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Editorial
. 2024 Dec 1;210(11):1293-1295.
doi: 10.1164/rccm.202408-1511ED.

Defining Interstitial Lung Disease Progression: It Is Time to (Get Our) Act Together

Vanessa Smith  1   2   3 , Maurizio Cutolo  4 , Elizabeth Volkmann  5
Affiliations
Editorial

Defining Interstitial Lung Disease Progression: It Is Time to (Get Our) Act Together

Vanessa Smith et al. Am J Respir Crit Care Med. .
No abstract available

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Figures

Figure 1.
Figure 1.
A total of 245 patients with systemic sclerosis (SSc) met the American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria (11). A score of 9 points (p.) is needed to meet the criteria, and each of the following items is associated with a certain number of points: a, skin thickening proximal to the metacarpophalangeal joint (9 p.); b1, puffy fingers (2 p.) or b2, sclerodactyly (4 p.); c1, digital tip ulcers (2 p.) or c2, fingertip pitting scars (3 p.); d, telangiectasia (2 p.); e, abnormal nailfold capillaries (2 p.); f, pulmonary arterial hypertension or interstitial lung disease (2 p.); g, scleroderma-related antibodies (anti-centromere, anti-topoisomerase, or anti–RNA-polymerase; 3 p.); h, Raynaud’s phenomenon (3 p.), categorized according to extension of skin involvement (green dots) into SSc with diffuse or limited cutaneous disease and ≥10% parenchymal involvement on baseline high-resolution computed tomography (HRCT). These were evaluated over a period of 24 months (i.e., the progression assessment period) with four frequent definitions for SSc interstitial lung disease progression. The OMERACT (Outcome Measures in Rheumatology) definition is based on the occurrence of ≥10% relative decrease in predicted FVC or ≥5% to <10% relative decrease in predicted FVC combined with a ≥15% relative decrease in predicted DLCO over 12 months (3). The minimal clinical important worsening definition is based on an absolute reduction of predicted FVC ≥3% over 12 months (4). The ERICE Interstitial Lung Disease Working Group definition is based on a relative decrease in predicted FVC of ≥10% or a relative decline in predicted FVC of ≥5% to <10% associated with worsened respiratory symptoms, an increased extent of fibrosis on HRCT, or a ≥15% relative decrease in DLCO over 24 months (5). Moreover, a worsening of respiratory symptoms was associated with increased fibrosis on HRCT over 24 months was considered as progression independently from the pulmonary function test evolution and the EUSTAR (European Scleroderma Trials and Research Group) definition, which is based on the same pulmonary function test variation cutoffs as the OMERACT definition but uses their absolute variation over a period of 12 months (6, 7, 11). Sixty-month disease-related mortality risk was compared between (non)progressors using the four definitions and is expressed as a hazard ratio when statistically significant. DcSSc = SSc with diffuse cutaneous disease; HR = hazard ratio; ILD = interstitial lung disease; LcSSc = SSc with limited cutaneous disease; MCIW = minimal clinical important worsening.
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Comment on

References

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