Abstract
Background Pulmonary fibrosis / intersitial lung disease (ILD) in systemic sclerosis (SSc) is a complicated restrictive pulmonary disease and the leading cause of disease-related mortality. Progressive skin fibrosis in diffuse-type SSc (dSSc) is associated with decreased forced vital capacity (FVC). Modified Rodnan Skin Score (mRSS) examination is used as a parameter to assess skin fibrosis, while high-resolution computed tomography (HRCT) and pulmonary function tests (PFTs) are used to assess pulmonary fibrosis. The HRCT test remains as the gold standard in diagnosing ILD. However, it costs a lot and is not available in all healthcare facilities. Krebs Von den Lungen-6 (KL-6) is a biomarker to evaluate pulmonary fibrosis. The aim of this study was to analyze the correlation of serum KL-6 levels with FVC and mRSS value of patients with restrictive lung disease in dSSc.
 Method This was a cross-sectional study that used primary data from dSSc patients who visited rheumatology outpatient clinic in Hasan Sadikin Hospital Bandung, Indonesia, during the period of June-July 2019. History taking, physical examination, mRSS, spirometry, and serum KL-6 levels were performed. Data were analyzed using the Rank Spearman correlation test. 
 Result There were 27 subjects with the mean age of 42 ± 12 years. Based on FVC (%) restrictive lung disease criteria, the majority of subjects (74.1%) had severe restrictive lung disease and the rest of all subjects (25.9%) were non severe restrictive lung disease. Serum KL-6 levels ranged from 0.545 to 8.138 ng/ml. The results showed that there was no correlation between serum KL-6 levels and FVC values (r = -0.118, p = 0.279) and mRSS (r = 0.101, p = 0.312 ).
 Conclusion There is no correlation between serum KL-6 levels with FVC and mRSS value of patient with restritive lung disease in diffuse type systemic sclerosis.
 Keywords : diffuse type systemic sclerosis, Forced Vital Capacity, KL-6, mRSS, restrictive lung disease.
 
 
Highlights
Systemic sclerosis (SSc) is a chronic and progressive autoimmune disease hallmarked by the distinctive triad of microvascular damage, dysregulation of innate and adaptive immunity, and most importantly, generalized fibrosis in multiple organs.[1]
Based on autopsy series performed in systemic sclerosis (SSc) patients, pulmonary fibrosis was found in 90 percent of cases.[6]
There were 27 subjects included in this study and all of them were female with the mean age of 42 years old
Summary
Systemic sclerosis (SSc) is a chronic and progressive autoimmune disease hallmarked by the distinctive triad of microvascular damage, dysregulation of innate and adaptive immunity, and most importantly, generalized fibrosis in multiple organs.[1]. High Resolution Computed Tomography (HRCT) scanning remains as the standard and the most sensitive method in diagnosing ILD.[7,8] HRCT is not available in all healthcare facilities and not affordable for most patients.[9] pulmonary function test (PFT) is commonly used to screen and evaluate long term pulmonary function. Decreased forced vital capacity (FVC) determines the severity level of restrictive lung disorder. Pulmonary fibrosis / intersitial lung disease (ILD) in systemic sclerosis (SSc) is a complicated restrictive pulmonary disease and the leading cause of disease-related mortality. Progressive skin fibrosis in diffuse-type SSc (dSSc) is associated with decreased forced vital capacity (FVC). The HRCT test remains as the gold standard in diagnosing ILD It costs a lot and is not available in all healthcare facilities. The aim of this study was to analyze the correlation of serum KL-6 levels with FVC and mRSS value of patients with restrictive lung disease in dSSc
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