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Abstrak :
This essential resource presents the most up-to-date information on scleroderma. A clear and concise synthesis of current concepts in pathogenesis and modern approaches to management, this book is comprised of the authoritative work of international experts. With an integrated multidisciplinary approach to comprehensive care, this book is easily accessible for health care professionals in many fields. It is a valuable resource for rheumatologists, pulmonologists, cardiologists, gastroenterologists, nephrologists and all those involved in the care of scleroderma patients.

Abstrak :
scleroderma is an autoimmune disease characterized by organ fibrosis, resistant to standard treatment. It is suspected the addition of Physalis angulata Linn. (Ciplukan) extract as adjuvant therapy can improve the scleroderma skin fibrosis. The aim at this study is to evaluate the effect of ciplukan extract as adjuvant on scleroderma skin fibrosis in standard therapy, based on modified Rodnan skin scale (MRSS), inflammatory biomarkers, immunology and serum fibrosis. Methods: double-blind, randomized clinical trial was performed in scleroderma patients with stable disease at Cipto Mangunkusumo hospital and Hasan Sadikin hospital during November 2015−March 2017 who met the selection criteria and continued to receive standard therapy. The subjects were randomly allocated into two groups: the study group received the ciplukan extract 3 x 250 mg / day for 12 weeks and the placebo group. Examination of MRSS, ESR, P1NP, BAFF and sCD40L was performed every 4 weeks until the end of the study. Results: fifty-nine subjects completed the study. They consisted of 29 subjects of the treatment group and 30 of the placebo group, with an average age of 41 (SD 9) years, the proportion of women: male = 9 : 1. There was a significant improvement of skin fibrosis in the study group with a highly significant decrease in MRSS (35.9% VS 6.3%, p <0.001) and a relative decrease in P1NP levels (17.8% VS 0.7%, p = 0.002). No decrease in ESR, BAFF and sCD40L levels in both groups. There was a weak but significant positive correlation between MRSS with P1NP levels (r = 0.236, p = 0.036). Conclusion: Ciplukan extract with dose 3 x 250 mg for 12 weeks as adjuvant on scleroderma standard therapy alleviates skin fibrosis significantly based on MRSS and P1NP levels.

Abstrak :
ABSTRACT
Skleroderma (sklerosis sistemik) merupakan penyakit autoimun dengan ciri fibrosis kulit dan organ viseral. Skleroderma menurunkan kualitas hidup penderitanya dan menyebabkan kematian, yang dapat diakibatkan oleh penyakit paru interstisial (ILD) sebagai manifestasi fibrotik pada skleroderma. Pemantauan ILD pada skleroderma memerlukan penilaian penanda inflamasi, termasuk di antaranya adalah laju endap darah (LED) dan kadar protein c-reaktif (CRP) darah, serta penilaian fungsi paru dengan menilai kapasitas vital paksa (KVP). Penelitian ini dilakukan untuk mengetahui korelasi antara LED dan CRP dengan KVP pada pasien skleroderma di Rumah Sakit Cipto Mangunkusumo Jakarta. Penelitian potong lintang dilakukan dengan mengumpulkan data dari rekam medis pasien tahun 2013-2015. Hasil penelitian menunjukkan tidak adanya korelasi yang signifikan antara LED dengan KVP (r=0,209; p=0,287) dan CRP dengan KVP (r=0,261; p=0,241).

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ABSTRACT
Scleroderma (systemic sclerosis) is an autoimmune disease characterized by the fibrosis of skin and visceral organs. Scleroderma affects the quality of life and cause mortality, which may be caused by interstitial lung disease (ILD) as the fibrotic manifestation of scleroderma. Monitoring of ILD in scleroderma requires the evaluation of markers of inflammation, including erythrocyte sedimentation rate (ESR) and CRP level, and also evaluation of lung function by measuring forced vital capacity (FVC). The objective of this study is to determine the correlation of ESR and CRP to FVC in scleroderma patients of Cipto Mangunkusumo Hospital Jakarta. A cross-sectional study was done by gathering data from medical records of patients from year 2013-2015. The study showed that there is no significant correlation between ESR and FVC (r=0,209; p=0,287 and between CRP and FVC (r=0,261; p=0,241).

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.

Abstrak :
Latar belakang - Skleroderma merupakan kelainan jaringan ikat yang ditandai dengan keterlibatan multisistem organ, bersifat kronik dan progresif, dan memiliki tingkat mortalitas serta disabilitas yang tinggi. Hipertensi pulmonal merupakan salah satu penyebab utama kematian pada pasien skleroderma. Tujuan - Penelitian ini bertujuan untuk mencari hubungan antara laju endap darah dan kadar protein reaktif C dengan kejadian hipertensi pulmonal pada pasien skleroderma. Metode - Desain studi pada penelitian ini adalah potong lintang dengan menggunakan data sekunder dari 115 pasien skleroderma di RSUPN Cipto Mangunkusumo Jakarta. Hasil - Dari 115 pasien, hanya 46 subjek yang memenuhi syarat. Hipertensi pulmonal terdeteksi pada 4 pasien berdasarkan hasil ekokardiografi. Tidak ada perbedaan rerata laju endap darah yang bermakna antara kelompok yang mengalami hipertensi pulmonal dengan yang tidak (p = 0,154; perbedaan rerata = 22,58; IK95%:  -8,80 – 53,97). Rerata kadar protein reaktif C juga tidak berbeda bermakna antara kelompok hipertensi pulmonal dengan yang tidak (p = 0,748; perbedaan rerata 0,80; IK95%: 0,19 – 3,29). Simpulan - Dari penelitian ini, tidak ada hubungan antara laju endap darah dan kadar Protein reaktif C terhadap kejadian hipertensi pulmonal pada pasien skleroderma. Walaupun demikian, laju endap darah dan kadar protein reaktif C cenderung lebih tinggi pada kelompok yang tidak mengalami hipertensi pulmonal. ......Background - Scleroderma is connective tissue disease characterized by multisystem organ involvement, chronic and progressive course, and high mortality and disability rate. Objectives - This study aimed to determine the association between erythrocyte sedimentation rate and C-reactive protein level to the occurrence of pulmonary hypertension in scleroderma patients. Methods - This study design was cross-sectional. One-hundred-and-fifteen medical records of scleroderma patient in Cipto Mangunkusumo National Hospital were reviewed. Results - From 115 patients, only 46 subject eligible in this study. Pulmonary hypertension was detected in 4 patients by echocardiography. There was no significant erythrocyte sedimentation rate mean  difference between the group with pulmonary hypertension and those without (p = 0,154; mean difference = 22,58; CI95%:  -8,80 – 53,97). Mean C-reactive protein level was not significantly different between those with pulmonary hypertension and those without (p = 0,748; mean difference = 0,80; CI95%: 0,19 – 3,29). Conclusions - From this study, there was no significant association between erythrocyte sedimentation rate and C-reactive protein level to the occurrence of pulmonary hypertension in scleroderma patients. Nevertheless, erythrocyte sedimentation rate and C-reactive protein tend to be higher in those without pulmonary hypertension.