Systemic sclerosis

ER Volkmann, K Andréasson, V Smith - The Lancet, 2023 - thelancet.com
Systemic sclerosis, also known as scleroderma, is a rare and complex autoimmune
connective-tissue disease. Once considered an untreatable and unpredictable condition …

2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: Developed by the task force for the diagnosis and treatment of pulmonary …

M Humbert, G Kovacs, MM Hoeper… - European heart …, 2022 - academic.oup.com
Pulmonary hypertension (PH) is a pathophysiological disorder that may involve multiple
clinical conditions and may be associated with a variety of cardiovascular and respiratory …

Statement on imaging and pulmonary hypertension from the Pulmonary Vascular Research Institute (PVRI)

DG Kiely, DL Levin, PM Hassoun, D Ivy… - Pulmonary …, 2019 - journals.sagepub.com
Pulmonary hypertension (PH) is highly heterogeneous and despite treatment advances it
remains a life-shortening condition. There have been significant advances in imaging …

Pulmonary hypertension phenotypes in patients with systemic sclerosis

A Haque, DG Kiely, G Kovacs… - European …, 2021 - Eur Respiratory Soc
Pulmonary hypertension (PH) commonly affects patients with systemic sclerosis (SSc) and is
associated with significant morbidity and increased mortality. PH is a heterogenous …

Right ventricular myofilament functional differences in humans with systemic sclerosis–associated versus idiopathic pulmonary arterial hypertension

S Hsu, KM Kokkonen-Simon, JA Kirk, TM Kolb… - Circulation, 2018 - Am Heart Assoc
Background: Patients with systemic sclerosis (SSc)–associated pulmonary arterial
hypertension (PAH) have a far worse prognosis than those with idiopathic PAH (IPAH). In …

Incidence of pulmonary hypertension and determining factors in patients with systemic sclerosis

JG Coghlan, M Wolf, O Distler… - European respiratory …, 2018 - Eur Respiratory Soc
The objective of this study was to evaluate the incidence of pulmonary hypertension (PH)
and determining factors in patients with systemic sclerosis (SSc) and a diffusing capacity of …

Long-term outcomes in systemic sclerosis-associated pulmonary arterial hypertension from the Pulmonary Hypertension Assessment and Recognition of Outcomes in …

KD Kolstad, S Li, V Steen, L Chung, MB Bolster… - Chest, 2018 - Elsevier
Background Pulmonary arterial hypertension (PAH) is a leading cause of death in patients
with systemic sclerosis (SSc). The purpose of this study was to assess long-term outcomes …

Major lung complications of systemic sclerosis

CP Denton, AU Wells, JG Coghlan - Nature Reviews Rheumatology, 2018 - nature.com
Systemic sclerosis (SSc) is associated with high mortality owing to internal organ
complications, and lung disease is the leading cause of SSc-associated death. The most …

Haemodynamics and serial risk assessment in systemic sclerosis associated pulmonary arterial hypertension

J Weatherald, A Boucly, D Launay… - European …, 2018 - Eur Respiratory Soc
The prognostic importance of follow-up haemodynamics and the validity of multidimensional
risk assessment are not well established for systemic sclerosis (SSc)-associated pulmonary …

Clinical–radiological–pathological correlation in pulmonary arterial hypertension

R Condliffe, C Durrington, A Hameed… - European …, 2023 - Eur Respiratory Soc
Pulmonary hypertension (PH) is defined by the presence of a mean pulmonary arterial
pressure> 20 mmHg. Current guidelines describe five groups of PH with shared …