scholarly journals Correlation between Serum Krebs von den Lungen-6 Levels with Forced Vital Capacity and Modified Rodnan Skin Score of Patients with Restrictive Lung Disease in Diffuse-Type Systemic Sclerosis

2019 ◽  
Vol 11 (2) ◽  
Author(s):  
Herlina Yani ◽  
Sumartini Dewi ◽  
Andri Reza Rahmadi
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Forced Vital Capacity ◽  
Vital Capacity ◽  
Skin Fibrosis ◽  
Diffuse Type ◽  
Skin Score ◽  
Restrictive Lung Disease ◽  
Modified Rodnan Skin Score

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.      

scholarly journals Correlation of sCD40L Level with Force Vital Capacity Value in Restrictive Lung Disease of Systemic Sclerosis Patients

2018 ◽  
Vol 10 (1) ◽  
Author(s):  
Susanto Salim ◽  
Rachmat Gunadi Wachjudi ◽  
Sumartini Dewi
Keyword(s):  
Systemic Sclerosis ◽  
Lung Disease ◽  
Forced Vital Capacity ◽  
Lung Fibrosis ◽  
Vital Capacity ◽  
High Resolution Computed Tomography ◽  
Cross Sectional ◽  
Restrictive Lung Disease ◽  
Potential Biomarker ◽  
Scd40l Level

Background: Interstitial Lung Disease (ILD) is one of the major cause of morbidity and mortality in Systemic Sclerosis (SSc). The gold standard to diagnose ILD is using High Resolution Computed Tomography (HRCT) scan. HRCT scan need a lot of cost and not always available, so another diagnosing test is needed as an alternative modality to diagnose ILD. ILD is a restrictive lung disease caused by lung fibrosis which is proved by the decrease of Forced Vital Capacity (FVC) in spirometry, and followed by the increase of soluble CD40L (sCD40L) level in plasma. This sCD40L may become a potential biomarker to evaluate lung fibrosis in SSc patients. The aim of this study is to analyze the correlation of sCD40L levels with FVC score in SSc patients with restrictive lung disease.Method:This cross sectional study was enrolled by the SSc patient who has restrictive lung disease based on spirometry test, at Rheumatology outpatient clinic dr. Hasan Sadikin Hospital from May 2015 to May 2016. All subject took underwent history, physical examination, spirometry and blood test for sCD40L. Data were analyzed using Pearson correlation.Result:There were 38 subjects involved in this study, dominated bywoman (92.1%) with mean age 41(±11) years. Subjects consist of 22(57,9%) with limited SSc, 16(42,1%) with diffuse SSc patients and 33 subjects treated with DMARD. Mean sCD40L serum in this study was 6.690,3(±2.377,3) pg/mL, with no statistical difference between limited and diffuse type (p=0.154). Mean FVC score in this study was 58.2(±10,8). There was no significant correlation between sCD40L serum with FVC (r=0.058; p=0.366). There was weak correlation on DMARD naïve subject between sCD40L serum and FVC (r=0.058; p=0.366) but statistically insignificant. There was no significant correlation between sCD40L serum with mRSS (r=0,066; p=0,346).Conclusion: This study founds no correlation between sCD40L with FVC in SSc at dr. Hasan Sadikin Hospital. Keyword : sCD40L, Forced Vital Capacity, Restrictive Lung Disease, Systemic Sclerosis

scholarly journals Correlation of sCD40L Level with Force Vital Capacity Value in Restrictive Lung Disease of Systemic Sclerosis Patients

2018 ◽  
Vol 10 (1) ◽  
Author(s):  
Susanto Salim ◽  
Rachmat Gunadi Wachjudi ◽  
Sumartini Dewi
Keyword(s):  
Systemic Sclerosis ◽  
Lung Disease ◽  
Forced Vital Capacity ◽  
Lung Fibrosis ◽  
Vital Capacity ◽  
High Resolution Computed Tomography ◽  
Cross Sectional ◽  
Restrictive Lung Disease ◽  
Potential Biomarker ◽  
Scd40l Level

