Systemic sclerosis: An update in 2016

Abstract

Systemic sclerosis (SSc) is a chronic immune disorder of unknown origin, dominated by excessive fibrosis responsible for cutaneous and pulmonary fibrosis, and by vascular endothelial dysfunction at the origin of skin ischemia, renal and pulmonary artery lesions. Renal and pulmonary complications are mainly responsible for the severity of the disease. Recent advances led to a better understanding of pathological mechanisms and a more accurate classification of patients according to clinical and biological (auto-antibodies) phenotype. Recent trials provided interesting data on different therapeutic strategies, depending on organ involvement. These data are of particular importance in such disease, still characterized by increased mortality and morbidity rates. In this review, we aim to synthetize recent advances in diagnosis and prognosis leading to better classification of SSc patients, and in therapeutic management.

Notch in fibrosis and as a target of anti-fibrotic therapy

“…Fibroblast proliferation, emergence of myofibroblasts, ECM deposition and tissue remodeling are additional key features [1]. Chronic progressive fibrosis can occur in virtually all organs including the lung[2], kidney[3], liver[4], skin[5] and heart[6]. It is commonly a result of excessive, prolonged or repeated injury with associated chronic inflammation [1-6].…”

Integrated, multicohort analysis of systemic sclerosis identifies robust transcriptional signature of disease severity

“…SSc is associated with high disease-related mortality with no known cures (1). Although SSc patients with progressive skin disease are currently prescribed many treatments, including immunosuppressants such as cyclophosphamide, methotrexate, and mycophenolate mofetil (MMF), only a subset of patients appears to improve during therapy (2).…”

Diagnosis and treatment of interstitial lung disease in scleroderma systematica

“…Так, наличие анти-Scl-70 (у 20-40% больных) указывает на диф-фузную форму болезни, быстропрогрессирующее течение и высокий риск тяжелого ИПЛ, а обнаружение АЦА (у 16-39% пациентов) -на лимитированную форму с длитель-ным изолированным синдромом Рейно с отсутствием тяже-лого ИПЛ и поражения почек, но с высоким риском разви-тия ЛАГ. Клинико-иммунологические ассоциации имеют важное значение для установления клинической формы ССД на раннем этапе (диффузная или лимитированная), прогнозирования развития органной патологии (ИПЛ или ЛАГ), определения темпов прогрессирования и прогноза [7]. Важность выявления «склеродермических» аАТ для диагно-стики ССД подчеркивает то, что они включены в новые классификационные критерии заболевания [8].…”