Hasil Pencarian  ::  Simpan CSV :: Kembali

"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."

"Latar belakang : Skleroderma adalah salah satu penyakit autoimun multifaktorial yang cukup jarang terjadi. Pasien yang mengalami skleroderma memiliki angka kesintasan yang kurang baik, dan data mengenai angka kesintasan serta faktor-faktor yg memengaruhinya pada berbagai penelitian menunjukkan hasil yang bervariasi. Selain itu juga masih belum ada data yang jelas mengenai angka kesintasan di Indonesia.Tujuan : Mengetahui kesintasan tiga tahun pasien skleroderma di Rumah Sakit Rujukan Tersier daerah Jakarta dan faktor-faktor yang memengaruhinya.Metode : Penelitian menggunakan desain kohort retrospektif di RS Cipto Mangunkusumo Jakarta, bulan Januari-September 2024. Kriteria inklusi penelitian ini adalah pasien diagnosis Skleroderma sesuai kriteria ACR EULAR 2013, umur diatas 18 tahun, dan berobat di RSCM dari tahun 2013 – 2021. Kriteria eksklusi penelitian ini adalah data pasien tidak lengkap dan rekam medis rusak. Faktor risiko yang diteliti pada penelitian ini antara lain jenis kelamin, usia saat didiagnosis, tipe penyakit skleroderma, adanya hipertensi pulmoner, adanya penyakit paru interstisial, adanya keterlibatan saluran cerna, adanya vaskulopati digiti, adanya anemia, serta adanya peningkatan kadar penanda inflamasi. Data diambil dari rekam medis, kemudian dilakukan pencatatan demografi serta faktor risiko yang sudah ditentukan. Data kemudian dilakukan analisis dengan metode Cox Regression. Nilai kesalahan yang dapat ditoleransi sebesar 5% (IK 95%) dengan nilai kemaknaan statistik apabila p-value < 0,05.Hasil : Sebanyak 153 pasien skleroderma masuk dalam penelitian ini. Kesintasan tiga tahun pasien sebesar 86,3% dengan mean survival sekitar 32 (31,6 – 34,2) bulan. Dari analisis multivariat diperoleh faktor risiko yang berhubungan dengan kesintasan tiga tahun adalah usia saat didiagnosis (p 0,010) dan adanya penyakit paru interstisial (p 0,031). Usia lebih dari 60 tahun meningkatkan risiko terjadinya kematian pasien skleroderma hampir 2 kali lipat dibandingkan pasien usia yang lebih muda [aHR 2,897 (1,518 - 5,530)]. Adanya penyakit paru interstisial menurunkan risiko kematian dalam tiga tahun sebanyak sekitar 40% dibandingkan dengan pasien tanpa penyakit paru interstisial [aHR 0,607 (0,386 - 0,954)]Kesimpulan : Kesintasan tiga tahun pasien skleroderma di RSCM masih baik, dengan usia saat didiagnosis dan kejadian penyakit paru interstisial merupakan faktor risiko independen terhadap kesintasan tiga tahun pasien skleroderma.

---

Background : Scleroderma is a multifactorial autoimmune disease that is quite rare. Patients with scleroderma have poor survival rates, and data on survival rates and factors that influence them in various studies show varying results. In addition, there is still no clear data on survival rates in Indonesia.

Objective : To determine the three-year survival of scleroderma patients at the Tertiary Referral Hospital in Jakarta and the factors that influence it.

Method : The study used a retrospective cohort design at Cipto Mangunkusumo Hospital, Jakarta, from January to September 2024. The inclusion criteria for this study were patients diagnosed with Scleroderma according to the ACR EULAR 2013 criteria, aged over 18 years, and treated at RSCM from 2013 to 2021. The exclusion criteria for this study were incomplete patient data and damaged medical records. The risk factors studied in this study included gender, age at diagnosis, type of scleroderma, pulmonary hypertension, interstitial lung disease, gastrointestinal involvement, digital vasculopathy, anemia, and increased levels of inflammatory markers. Data were taken from medical records, then demographics and predetermined risk factors were recorded. The data were then analyzed using the Cox Regression method. The tolerable error value was 5% (95% CI) with a statistical significance value if the p-value <0.05.

