Background/Purpose:

Systemic Sclerosis (SSc) is a chronic autoimmune disease characterized by fibrosis of skin and internal organs with an estimated worldwide prevalence of 110-430 cases/million. SSc involves the lung, with Interstitial Lung Disease (ILD) being the leading cause of death. The objective of this systematic literature review (SLR) was to review the available scientific evidence to guide decisions on screening; treatment initiation, change or escalation; disease progression and influence on subsequent treatment decisions in SSc-ILD.

Methods:

The SLR was conducted according to NICE, CRD and IQWiG guidance for undertaking reviews in healthcare, and the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) methodology. To update on most recent developments, the search strategy covered literature published from 2016-2018. Population, Intervention, Comparator and Outcomes criteria (PICO) were used to select publications at title/abstract and full-text screening. Data was extracted in six categories: screening/risk stratification; current treatments; treatment initiation/escalation; disease progression; treatment algorithms and biomarkers for ILD in SSc. The quality of evidence extracted was assessed using GRADE 2007 criteria.

Results:

After title and abstract screening of the initial 887 citations found, 260 publications were full-text screened and 163 included in the analysis.

Although evidence was most prolific in the screening/risk stratification category for SSc-ILD, the information extracted in the biomarkers category showed a higher grade of evidence (average medium/high vs medium/low). Although HRCT and DLCO still remain as main screening tools, some biomarkers are already in use for diagnosis and prognosis of SSc-ILD. These include genetic- (ALOX5AP gene polymorphisms), cellular- (neutrophil/lymphocyte ratio), and plasma biomarkers (antibodies such as ATA, ACA, anti-CXT, or chemokines such as CCl18, KL6, CCL-2 or IL-10).

Evidence on lung disease progression was also graded medium/low, and the lowest volume and weakest evidence was observed in the current treatment; treatment algorithms; and treatment initiation/escalation categories. Immunosuppressive drugs (mycophenolate mofetil, cyclophosphamide, azathioprine and rituximab) were used as the recommended therapeutic approach.

Conclusion:

This SLR found that the identification, validation and application of biomarkers as the main field of interest in SSc-ILD research, reflecting progress for early diagnosis and subsequent prognosis of this disease. In contrast, there was a dearth of robust evidence and no clear consensus on therapeutic interventions, highlighting the need for further evidence to support treatment options.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/assessment-of-recent-evidence-to-support-treatment-recommendations-in-patients-with-ssc-ild/