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Interstitial Lung Disease in Systemic Sclerosis

A Simple Staging System

¹ Royal Brompton Hospital and National Heart and Lung Institute, London, United Kingdom; ² King's College Hospital, London, United Kingdom; ³ Hammersmith Hospital, London, United Kingdom; and ⁴ Royal Free Hospital, London, United Kingdom

Correspondence and requests for reprints should be addressed to Athol U. Wells, M.D., Interstitial Lung Disease Unit, Royal Brompton Hospital and NHLI, Imperial College, Emmanuel Kaye Building, 1B Manresa Road, London SW3 6LP, UK. E-mail: a.wells{at}rbht.nhs.uk

Rationale: In interstitial lung disease complicating systemic sclerosis (SSc-ILD), the optimal prognostic use of baseline pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) is uncertain.

Objectives: To construct a readily applicable prognostic algorithm in SSc-ILD, integrating PFTs and HRCT.

Methods: The prognostic value of baseline PFT and HRCT variables was quantified in patients with SSc-ILD (n = 215) against survival and serial PFT data.

Measurements and Main Results: Increasingly extensive disease on HRCT was a powerful predictor of mortality (P < 0.0005), with an optimal extent threshold of 20%. In patients with HRCT extent of 10–30% (termed indeterminate disease), an FVC threshold of 70% was an adequate prognostic substitute. On the basis of these observations, SSc-ILD was staged as limited disease (minimal disease on HRCT or, in indeterminate cases, FVC 70%) or extensive disease (severe disease on HRCT or, in indeterminate cases, FVC < 70%). This system (hazards ratio [HR], 3.46; 95% confidence interval [CI], 2.19–5.46; P < 0.0005) was more discriminatory than an HRCT threshold of 20% (HR, 2.48; 95% CI, 1.57–3.92; P < 0.0005) or an FVC threshold of 70% (HR, 2.11; 95% CI, 1.34–3.32; P = 0.001). The system was evaluated by four trainees and four practitioners, with minimal and severe disease on HRCT defined as clearly < 20% or clearly > 20%, respectively, and the use of an FVC threshold of 70% in indeterminate cases. The staging system was predictive of mortality for all scorers, with prognostic separation higher for practitioners (HR, 3.39–3.82) than trainees (HR, 1.87–2.60).

Conclusions: An easily applicable limited/extensive staging system for SSc-ILD, based on combined evaluation with HRCT and PFTs, provides discriminatory prognostic information.

Key Words: prognosis • high-resolution computed tomography • pulmonary function test • limited • extensive

AT A GLANCE COMMENTARY Scientific Knowledge on the Subject Optimal evaluation of prognosis in interstitial lung disease complicating systemic sclerosis, using a combination of high-resolution computed tomography and pulmonary function tests, is currently unavailable. What This Study Adds to the Field A combination of high-resolution computed tomography and pulmonary function test data, constructing a simple staging algorithm, is applicable to routine clinical practice and provides discriminatory prognostic information for interstitial lung disease due to systemic sclerosis.

 

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