[HTML][HTML] Unraveling SSc pathophysiology; the myofibroblast

A van Caam, M Vonk, F van den Hoogen… - Frontiers in …, 2018 - frontiersin.org
Systemic sclerosis (SSc) is a severe auto-immune disease, characterized by vasculopathy
and fibrosis of connective tissues. SSc has a high morbidity and mortality and unfortunately …

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 …

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 …

[PDF][PDF] One year in review 2018: systemic sclerosis

M Orlandi, S Barsotti, G Lepri, V Codullo… - Clin Exp …, 2018 - researchgate.net
Systemic sclerosis is a rare acquired systemic disease characterised by a complex
pathogenesis and multi organ involvement. Every year the scientific world contributes to …

Pirfenidone in patients with unclassifiable progressive fibrosing interstitial lung disease: design of a double-blind, randomised, placebo-controlled phase II trial

TM Maher, TJ Corte, A Fischer… - BMJ open …, 2018 - bmjopenrespres.bmj.com
Introduction Despite extensive multidisciplinary team (MDT) assessment, some patients
have interstitial lung disease (ILD) that is considered unclassifiable (uILD), for which there …

Radiographic fibrosis score predicts survival in systemic sclerosis‐associated interstitial lung disease

R Takei, M Arita, S Kumagai, Y Ito, F Tokioka… - …, 2018 - Wiley Online Library
Background and objective Interstitial lung disease (ILD) is a common pulmonary
manifestation of systemic sclerosis (SSc). It is unknown whether radiographic fibrosis score …

New treatment paradigms for connective tissue disease-associated interstitial lung disease

V Kouranos, G Miranda, TJ Corte… - Current Opinion in …, 2018 - journals.lww.com
New treatment paradigms for connective tissue disease-associ... : Current Opinion in Pulmonary
Medicine New treatment paradigms for connective tissue disease-associated interstitial lung …

Efficacy and safety of combination therapy with prednisolone and oral tacrolimus for progressive interstitial pneumonia with systemic sclerosis: a retrospective study

J Konma, T Kotani, T Shoda, T Suzuka… - Modern …, 2018 - academic.oup.com
Objectives: We retrospectively investigated efficacy and safety of combination therapy with
prednisolone (PSL) and tacrolimus (TAC) for progressive interstitial pneumonitis with …

Anti-fibrotic nintedanib—a new opportunity for systemic sclerosis patients?

AC Duarte, MJ Santos, A Cordeiro - Clinical Rheumatology, 2018 - Springer
Systemic sclerosis is a connective tissue disease characterized by progressive skin
thickening and a wide spectrum of internal organ involvement. Pathogenesis includes …