients in these two clusters had higher percentages of cough and dyspnea, higher incidence of traction bronchiectasis and honeycomb-like cysts in computed tomography, and a decrease in percentage predicted of vital capacity. In contrast, patients with anti-centromere antibody had few clinical and laboratory findings and the lowest incidence of interstitial lung disease (16.8%). Patients with anti- ribonucleic antibody or nucleolar pattern in anti-nuclear antibody had moderate findings among the 5 clusters. Conclusion: The results showed that there were differences in clinical and radiological findings among patients with systemic sclerosis-specific autoantibodies.
Highlights
Systemic sclerosis (SSc) is a collagen vascular disease characterized by mircovascular damage and excessive fibrosis of the skin and internal organs including the lungs [1]
The results showed that there were differences in clinical and radiological findings among patients with systemic sclerosis-specific autoantibodies
To reveal the distribution of interstitial lung disease (ILD) in SSc, the number of patients with the presence of ILD was calculated in each block of the lung (Table 2)
Summary
Systemic sclerosis (SSc) is a collagen vascular disease characterized by mircovascular damage and excessive fibrosis of the skin and internal organs including the lungs [1]. Survival from SSc has improved over the past two decades because angiotensin converting enzyme inhibitors became available to treat renal crisis [2,3]. Lung involvement, such as interstitial lung disease (ILD) and pulmonary hypertension, is the major cause of death in SSc [4,5,6]. Steen et al reported that only 15-20% of SSc patients developed severe restrictive lung disease [11] This finding implies that the progression of pulmonary fibrosis is much slower or ceases in surviving patients. We retrospectively evaluated the clinical and radiological features of systemic sclerosis in patients with interstitial lung disease on the basis of these autoantibodies
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