What's in a name? That which we call IPF, by any other name would act the same

AU Wells, KK Brown, KR Flaherty… - European …, 2018 - Eur Respiratory Soc
Idiopathic pulmonary fibrosis (IPF) remains a truly idiopathic fibrotic disease, with a modest
genetic predilection and candidate triggers but no overall explanation for the development of …

Cyclophosphamide for connective tissue disease–associated interstitial lung disease

H Barnes, AE Holland, GP Westall… - Cochrane Database …, 2018 - cochranelibrary.com
Background Approximately one‐third of individuals with interstitial lung disease (ILD) have
associated connective tissue disease (CTD). The connective tissue disorders most …

Safety and efficacy of subcutaneous tocilizumab in systemic sclerosis: results from the open-label period of a phase II randomised controlled trial (faSScinate)

D Khanna, CP Denton, CJF Lin, JM Van Laar… - Annals of the …, 2018 - ard.bmj.com
Objectives Assess the efficacy and safety of tocilizumab in patients with systemic sclerosis
(SSc) in a phase II study. Methods Patients with SSc were treated for 48 weeks in an open …

Intravenous cyclophosphamide vs rituximab for the treatment of early diffuse scleroderma lung disease: open label, randomized, controlled trial

G Sircar, RP Goswami, D Sircar, A Ghosh… - Rheumatology, 2018 - academic.oup.com
Objectives SSc is characterized by fibrotic changes in the skin and lung, and the mainstay of
treatment has been CYC. B cell involvement suggests that rituximab (RTX) may also be of …

Prediction of progression of interstitial lung disease in patients with systemic sclerosis: the SPAR model

W Wu, S Jordan, MO Becker, R Dobrota… - Annals of the …, 2018 - ard.bmj.com
Objectives To identify the predictive clinical characteristics and establish a prediction model
for the progression of mild interstitial lung disease (ILD) in patients with systemic sclerosis …

Pharmacological management of progressive-fibrosing interstitial lung diseases: a review of the current evidence

L Richeldi, F Varone, M Bergna… - European …, 2018 - Eur Respiratory Soc
A proportion of patients with interstitial lung diseases (ILDs) are at risk of developing a
progressive-fibrosing phenotype, which is associated with a deterioration in lung function …

Reliability and minimal clinically important differences of FVC. Results from the Scleroderma Lung Studies (SLS-I and SLS-II)

S Kafaja, PJ Clements, H Wilhalme… - American journal of …, 2018 - atsjournals.org
Rationale: FVC percent predicted (FVC%) is the primary outcome measure in clinical trials of
systemic sclerosis interstitial lung disease. For interpretation of change in the FVC% over …

[HTML][HTML] IL-1-IL-17 signaling axis contributes to fibrosis and inflammation in two different murine models of systemic sclerosis

MJ Park, SJ Moon, EJ Lee, KA Jung, EK Kim… - Frontiers in …, 2018 - frontiersin.org
Objective Systemic sclerosis (SSc) is a progressive fibrotic disease that affects the skin and
internal organs. Despite evidence implicating increased interleukin-17 (IL-17) activity in SSc …

Longitudinal changes in quantitative interstitial lung disease on computed tomography after immunosuppression in the Scleroderma Lung Study II

JG Goldin, GHJ Kim, CH Tseng… - Annals of the …, 2018 - atsjournals.org
Rationale: The Scleroderma Lung Study II (SLS II) demonstrated significant improvements in
pulmonary function and dyspnea at 24 months compared with baseline when patients with …

Interstitial lung disease in systemic autoimmune rheumatic diseases: a comprehensive review

F Atzeni, MC Gerardi, G Barilaro, IF Masala… - Expert review of …, 2018 - Taylor & Francis
ABSTRACT Background: Interstitial lung diseases (ILDs) are among the most serious
complications associated with systemic rheumatic diseases, and lead to significant morbidity …