Management of systemic sclerosis-associated interstitial lung disease

Abstract

Purpose of review: To review the recently published data and provide a practical overview for management of systemic sclerosis-interstitial lung disease (SSc-ILD).

Recent findings: Published evidence shows considerable practitioner variability in screening patients for ILD. Recent published data support use of cyclophosphamide or mycophenolate mofetil as first-line treatment of SSc-ILD. For patients not responding to first-line therapies, consideration is given to rituximab as rescue therapy. Recent trials of hematopoietic autologous stem cell transplantation have demonstrated benefit in patients with progressive SSc-ILD. Antifibrotic agents are approved in idiopathic pulmonary fibrosis; studies with antifibrotics are underway for SSc-ILD.

Summary: The specter of rapidly progressive lung disease requires clinicians to risk stratify patients according to known predictors for progression and rigorously monitor for symptoms and advancing disease. The abovementioned therapies promise improved efficacy and favorable side-effect profiles compared to cyclophosphamide.

Figure 1:. Screening and Monitoring Algorithm for ILD in Patients with SSc

* Lower limit of normal ** Clinically meaningful decline defined as FVC levels of >10% from baseline or decline in FVC ≥ 5% to < 10% and ≥ 15% relative decline in DLCO @ Other causes of desaturation such as pulmonary hypertension should be ruled out + Based on Sircar et al., 2018 ! Based on Khanna et al., 2016^,

Figure 2:. Work-up for SSc-ILD Patients with Worsening Respiratory Symptoms

* Based on Coghlan et al., 2015 WSPH World Symposium on Pulmonary Hypertension PH Pulmonary Hypertension

Figure 3:. HRCT in SSc-ILD

Inspiratory and prone HRCT demonstrating minimal reticulation, subpleural groundglass opacity (↑) that persists on prone imaging (↓) suggestive of interstitial lung abnormalities and an early fibrotic lung disease. No honeycombing or traction bronchiectasis. Note slightly dilated esophagus (*).

Figure 4:. Non-Specific Interstitial Pneumonitis Pattern

Lower lung predominant homogeneously distributed ground glass opacity, reticulation, traction bronchiectasis(↑) and dilated esophagus (*)without honeycombing. Appearances are compatible with scleroderma related interstitial lung disease (NSIP pattern).

Figure 5:. Usual Interstitial Pneumonitis Pattern

Heterogeneously distributed interstitial lung disease with honeycombing (↑) compatible with usual interstitial pneumonitis due to scleroderma, note dilated esophagus (*).