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"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.

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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.."

"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."

"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.

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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."