Background: Interstitial Lung Disease (ILD) is one of the major cause of morbidity and mortality in Systemic Sclerosis (SSc). The gold standard to diagnose ILD is using High Resolution Computed Tomography (HRCT) scan. HRCT scan need a lot of cost and not always available, so another diagnosing test is needed as an alternative modality to diagnose ILD. ILD is a restrictive lung disease caused by lung fibrosis which is proved by the decrease of Forced Vital Capacity (FVC) in spirometry, and followed by the increase of soluble CD40L (sCD40L) level in plasma. This sCD40L may become a potential biomarker to evaluate lung fibrosis in SSc patients. The aim of this study is to analyze the correlation of sCD40L levels with FVC score in SSc patients with restrictive lung disease.Method:This cross sectional study was enrolled by the SSc patient who has restrictive lung disease based on spirometry test, at Rheumatology outpatient clinic dr. Hasan Sadikin Hospital from May 2015 to May 2016. All subject took underwent history, physical examination, spirometry and blood test for sCD40L. Data were analyzed using Pearson correlation.Result:There were 38 subjects involved in this study, dominated bywoman (92.1%) with mean age 41(±11) years. Subjects consist of 22(57,9%) with limited SSc, 16(42,1%) with diffuse SSc patients and 33 subjects treated with DMARD. Mean sCD40L serum in this study was 6.690,3(±2.377,3) pg/mL, with no statistical difference between limited and diffuse type (p=0.154). Mean FVC score in this study was 58.2(±10,8). There was no significant correlation between sCD40L serum with FVC (r=0.058; p=0.366). There was weak correlation on DMARD naïve subject between sCD40L serum and FVC (r=0.058; p=0.366) but statistically insignificant. There was no significant correlation between sCD40L serum with mRSS (r=0,066; p=0,346).Conclusion: This study founds no correlation between sCD40L with FVC in SSc at dr. Hasan Sadikin Hospital. Keyword : sCD40L, Forced Vital Capacity, Restrictive Lung Disease, Systemic Sclerosis

scholarly journals Diagnosis and management of interstitial lung disease of systemic sclerosis in challenging health conditions

2019 ◽  
pp. 18-20
Author(s):  
Daniel Rivas-Vargas
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Vital Capacity ◽  
Screening Method ◽  
Skin Fibrosis ◽  
Number Of Patients ◽  
History Of ◽  
Salt And Pepper

A 51-year-old woman presented with swelling in both hands and a 3-months history of triphasic Raynaud phenomenon. She denied cough and dyspnea. The physical examination was notable for swollen hands, facial telangiectasia and salt and pepper lesions. She had no skin fibrosis. The determination of antinuclear antibodies and antitopoisomerase were positive. A spirometry demonstrated a forced vital capacity of 86% of the predicted. High resolution chest computed tomography revealed bilateral, basal and subpleural ground glass opacities. These findings were consistent with the diagnosis of Scleroderma-Related Interstitial Lung Disease. Moreover, due to the absence of skin fibrosis, a diagnosis of systemic sclerosis sine scleroderma was made. Infusions of endovenous cyclophosphamide were indicated at 4-week intervals, followed by oral azathioprine. This case showed that using PFT as the single screening method for SSc-ILD may cause clinicians to miss a significant number of patients and that the absence of pulmonary symptoms does not exclude lung disease in patients with normal FVC.

scholarly journals P151 Forced vital capacity in patients with systemic sclerosis associated pulmonary fibrosis: predictors of meaningful decline

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Svetlana I Nihtyanova ◽  
Emma C Derrett-Smith ◽  
Carmen Fonseca ◽  
Voon H Ong ◽  
Christopher P Denton
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Fibrosis ◽  
Forced Vital Capacity ◽  
Vital Capacity ◽  
Cox Regression ◽  
Pulmonary Function Tests ◽  
Age At Onset ◽  
Time Factors ◽  
Standard Management ◽  
Over Time

