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Treatment of Systemic Sclerosis-related Interstitial Lung Disease: A Review of
Existing and Emerging Therapies
#MMPMID27560196
Volkmann ER
; Tashkin DP
Ann Am Thorac Soc
2016[Nov]; 13
(11
): 2045-2056
PMID27560196
show ga
Although interstitial lung disease accounts for the majority of deaths of
patients with systemic sclerosis, treatment options for this manifestation of the
disease are limited. Few high-quality, randomized, controlled trials exist for
systemic sclerosis-related interstitial lung disease, and historically, studies
have favored the use of cyclophosphamide. However, the benefit of
cyclophosphamide for this disease is tempered by its complex adverse event
profile. More recent studies have demonstrated the effectiveness of mycophenolate
for systemic sclerosis-related interstitial lung disease, including Scleroderma
Lung Study II. This review highlights the findings of this study, which was the
first randomized controlled trial to compare cyclophosphamide with mycophenolate
for the treatment of systemic sclerosis-related interstitial lung disease. The
results reported in this trial suggest that there is no difference in treatment
efficacy between mycophenolate and cyclophosphamide; however, mycophenolate
appears to be safer and more tolerable than cyclophosphamide. In light of the
ongoing advances in our understanding of the pathogenic mechanisms underlying
interstitial lung disease in systemic sclerosis, this review also summarizes
novel treatment approaches, presenting clinical and preclinical evidence for
rituximab, tocilizumab, pirfenidone, and nintedanib, as well as hematopoietic
stem cell transplantation and lung transplantation. This review further explores
how reaching a consensus on appropriate study end points, as well as trial
enrichment criteria, is central to improving our ability to judiciously evaluate
the safety and efficacy of emerging experimental therapies for systemic
sclerosis-related interstitial lung disease.
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