Assessment of recent evidence for the management of patients with systemic sclerosis-associated interstitial lung disease: a systematic review

Abstract

This systematic review summarises current evidence to help guide treatment decisions for patients with systemic sclerosis (SSc) associated interstitial lung disease (ILD). A systematic search of the literature (January 2012−April 2018), including grey literature (searched between 1992 and 2011), was conducted using multiple electronic databases. Guidelines, meta-analyses, randomised controlled trials and observational studies reporting on risk stratification, screening, diagnosis, treatment, and management outcomes for patients with SSc-ILD were included. A quality assessment of the included evidence was undertaken.In total, 2464 publications were identified and 280 included. Multiple independent risk factors for ILD in patients with SSc were identified, including older age, male gender and baseline pulmonary function. High-resolution computed tomography (HRCT) has been used for characterising ILD in patients with SSc, and pulmonary function tests are a key adjunctive component in the diagnostic and monitoring pathway. The clinical value of biomarkers relating to SSc-ILD diagnosis or assessment for disease progression is unknown at present. Immunosuppressive therapy (monotherapy or combined therapy) is the current standard of care for SSc-ILD; long-term evidence for effective and safe treatment of SSc-ILD is limited.Identification of patients at risk for SSc-ILD remains challenging. HRCT and pulmonary function tests are key to diagnosing and monitoring for disease progression. Although immunosuppressive therapy is considered current first-line treatment, it is partly associated with adverse effects and long-term follow-up evidence is limited. Novel therapies and biomarkers should be further explored in well-controlled clinical studies.

Systemic Sclerosis–Associated Interstitial Lung Disease: How to Incorporate Two Food and Drug Administration–Approved Therapies in Clinical Practice

“…The main therapeutic agents for the treatment of SSc-ILD have immunomodulatory properties, antifibrotic properties, or both (23). The results of the main phase II and III RCTs and their targeted populations are detailed in Table 2.…”

“…Despite these recent FDA approvals, the optimal therapeutic strategy for the management of SSc-ILD is yet to be determined, especially given the heterogeneity of the disease (23). The objectives of this review are 2-fold: 1) to review the body of research focused on diagnosis and treatment of SSc-ILD and 2) to propose a practical approach for diagnosis, stratification, management, and therapeutic decision-making in this clinical context.…”

Patient Reported Experiences and Delays During the Diagnostic Pathway for Pulmonary Fibrosis: A Multinational European Survey

“…Rapid diagnosis is becoming increasingly important because several treatments are currently available to slow disease progression, improve quality of life, and may extend life expectancy (32)(33)(34). Although there are guidelines and other guidance documents on features, diagnosis, and management of ILD (26,(35)(36)(37) many patients have a diagnosis that is not confirmed by a multidisciplinary discussion and do not receive treatment (38). Additionally, geographical differences that may influence time to diagnosis and access to treatment still exists between countries (10).…”

Advances in the Treatment of Systemic Sclerosis

“…63 As stated earlier, patients who are anti-topoisomerase positive are at especially high risk. 8,64 It is important to diagnose ILD early, so that treatment can be commenced without delay, 65 hence (as with PAH) the rationale for regular screening, with history, examination (listening for late inspiratory crackles) and pulmonary function testing (looking for a restrictive defect and for a fall in the FVC and/or diffusing capacity for carbon monoxide from when last tested). Best practice is for all patients with SSc to have a baseline high-resolution computed tomography scan, 66 which should be repeated if there is clinical concern that ILD might have newly developed or progressed.…”