Result : A total of 153 scleroderma patients were included in this study. The three-year survival rate of patients was 86.3% with a mean survival of around 32 (31,6 – 34,2) months. From the multivariate analysis, the risk factors associated with three-year survival were age at diagnosed (p 0.010) and ILD (p 0.031). Age over 60 years increased the risk of death in scleroderma patients almost 2 times compared to younger patients [aHR 2.897 (1.518 - 5.530)]. The presence of ILD reduced the risk of death within three years by around 40% compared to patients without ILD [aHR 0.607 (0.386 - 0.954)]

Conclusion : The three-year survival of scleroderma patients at RSCM is good, with age at diagnosed and ILD occurrence being independent risk factors for the three-year survival of scleroderma patients.."

"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.

---

Latar belakang: skleroderma merupakan penyakit autoimun yang resisten terhadap pengobatan standar, penambahan ekstrak herba ciplukan (Physalis angulata Linn) diduga dapat memperbaiki fibrosis kulit skleroderma. Penelitian ini bertujuan mengkaji peran ekstrak herba Ciplukan sebagai terapi ajuvan untuk fibrosis kulit skleroderma yang mendapat terapi standar, berdasarkan MRSS, biomarker inflamasi, imunologi dan fibrosis serum. Metode: uji klinis acak tersamar ganda pada pasien skleroderma stabil yang berobat jalan di RSCM dan RSHS sejak November 2015−Maret 2017 yang memenuhi kriteria inklusi dan menerima terapi standar. Subjek secara random terbagi dua: kelompok uji yang mendapat ekstrak herba C iplukan 3x 250 mg/hari selama 12 minggu dan kelompok plasebo. Pemeriksaan MRSS, LED, P1NP, BAFF dan sCD40L dilakukan setiap 4 minggu hingga akhir penelitian. Hasil: lima puluh sembilan subjek menyelesaikan penelitian, 29 subjek kelompok uji dan 30 subjek kelompok plasebo, rerata usia 41 (SB 9) tahun, proporsi wanita : pria = 9 : 1. Ditemukan perbaikan fibrosis kulit bermakna pada kelompok uji dengan penurunan relatif MRSS sebesar 35,9% dibandingkan plasebo 6,3% dengan p < 0,001 dan penurunan relatif bermakna kadar P1NP sebesar 17,8% dibandingkan plasebo 0,7% dengan p = 0,002. Tidak ditemukan penurunan kadar LED, BAFF dan sCD40L pada kedua kelompok. Terdapat korelasi positif bermakna antara MRSS dengan kadar P1NP (r = 0,236, p = 0,036). Kesimpulan: pemberian ekstrak etanol herba ciplukan dosis 3 x 250 mg selama 12 minggu sebagai terapi ajuvan pada skleroderma dalam terapi standar, secara klinis dan statistik menunjukkan perbaikan kelainan fibrosis kulit berdasarkan MRSS dan biomarker fibrosis P1NP serum secara bermakna dibandingkan kontrol."

"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."

"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).

---

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)."

