Systemic sclerosis

Elizabeth R. Volkmann

doi:10.1016/S0140-6736(22)01692-0

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Systemic sclerosis

2023-01-01

174

Elizabeth R. Volkmann *

Kristofer Andréasson

Vanessa Smith

DOI：10.1016/S0140-6736(22)01692-0

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Abstract

En 中文

Systemic sclerosis, also known as scleroderma, is a rare and complex autoimmune connective-tissue disease. Once considered an untreatable and unpredictable condition, research advancements have improved our understanding of its disease pathogenesis and clinical phenotypes and expanded our treatment armamentarium. Early and accurate diagnosis is essential, while ongoing efforts to risk stratify patients have a central role in predicting both organ involvement and disease progression. A holistic approach is required when choosing the optimal therapeutic strategy, balancing the side-effect profile with efficacy and tailoring the treatment according to the goals of care of the patient. This Seminar reviews the multiple clinical dimensions of systemic sclerosis, beginning at a precursor very early stage of disease, with a focus on timely early detection of organ involvement. This Seminar also summarises management considerations according to the pathological hallmarks of systemic sclerosis (eg, inflammation, fibrosis, and vasculopathy) and highlights unmet needs and opportunities for future research and discovery.

Keywords:

PULMONARY-ARTERIAL-HYPERTENSION

INTERSTITIAL LUNG-DISEASE

STEM-CELL TRANSPLANTATION

CONTINUOUS INTRAVENOUS EPOPROSTENOL

SCLERODERMA RENAL CRISIS

DOUBLE-BLIND

CONNECTIVE-TISSUE

MYCOPHENOLATE-MOFETIL

PROSTACYCLIN ANALOG

SKIN THICKNESS

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