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Combined Interstitial Lung Disease and Pulmonary Hypertension in Systemic Sclerosis: Pathophysiology and Management

Hidekata Yasuoka, MD, PhD, and Masataka Kuwana, MD, PhD

Systemic sclerosis (SSc), also known as scleroderma, is a connective tissue disease (CTD) characterized by excessive fibrosis, microvasculopathy, and autoimmunity. Typical histological changes are found in multiple organs, including the skin, gastrointestinal tract, lungs, heart, and kidneys. Lung involvement is a major cause of morbidity and mortality in SSc. In fact, University of Pittsburgh (Pittsburgh, PA, USA) database records of SSc-related mortality show interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) as the two major causes of death [1]. Thus, to improve SSc patient survival, it is imperative to suppress the progression of ILD and PAH. Currently, treatment for SSc-associated ILD is limited to immunosuppressive agents. A recent randomized placebo-controlled trial (the Scleroderma Lung Study) reported that oral cyclophosphamide provides a modest but significant benefit in terms of lung function and health-related quality of life (QoL) in SSc patients with active ILD [2]. Molecularly targeted drugs for PAH can greatly benefit patients with SSc-associated PAH, improving symptoms, QoL, hemodynamics, and survival in comparison with historical controls [3]. However, it is still unclear whether these new treatments will improve long-term prognosis [4].

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