"klerosis sistemik atau skleroderma adalah suatu penyakit jaringan ikat yang dimediasi imun yang ditandai dengan fibrosis kulit dan organ dalam serta vaskulopati. Penyebab kematian utama pada sklerosis sistemik adalah penyakit paru interstisial. Pengobatan penyakit paru interstisial pada sklerosis sistemik saat ini belum memuaskan. Herba ciplukan (Physalis angulata) merupakan salah satu terapi alternatif yang potensial dan terbukti dapat memperbaiki fibrosis kulit pada pasien sklerosis sistemik namun data pada manifestasi paru belum ada. Penelitian ini bertujuan untuk menilai efek herba ciplukan dalam mencegah dan memperbaiki inflamasi dan fibrosis paru pada model tikus sklerosis sistemik dan mencari dosis optimal ciplukan untuk memperbaiki fibrosis. Penelitian ini terbagai dalam 2 tahap yaitu tahap kuratif fibrosis (tahap 1) dan tahap preventif inflamasi dan fibrosis (tahap 2). Pada tahap 1, 33 tikus (Rattus norvegicus) galur Sprague-Dawley 10−12 minggu dibagi dalam 6 kelompok yaitu kelompok yang mendapat bleomisin dan ciplukan (dosis 50,100,150, dan 200 mg/kg), bleomisin dan salin, dan kontrol negatif. Bleomisin diberikan subkutan per hari selama 14 hari dan ciplukan atau salin diberikan mulai hari ke-21 selama 30 hari lalu hewan diterminasi. Fibrosis dinilai dengan derajat fibrosis dan luas fibrosis pada histopatologi, kadar hidroksiprolin, TGF-β dan MMP13 jaringan paru. Pada tahap 2, 36 ekor tikus dibagi dalam 6 kelompok yaitu 2 kelompok yang mendapat bleomisin dan ciplukan (50 dan 100 mg/kgBB) dan 2 kelompok bleomisin dan salin. Tiga kelompok diterminasi di H14 dan 3 kelompok di H51. Pada tahap 2, bleomisin dan ciplukan diberikan bersamaan selama 14 hari pertama. Luaran yang dinilai di H14 adalah kadar IL-6 paru, jumlah leukosit dari BAL dan skor inflamasi paru secara histopatologi. Luaran yang dinilai di H52 adalah derajat fibrosis dan luas fibrosis, kadar hidroksiprolin, TGF-β dan MMP13 paru. Kadar IL-6, TGF-β dan MMP 13 dinilai dengan ELISA dari jaringan paru, Hidroksiprolin dinilai dari jaringan paru dengan metode kolorimetri. Pada tahap 1 terdapat perbedaan luas fibrosis yang secara statistik bermakna antara kelompok yang mendapat ciplukan dosis 100, 150, dan 200 mg/kgBB dibandingkan kelompok bleomisin. Tidak terdapat perbedaan skor fibrosis antara kelompok yang mendapat ciplukan 50, 100, dan 150 mg/kgBB dengan kontrol negatif. Tidak terdapat perbedaan hidroksiprolin antara kelompok yang mendapat ciplukan dengan kontrol negatif. Tidak terdapat perbedaan kadar TGF-β dan MMP13 yang secara statistik bermakna antar kelompok. Pada tahap 2 penelitian tidak didapatkan perbedaan kadar IL-6, jumlah leukosit cairan BAL dan skor inflamasi yang bermakna antar kelompok dan tidak terdapat perbedaan skor fibrosis, luas fibrosis, kadar hidroksiprolin, TGF-β dan MMP13 antar kelompok. Sebagai simpulan ekstrak ciplukan memiliki efek kuratif untuk menurunkan luas fibrosis paru dengan dosis optimal 100 mg/kgBB. Ciplukan tidak memiliki efek preventif terhadap inflamasi dan fibrosis.

