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Abstrak :
Background systemic sclerosis (SSc) is a chronic autoimmune disease which presents immunological, endothelilal dysfunction, skin and organs fibrosis. The inflammatory process is an important pathopshsiology of systemic sclerosis. Disease activity assessment using clinical parameters of c creative protein (CRP), erytrocyte sedimentation rate (ESR) and soluble CD40 lingand.

Abstrak :
Background: Systemic sclerosis is a chronic progressive multisystem autoimmune disease in connective tissue, characterized by its heterogeneous clinical manifestation. The purpose of this study is to give information regarding clinical manifestations and laboratory findings of systemic sclerosis patients to establish diagnosis of disease. Methods: This study was conducted using descriptive quantitative design in September until October 2016. Data was collected from medical records of patients visiting Rheumatology Clinic Dr. Hasan Sadikin General Hospital from 1 July 2015 until 30 June 2016 using total sampling method. The collected data were expected to comprise patients clinical manifestation and laboratory finding. Results: Most of patients had cutaneous 57 100.0 pecent and musculoskeletal 40 70.2 pecent involvement. Some of the disease manifestations were Raynauds phenomenon 38 66.7 pecent , fingertip lesion 33 57.9 pecent, stiffness in skin 34 59.6 pecent, and arthalgia 29 50.9 pecent. Gastrointestinal involvements were present in 29 50.9 pecent patients. Renal involvement were determined from urinalysis result showed proteinuria 10 17.5 pecent and hematuria 8 14.0 pecent, found in 24 42.1 pecent patients, while pulmonary and cardiac involvements were found in 30 52.6 pecent patients, acknowledged from clinical symptoms such as dyspnea 12 21.1 pecent. Identification of autoantibodies was found in 12 21.1 pecent patients, with 10 17.5 pecent patients had reactive ANA and 3 3.5 pecent had positive anti Scl70. Conclusion: Most of systemic sclerosis patients had cutaneous involvement. Renal, pulmonary, and cardiac involvement were concluded based on laboratory findings.

Abstrak :
Background: Interstitial Lung Disease is one of the major cause of morbidity and mortality in Systemic Sclerosis. The gold standard to diagnose ILD is using High Resolution Computed Tomography 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 in spirometry, and followed by the increase of soluble CD40L 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 pecent with mean age 41years. Subjects consist of 22 57,9 pecent with limited SSc, 16 42,1 pecent with diffuse SSc patients and 33 subjects treated with DMARD. Mean sCD40L serum in this study was 6.690,3 pg/mL, with no statistical difference between limited and diffuse type p 0.154. Mean FVC score in this study was 58.2. 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.