Abstract Background Pulmonary fibrosis (PF) is common in systemic sclerosis (SSc) and serial pulmonary function tests (PFTs) are used for routine PF monitoring. Forced vital capacity (FVC) decline reflects progression in PF and FVC is frequently used as an endpoint in clinical trials. We explore the changes in FVC over time in patients with SSc-PF, receiving standard management, including immunosuppression. Methods Only SSc patients with CT-confirmed PF were included. FVC changes over the first 10 years from onset and their associations were assessed using linear mixed effects models. Predictors of time from first PFT to development of threshold FVC (FVC<70% and FVC<50%) were analysed using Cox regression. Results We identified 505 SSc-PF subjects, 21.6% were male, average age at onset was 47 years and 49.3% had diffuse cutaneous subset (dcSSc). The most common autoantibody was anti-Scl70 in 40.4%, followed by anti-RNA polymerase (ARA) in 11.7% and anti-centromere (ACA) in 7.1%. In 16.4% of the patients, ANA was positive, but no ENAs were identified (ANA+ENA-). Average FVC at 12 months from onset was 80.1% (SD 19.3) and this declined by 0.32% per year (p = 0.007) at a group level. There was no significant correlation between baseline FVC and subsequent change (correlation coefficient -0.13; 95%CI -0.26, 0.01). For every year older age at onset, average FVC increased by 0.32%, p < 0.001. Males had 3.3% lower FVC at 1 year from onset (p < 0.001) and 0.6% faster decline per year (p = 0.034) compared to females. DcSSc subjects had 5.6% lower FVC compared to limited disease (p = 0.003). Average FVC at 1 year from onset in ARA+ subjects were higher than any other antibody (15.1% v. ANA+ENA-, p < 0.001; 14.6% v. ATA, p < 0.001 and 12.5% v. ACA, p = 0.010). Nevertheless, ARA+ subjects had FVC decline rates similar to ATA+ and ANA+ENA- subjects, while ACA+ patients had a small but significant increase in FVC over time. Factors that increased the risk for FVC drop below the thresholds were male sex, ATA and low baseline FVC (Table 1). Conclusion This study provides insight into long-term patterns of FVC change and develops a model that may help predict those most at risk of significant decline. Disclosures S.I. Nihtyanova None. E.C. Derrett-Smith None. C. Fonseca None. V.H. Ong None. C.P. Denton None.

scholarly journals OP0272 68GA-FAPI-04 PET/CT STUDY EXTENSION FOR THE ASSESSMENT OF FIBROBLAST ACTIVATION AND RISK EVALUATION IN SYSTEMIC SCLEROSIS-RELATED INTERSTITIAL LUNG DISEASE

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 165.1-166
Author(s):  
C. Bergmann ◽  
J. H. W. Distler ◽  
C. Treutlein ◽  
K. Tascilar ◽  
A. T. Mueller ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Disease Progression ◽  
Forced Vital Capacity ◽  
Vital Capacity ◽  
Control Group ◽  
Research Support ◽  
Fibroblast Activation ◽  
Pet Ct