---

Systemic sclerosis or scleroderma is an immune mediated connective tissue disease which is manifested by fibrosis on skin and internal organ and vasculopathy. Interstitial lung disease (ILD) is the main cause of death of systemic sclerosis however the treatment of ILD in systemic sclerosis is still unsatisfactory. Ciplukan (Physalis angulata) herb is a potential alternative treatment for systemic sclerosis and has been proven to improve skin sclerosis in systemic sclerosis patients however the study on its effect on lung has been lacking. The aim of this study is to evaluate the effect of ciplukan herb for treating and preventing inflammation and fibrosis in systemic sclerosis animal model and to find out its optimal dose in improving lung fibrosis. This study was done in 2 stages. For the first stage (treatment of fibrosis), 33 Sprague-Dawley rats aged 10−12 weeks were divided into 6 groups (4 groups were given bleomycin and ciplukan extract dose 50,100,150, and 200 mg/kgBW, respectively, bleomycin and saline and negative control). Bleomycin was given subcutaneously daily for 14 days and ciplukan or saline were given from day 21 until the next 30 days and then the animals were sacrificed. At the end of observation, degree of fibrosis and width of fibrosis from lung histopathology, hydroxyproline, TGF-β, and MMP13 levels were analyzed. For the second stage (prevention), 36 rats were divided into 6 groups (bleomycin and ciplukan dose 50 and 100 mg/kgBW, and bleomycin only). Three groups were sacrificed after 14 days of observation for evaluation of IL-6 level in lung tissue, leucocyte count on BAL fluid and inflammation score. Three groups were sacrificed after 51 days observation and were analyzed for degree of fibrosis and width of fibrosis from lung histopathology, hydroxyproline, TGF-β, and MMP13 levels. For the second stage, bleomycin and ciplukan were given simultaneously for 14 days. IL-6, TGF-β, and MMP13 levels were measured using ELISA methods while hydroxyproline was analyzed using colorimetric method. From the stage 1, there was a significant reduction in width of lung fibrosis on groups receiving bleomycin and ciplukan dose 100, 150, and 200 mg/kgBW compared with bleomycin group. There was no difference of fibrosis score among groups who received ciplukan 50,100, and 150 mg/kgBW compared to the negative control. There was no difference of hydroxyproline among groups who received ciplukan compared with negative control. There was no difference of TGF-β, and MMP13 levels among groups. From the stage 2, there were no difference of IL-6 levels, BAL leukocyte count and inflammation score among groups after 14 days and no difference of fibrosis score, extension of fibrosis, hydroxyproline, TGF-β and MMP13 levels among groups after 51 days observation. As a conclusion, ciplukan herb has a role as a treatment of fibrosis to reduce extent of lung fibrosis with optimal dose of 100 kg/BW but shows no effect on prevention of lung inflammation and lung fibrosis."

"klerosis sistemik atau skleroderma adalah suatu penyakit jaringan ikat yang dimediasi imun yang ditandai dengan fibrosis kulit dan organ dalam serta vaskulopati. Penyebab kematian utama pada sklerosis sistemik adalah penyakit paru interstisial. Pengobatan penyakit paru interstisial pada sklerosis sistemik saat ini belum memuaskan. Herba ciplukan (Physalis angulata) merupakan salah satu terapi alternatif yang potensial dan terbukti dapat memperbaiki fibrosis kulit pada pasien sklerosis sistemik namun data pada manifestasi paru belum ada. Penelitian ini bertujuan untuk menilai efek herba ciplukan dalam mencegah dan memperbaiki inflamasi dan fibrosis paru pada model tikus sklerosis sistemik dan mencari dosis optimal ciplukan untuk memperbaiki fibrosis. Penelitian ini terbagai dalam 2 tahap yaitu tahap kuratif fibrosis (tahap 1) dan tahap preventif inflamasi dan fibrosis (tahap 2). Pada tahap 1, 33 tikus (Rattus norvegicus) galur Sprague-Dawley 10−12 minggu dibagi dalam 6 kelompok yaitu kelompok yang mendapat bleomisin dan ciplukan (dosis 50,100,150, dan 200 mg/kg), bleomisin dan salin, dan kontrol negatif. Bleomisin diberikan subkutan per hari selama 14 hari dan ciplukan atau salin diberikan mulai hari ke-21 selama 30 hari lalu hewan diterminasi. Fibrosis dinilai dengan derajat fibrosis dan luas fibrosis pada histopatologi, kadar hidroksiprolin, TGF-β dan MMP13 jaringan paru. Pada tahap 2, 36 ekor tikus dibagi dalam 6 kelompok yaitu 2 kelompok yang mendapat bleomisin dan ciplukan (50 dan 100 mg/kgBB) dan 2 kelompok bleomisin dan salin. Tiga kelompok diterminasi di H14 dan 3 kelompok di H51. Pada tahap 2, bleomisin dan ciplukan diberikan bersamaan selama 14 hari pertama. Luaran yang dinilai di H14 adalah kadar IL-6 paru, jumlah leukosit dari BAL dan skor inflamasi paru secara histopatologi. Luaran yang dinilai di H52 adalah derajat fibrosis dan luas fibrosis, kadar hidroksiprolin, TGF-β dan MMP13 paru. Kadar IL-6, TGF-β dan MMP 13 dinilai dengan ELISA dari jaringan paru, Hidroksiprolin dinilai dari jaringan paru dengan metode kolorimetri. Pada tahap 1 terdapat perbedaan luas fibrosis yang secara statistik bermakna antara kelompok yang mendapat ciplukan dosis 100, 150, dan 200 mg/kgBB dibandingkan kelompok bleomisin. Tidak terdapat perbedaan skor fibrosis antara kelompok yang mendapat ciplukan 50, 100, dan 150 mg/kgBB dengan kontrol negatif. Tidak terdapat perbedaan hidroksiprolin antara kelompok yang mendapat ciplukan dengan kontrol negatif. Tidak terdapat perbedaan kadar TGF-β dan MMP13 yang secara statistik bermakna antar kelompok. Pada tahap 2 penelitian tidak didapatkan perbedaan kadar IL-6, jumlah leukosit cairan BAL dan skor inflamasi yang bermakna antar kelompok dan tidak terdapat perbedaan skor fibrosis, luas fibrosis, kadar hidroksiprolin, TGF-β dan MMP13 antar kelompok. Sebagai simpulan ekstrak ciplukan memiliki efek kuratif untuk menurunkan luas fibrosis paru dengan dosis optimal 100 mg/kgBB. Ciplukan tidak memiliki efek preventif terhadap inflamasi dan fibrosis.

