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2017 ; 11
(8
): 327-340
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Evaluation and management approaches for scleroderma lung disease
#MMPMID28621173
Schoenfeld SR
; Castelino FV
Ther Adv Respir Dis
2017[Aug]; 11
(8
): 327-340
PMID28621173
show ga
Interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are
leading causes of morbidity and mortality in systemic sclerosis (SSc). As
symptoms are often under-reported in SSc, early screening of ILD and PAH is of
paramount importance, and early treatment may be associated with better clinical
outcomes. Serologies are particularly helpful in identifying patients at risk for
pulmonary involvement. Pulmonary function testing, high-resolution computed
tomography of the chest and echocardiography are important tools in the initial
screening of these patients. Extensive research has also led to an improved
understanding of the mediators involved in the pathogenesis of ILD and PAH. As a
result, there have been significant advances in the development of novel targeted
therapeutics and an increase in the number of early-phase clinical trials in SSc.