Background:Interstitial lung disease (ILD) is the most common cause of death in systemic sclerosis (SSc). To date, the progression of SSc-ILD is judged by the accrual of lung damage on computed tomography (CT) and functional decline (forced vital capacity). However, this approach does not directly assess the activity of tissue remodeling. Moreover, prediction of the course of ILD in individual SSc patients remains challenging. Fibroblast Activation Protein (FAP) is a specific, ex vivo validated marker for activated fibroblasts.Objectives:The aims of this study were: 1. To assess differences in the uptake of 68GA-FAPI 04 in SSc-ILD patients compared to controls, to analyze 2. whether 68GA-FAPI 04 uptake at baseline correlates with other risk factors of disease progression and 3. Whether 68GA-FAPI 04 uptake is associated with the course of SSc-ILD.Methods:Between September 2018 and April 2020, 21 patients with SSc-ILD confirmed by HRCT and onset of SSc-ILD within ≤ 5 years or signs of progressive ILD and 21 controls without ILD were consecutively enrolled. All participants underwent 68Ga-FAPI-04 PET/CT imaging and standard-of-care procedures including HRCT and lung function testing (PFT) at baseline. Patients with SSc-ILD patients were followed-up for 6 months with HRCT and PFT. Follow-up 68Ga-FAPI-04 PET/CT scans were obtained in a subset of patients treated with nintedanib. We compared baseline 68Ga-FAPI-04 PET/CT uptake to standard diagnostic tools and currently used predictors of ILD progression. The association of 68Ga-FAPI-04 uptake with changes in FVC was analyzed using mixed-effects models.Results:68Ga-FAPI-04 accumulated in fibrotic areas of the lungs in SSc-ILD compared to controls with a median (q1-q3 interval) wlSUVmean of 0.8 (0.6 to 2.1) in the SSc-ILD group and 0.5 (0.4 to 0.5) in the control group (p<0.0001 with Mann-Whitney test) and a median whole lung maximal standardized uptake value (wlSUVmax) of 4.4 (3.05 to 5.2) in the SSc-ILD group compared to 0.7 (0.65 to 0.7) in the control group (p<0.0001). wlFAPI-MAV and wlTL-FAPI were not measurable in control subjects, as no 68Ga-FAPI-04 uptake above background level was observed. In the SSc-ILD group the median wlFAPI-MAV was 254cm3 (163.4 to 442.3) and the median wlTL-FAPI was 183.6 cm3 (98.04 to 960.7). 68Ga-FAPI-04 uptake was higher in patients with extensive disease, with previous ILD progression or high EUSTAR activity scores. Increased 68Ga-FAPI-04 uptake at baseline was associated with progression of ILD independently of extent of involvement on HRCT scan and the forced vital capacity at baseline. In consecutive 68Ga-FAPI-04-PET/CTs, changes in 68Ga-FAPI-04 uptake was concordant with the observed response to the fibroblast-targeting antifibrotic agent nintedanib.Conclusion:Our study presents first in human evidence that 68Ga-FAPI-04-fibroblast uptake correlates with fibrotic activity and disease progression in the lungs of SSc-ILD patients and that 68Ga-FAPI-04-PET/CT may be of potential to improve risk assessment of SSc-ILD.Figure 1.A and B:68Ga-FAPI-04 PET/CT scan from a patient with SSc-ILD with selective 68Ga-FAPI-04 uptake in fibrotic areas of the left- and right lower lung lobes (red arrows), but not in non-fibrotic areas such as the middle lobe (green arrow). B Corresponding CT component.Acknowledgements:We gratefully acknowledge Prof. Uwe Haberkorn (University Hospital Heidelberg and DKFZ, Heidelberg, Germany) and iTheranostics Inc. (Dulles, VA, USA) for providing the precursor FAPI-04.Disclosure of Interests:Christina Bergmann: None declared, Jörg H.W. Distler Speakers bureau: Actelion, Anamar, ARXX, Pharma, Boehringer Ingelheim, Celgene, Galapagos, GSK, Inventiva, JB Therapeutics, and UCB, Grant/research support from: Anamar, Active Biotech, Array Biopharma, ARXX, aTyr, BMS, Bayer Pharma, Boehringer Ingelheim, Celgene, Galapagos, GSK, Inventiva, Novartis, Sanofi-Aventis, RedX, UCB, Christoph Treutlein: None declared, Koray Tascilar Speakers bureau: Gilead sciences GmbH, Pfizer Turkey, UCB Turkey, Anna-Theresa Mueller: None declared, Armin Atzinger: None declared, Alexandru-Emil Matei: None declared, Johannes Knitza: None declared, Andrea-Hermina Györfi: None declared, Anja Lueck: None declared, Clara Dees: None declared, Alina Soare: None declared, Andreas Ramming: None declared, Verena Schönau: None declared, Oliver Distler Speakers bureau: Arxx Therapeutics, Baecon Discovery, Blade Therapeutics,Bayer, Böhringer Ingelheim, Catenion,Competitive Drug Development International Ltd, Corbuspharma, CSL Behring, ChemomAb, Horizon Pharmaceuticals, Ergonex, Galaapagos NV, Glenmark Pharmaceuticals,GSK, Inventiva, Italfarmaco, IQvia, Kymera, Lilly, Medac, Medscape, MSD, Novartis, Pfizer, Roche, Sanofi, Taget Bio Sciencec, UCB, Grant/research support from: Bayer,Böhringer Ingelheim, Mitsubishi Tanabe Pharma, Olaf Prante: None declared, Philipp Ritt: None declared, Theresa Ida Goetz: None declared, Markus Koehner: None declared, Michael Cordes: None declared, Tobias Baeuerle: None declared, Torsten Kuwert Speakers bureau: Honoraria for occasional lectures by Siemens Healthineers, Grant/research support from: Research grant to the Clinic of Nuclear Medicine by this entity covering projects in the field of SPECT/CT, Georg Schett: None declared, Christian Schmidkonz: None declared