---

Systemic sclerosis or scleroderma is an immune mediated connective tissue disease which is manifested by fibrosis on skin and internal organ and vasculopathy. Interstitial lung disease (ILD) is the main cause of death of systemic sclerosis however the treatment of ILD in systemic sclerosis is still unsatisfactory. Ciplukan (Physalis angulata) herb is a potential alternative treatment for systemic sclerosis and has been proven to improve skin sclerosis in systemic sclerosis patients however the study on its effect on lung has been lacking. The aim of this study is to evaluate the effect of ciplukan herb for treating and preventing inflammation and fibrosis in systemic sclerosis animal model and to find out its optimal dose in improving lung fibrosis. This study was done in 2 stages. For the first stage (treatment of fibrosis), 33 Sprague-Dawley rats aged 10−12 weeks were divided into 6 groups (4 groups were given bleomycin and ciplukan extract dose 50,100,150, and 200 mg/kgBW, respectively, bleomycin and saline and negative control). Bleomycin was given subcutaneously daily for 14 days and ciplukan or saline were given from day 21 until the next 30 days and then the animals were sacrificed. At the end of observation, degree of fibrosis and width of fibrosis from lung histopathology, hydroxyproline, TGF-β, and MMP13 levels were analyzed. For the second stage (prevention), 36 rats were divided into 6 groups (bleomycin and ciplukan dose 50 and 100 mg/kgBW, and bleomycin only). Three groups were sacrificed after 14 days of observation for evaluation of IL-6 level in lung tissue, leucocyte count on BAL fluid and inflammation score. Three groups were sacrificed after 51 days observation and were analyzed for degree of fibrosis and width of fibrosis from lung histopathology, hydroxyproline, TGF-β, and MMP13 levels. For the second stage, bleomycin and ciplukan were given simultaneously for 14 days. IL-6, TGF-β, and MMP13 levels were measured using ELISA methods while hydroxyproline was analyzed using colorimetric method. From the stage 1, there was a significant reduction in width of lung fibrosis on groups receiving bleomycin and ciplukan dose 100, 150, and 200 mg/kgBW compared with bleomycin group. There was no difference of fibrosis score among groups who received ciplukan 50,100, and 150 mg/kgBW compared to the negative control. There was no difference of hydroxyproline among groups who received ciplukan compared with negative control. There was no difference of TGF-β, and MMP13 levels among groups. From the stage 2, there were no difference of IL-6 levels, BAL leukocyte count and inflammation score among groups after 14 days and no difference of fibrosis score, extension of fibrosis, hydroxyproline, TGF-β and MMP13 levels among groups after 51 days observation. As a conclusion, ciplukan herb has a role as a treatment of fibrosis to reduce extent of lung fibrosis with optimal dose of 100 kg/BW but shows no effect on prevention of lung inflammation and lung fibrosis."

"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."