Abstrak :
Latar Belakang: Penyakit paru interstisial (ILD) merupakan salah satu manifestasi sklerosis sistemik (SSc) pada paru dan faktor mortalitas utama SSc. SSc-ILD meningkatkan angka mortalitas 5 tahun pasien SSc sebesar 3 kali lipat. Hampir dua pertiga pasien SSc-ILD dengan kelainan minimal pada high resolution computed tomography (HRCT) toraks memperlihatkan progresivitas signifikan dalam 2 tahun. Model prediksi progresivitas SSc-ILD yang tersedia, yakni GAP (gender, age, and lung physiology) dan SADL (smoking history, age, and diffusion capacity of the lung), terbukti memiliki nilai prognostik yang baik. Model prognostik yang melibatkan parameter HRCT toraks dan Modified Rodnan Skin Score (mRSS) diharapkan dapat membantu seleksi pasien SSc-ILD yang memerlukan pemantauan ketat atau terapi dini untuk mencegah progresivitas. Metode: Studi ini melibatkan pasien SSc-ILD yang menjalani pemeriksaan HRCT toraks awal dan evaluasi di Rumah Sakit Umum Pusat Nasional Dokter Cipto Mangunkusumo pada periode Januari 2016 hingga Desember 2021. Dilakukan volumetri kuantitatif menggunakan piranti lunak 3DSlicer® pada HRCT toraks awal untuk menghasilkan persentase volume paru abnormal, high attenuation area (HAA), dan low attenuation area (LAA) yang selanjutnya dianalisa sebagai faktor prognostik. Pola ILD pada HRCT toraks awal dan nilai mRSS masing-masing subyek diidentifikasi dan dianalisa sebagai faktor prognostik progresivitas SSc-ILD. Progresivitas SSc-ILD dikategorikan menjadi progresif dan non-progresif berdasarkan selisih persentase volume paru abnormal antara HRCT toraks awal dan evaluasi. Hasil: Perbedaan rerata yang bermakna ditemukan pada volume paru abnormal, volume HAA, dan volume LAA, nilai mRSS antara SSc-ILD progresif dan non-progresif (p < 0,001). Berdasarkan receiver operating characteristic curve, ditetapkan nilai titik potong dari masing-masing variabel. Nilai titik potong persentase volume paru abnormal ditetapkan sebesar 32,82% dengan nilai sensitivitas 100% dan spesifisitas 93,8%. Nilai titik potong persentase volume HAA ditetapkan sebesar 19,76% dengan nilai sensitivitas 93,8% dan spesifisitas 93,8%. Nilai titik potong persentase volume LAA ditetapkan sebesar 9,89% dengan nilai sensitivitas 62,5% dan spesifisitas 62,5%. Nilai titik potong mRSS ditetapkan sebesar 18,5 dengan sensitivitas 93,8% dan spesifisitas 100%. Tidak ada perbedaan proporsi pola ILD antara kedua kelompok tersebut (p 0,220). Kesimpulan: Volume paru abnormal > 32,82%, volume HAA > 19,76%, volume LAA > 9,89%, dan/atau nilai mRSS > 18,5 merupakan prediktor progresivitas SSc-ILD. Hasil volumetri kuantitatif abnormalitas paru pada HRCT toraks dan nilai mRSS merupakan faktor prognostik progresivitas SSc-ILD yang mudah diperoleh dan diaplikasikan dalam praktik klinis sehari-hari. ......Background: Interstitial pulmonary disease (ILD) is one of the manifestations of systemic sclerosis (SSc) in the lungs and the main mortality factor of SSc. SSc-ILD multiplies the 5-year mortality rate of SSc patients by 3 times. Nearly two-thirds of SSc-ILD patients with minimal abnormalities in chest high resolution computed tomography (HRCT) showed significant progressivity within 2 years. The available prediction models of SSc-ILD progression, namely GAP (gender, age, and lung physiology) and SADL (smoking history, age, and diffusion capacity of the lungs), have been proven to demonstrate excellent prognostic values. Prognostic models involving chest HRCT parameters and Modified Rodnan Skin Score (mRSS) are expected to aid the selection of SSc-ILD patients who require close monitoring or early therapy to prevent progression. Method: This study involved SSc-ILD patients who underwent initial and follow-up chest HRCT examination and evaluation at the National Central General Hospital of Doctor Cipto Mangunkusumo in the period from January 2016 to December 2021. Quantitative volumetric measurement was performed using 3DSlicer® software on the initial chest HRCT to yield abnormal pulmonary volume, high attenuation area (HAA) volume, and low attenuation area (LAA) volume percentage which were subsequently analyzed as prognostic factors. ILD patterns in the initial chest HRCT and mRSS values of each subject were identified and analyzed as prognostic factors of SSc-ILD progression. The progression of SSc-ILD is classified into progressive and non-progressive based on the abnormal pulmonary volume percentage difference between the initial and follow-up chest HRCT. Result: Significant mean differences were found in abnormal lung volume percentage, HAA volume percentage, LAA volume percentage, and mRSS values between progressive and non-progressive SSc-ILD groups (p < 0.001). Based on the receiver operating characteristic curve, the cut-off point value of each variable is determined. The cut-off point value of the percentage of abnormal pulmonary volume was set at 32.82% with a sensitivity value of 100% and a specificity of 93.8%. The cut point value of the HAA volume percentage was set at 19.76% with a sensitivity value of 93.8% and a specificity of 93.8%. The LAA volume percentage cut point value was set at 9.89% with a sensitivity value of 62.5% and a specificity of 62.5%. The mRSS cut-off value was set at 18.5 with a sensitivity of 93.8% and a specificity of 100%. There was no significant in the proportion of ILD patterns between the two groups (p 0.220). Conclusion: Abnormal lung volume > 32.82%, HAA volume > 19.76%, LAA volume > 9.89%, and/or mRSS value > 18.5 are predictors of SSc-ILD progression. Quantitative volumetric results of pulmonary abnormalities in chest HRCT and mRSS values are prognostic factors of SSc-ILD progression that are easily obtained and applied in daily clinical practice.