Mycophenolate in scleroderma-associated interstitial lung disease: Real-world data from rheumatology and pulmonology clinics in South Asia

2021 ◽  
pp. 239719832110244
Author(s):  
Ramya Janardana ◽  
Aparna Irodi ◽  
Pramod P Chebbi ◽  
Ruchika Goel ◽  
Leena R Vimala ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Mycophenolate Mofetil ◽  
Systemic Sclerosis ◽  
High Resolution ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Forced Vital Capacity ◽  
Vital Capacity ◽  
High Resolution Computed Tomography

Introduction: There is a paucity of real-world data on mycophenolate mofetil/mycophenolate sodium in systemic sclerosis-related interstitial lung disease. Aim: To study the efficacy of mycophenolate mofetil/ mycophenolate sodium in systemic sclerosis-related interstitial lung disease. Methods: In this single-centre study, clinical, laboratory and imaging details of consecutive patients with systemic sclerosis-related interstitial lung disease receiving mycophenolate mofetil/mycophenolate sodium from rheumatology and pulmonology clinics between January 2008 and March 2017 were retrospectively retrieved. The change in percentage of predicted normal forced vital capacity at last follow-up visit as compared with baseline was studied. In addition, high-resolution computed tomography scans at baseline and 2-year follow-up visit were scored as either stable/improved or worsened by experienced thoracic radiologists blinded to the clinical details of patients. Results: Altogether, 88 patients (85.2% females) with mean age (SD) of 33.8 years (± 11.3) and median (interquartile range) duration of disease since non-Raynaud’s symptoms of 36 months (13.5–60) were studied. Diffuse systemic sclerosis comprised 85.2% of them. The mean baseline forced vital capacity was 61.2 ± 17.9% and median scores for ground glass opacities and fibrosis in high-resolution computed tomography were 0.5 (0–1.3) and 1 (0–1.3), respectively. At a median follow-up duration of 30 months (interquartile range = 16.5–49), the percentage of forced vital capacity improved by 1.8% (–3.82 to 9.07) as compared with baseline visit ( p = 0.02). In the 2-year follow-up, the ground glass opacity and fibrosis scores in high-resolution computed tomography improved in 17.3% and 7.7% of patients and stabilized in 63.5% and 78.8% patients, respectively. Conclusion: Mycophenolate mofetil/mycophenolate sodium was efficacious in improving /stabilizing forced vital capacity irrespective of the baseline high-resolution computed tomography lung scores in our patients with systemic sclerosis-related interstitial lung disease during the ⩾ 2-year follow-up period.

Mycophenolic acid drug monitoring in patients with systemic sclerosis associated with diffuse skin and/or pulmonary involvement: A monocentric and retrospective French study

2020 ◽  
pp. 239719832094434
Author(s):  
Paul Legendre ◽  
Benoit Blanchet ◽  
Raphael Porcher ◽  
Alice Bérezné ◽  
Marie Allard ◽  
...  
Keyword(s):  
Carbon Monoxide ◽  
Mycophenolate Mofetil ◽  
Systemic Sclerosis ◽  
Mycophenolic Acid ◽  
Vital Capacity ◽  
Area Under The Curve ◽  
Skin Involvement ◽  
Skin Score ◽  
Lung Capacity ◽  
Modified Rodnan Skin Score

