Diagnosis and management of connective tissue disease-associated interstitial lung disease in Australia and New Zealand: A position statement from the Thoracic Society of Australia and New Zealand

Abstract

Pulmonary complications in CTD are common and can involve the interstitium, airways, pleura and pulmonary vasculature. ILD can occur in all CTD (CTD-ILD), and may vary from limited, non-progressive lung involvement, to fulminant, life-threatening disease. Given the potential for major adverse outcomes in CTD-ILD, accurate diagnosis, assessment and careful consideration of therapeutic intervention are a priority. Limited data are available to guide management decisions in CTD-ILD. Autoimmune-mediated pulmonary inflammation is considered a key pathobiological pathway in these disorders, and immunosuppressive therapy is generally regarded the cornerstone of treatment for severe and/or progressive CTD-ILD. However, the natural history of CTD-ILD in individual patients can be difficult to predict, and deciding who to treat, when and with what agent can be challenging. Establishing realistic therapeutic goals from both the patient and clinician perspective requires considerable expertise. The document aims to provide a framework for clinicians to aid in the assessment and management of ILD in the major CTD. A suggested approach to diagnosis and monitoring of CTD-ILD and, where available, evidence-based, disease-specific approaches to treatment have been provided.

Keywords: clinical diagnosis and management; collagen vascular disease; connective tissue disease; interstitial lung disease.

Figure 1

Common radiological patterns of interstitial lung disease (ILD) in connective tissue disease (CTD). (A) Non‐specific interstitial pneumonia (NSIP): ground‐glass change and limited reticulation; predominantly basal, subpleural distribution. (B) Usual interstitial pneumonia (UIP): subpleural, basal predominant reticulation with honeycomb change. No ground‐glass change, nodules or air‐trapping (features ‘inconsistent’ with radiological UIP). (C) Organizing pneumonia (OP): dense, patchy predominantly peribronchial consolidation affecting the lower lobes bilaterally and symmetrically. (D) Acute interstitial pneumonia (AIP): widespread ground‐glass infiltrates in a patient with previously undiagnosed systemic lupus erythematosus (SLE) presenting with acute respiratory failure. (E) Lymphocytic interstitial pneumonia (LIP): variably sized thin‐walled cysts. Ground‐glass change and centrilobular nodules may also occur (not seen here).

Figure 2

Suggested algorithm for the diagnosis and management of connective tissue disease‐associated interstitial lung disease (CTD‐ILD). ^†For detailed discussion, see the section ‘Deciding when to treat’.

Figure 3

(A) A simple staging system for systemic sclerosis‐associated interstitial lung disease (SSc‐ILD). (B) Survival curves for SSc‐ILD patients with ‘limited’ versus ‘extensive’ disease (hazard ratio (HR) = 3.46; P < 0.0005) (Adapted from Goh et al., ⁵⁴ with permission).