Objective: To explore pharmacokinetic/pharmacodynamic relationship between mycophenolic acid area under the curve and clinical response at 1 year on skin involvement or interstitial lung disease in patients with systemic sclerosis. Method: Retrospective, monocentric study based on French Scleroderma Database in patients receiving mycophenolate mofetil who experienced a limited sampling strategy to estimate individual mycophenolic acid area under the curve plus two pulmonary function tests and skin evaluation after 1 month and 1 year. Efficacy criterions were variations of modified Rodnan skin score, forced vital capacity, and diffusing lung capacity for carbon monoxide at 1 year. Results: We included 52 patients; mean age was 49 years (range 17–79), and 36 (69%) were females. Fifty patients (96%) had skin sclerosis, 39 (75%) had diffuse skin involvement with a median modified Rodnan skin score of 14 (0–38). Thirty-eight (76%) had interstitial lung disease, with median forced vital capacity and diffusing lung capacity for carbon monoxide of 81% (37–127) and 56% (28–103) from predicted values, respectively. Twenty-five (51%) patients had pulmonary fibrosis. Mycophenolate mofetil was given for 10 months (0–173) at a median dose of 2000 mg/day (500–3000). In the entire population, no relationship was found between area under the curve and modified Rodnan skin score (p = 0.085), forced vital capacity (p = 0.80), or diffusing lung capacity for carbon monoxide (p = 0.72) variations at 1 year. Conclusion: In this retrospective study, we failed to document any relationship between mycophenolic acid area under the curve and skin involvement or interstitial lung disease evolution. Routine monitoring of mycophenolic acid in systemic sclerosis patients treated with mycophenolate mofetil cannot be recommended based on our results.

scholarly journals Correlation Between Serum Procollagen Type 1 N-Terminal Propeptide Level With Modified Rodnan Skin Score In Systemic Sclerosis Patients.

2018 ◽  
Vol 9 (2) ◽  
Author(s):  
Vincent Vincent ◽  
Sumartini Dewi ◽  
Rachmat Gunadi Wachjudi
Keyword(s):  
Systemic Sclerosis ◽  
Cross Sectional Study ◽  
Skin Fibrosis ◽  
Cross Sectional ◽  
Skin Score ◽  
Procollagen Type ◽  
Potential Biomarker ◽  
Modified Rodnan Skin Score ◽  
Collagen Type 1

Introduction: Systemic Sclerosis (SSc) is a chronic autoimmune disease, characterized by vasculopathy, specific autoimmune, and fibrosis. Assesment of skin fibrosis by modified Rodnan Skin Score (mRSS) can not detect the minimal changes of skin fibrosis within lessthan 3 months. A biomarker is needed to assess the minimal changes of skin fibrosis progressivity with a more objective, quantitative, and rapid way. Procollagen type-1 N-Terminal Propeptide (P1NP), a degradation product of collagen type-1, may become a potential biomarker for skin fibrosis. This study aims to evaluate the correlation between skin fibrosis by mRSS with P1NP serum in systemic sclerosis.Methods: This was a cross-sectional study performed among systemic sclerosis patients at Rheumatology outpatient clinic, Dr.Hasan Sadikin Hospital Bandung, from May 2016 to July 2016. Skin fibrosis was measured by mRSS. P1NP level was determined by ELISA. Data were analyzed using Rank-Spearman Correlation.Result: There were thirty-seven subjects, with mean age 37 (SD ±7) years old. Most of subjects were female (91.9%). Subjects consisted of 23 (62.2%) limited SSc and 14 (37.8%) diffuse SSc. Six subjects (16.2%) were DMARD naïve. We found median (range) P1NP serum was 43.85 (9.81-127.90) ng/dL, while the median of MRSS was 14 (3-36). There is a moderate correlation between MRSS and P1NP serum (r=0.443, p=0.003)Conclusion: There was a significant correlation between mRSS and P1NP serum in systemic sclerosis patient at Dr. Hasan Sadikin Hospital Bandung.Keywords: systemic sclerosis, P1NP, modified rodnan skin score

scholarly journals Dependence of forced vital capacity manoeuvre on time course of predicting inspiration in patients with restrictive lung disease

1997 ◽  
Vol 10 (10) ◽  
pp. 2366-2370 ◽  
Author(s):  
N.G. Koulouris ◽  
P Rapakoulias ◽  
A. Rassidakis ◽  
J. Dimitroulis ◽  
M. Gaga ◽  
...  
Keyword(s):  
Lung Disease ◽  
Forced Vital Capacity ◽  
Time Course ◽  
Vital Capacity ◽  
Restrictive Lung Disease
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