Silje Reiseter1,2, Ragnar Gunnarsson2, Trond Mogens Aaløkken3, May Brit Lund4, Georg Mynarek3, Jukka Corander5, Joanna Haydon6, Øyvind Molberg1,2. 1. Institute of Clinical Medicine, University of Oslo, Norway. 2. Department of Rheumatology, Dermatology and Infectious Diseases, Oslo University Hospital, Rikshospitalet, Norway. 3. Department of Radiology and Nuclear Medicine, Oslo University Hospital Rikshospitalet, Oslo, Norway. 4. Department of Respiratory Medicine, Oslo University Hospital, Norway. 5. Faculty of Medicine, Biostatistics, University of Oslo, Norway. 6. Department of Rheumatology, Vestre Viken Hospital, Drammen, Norway.
Abstract
Objectives: To assess the prevalence, extent, progression, functional impact and mortality of interstitial lung disease (ILD) in a nationwide unselected MCTD cohort. Methods: The study cohort included patients with high-resolution CT lung scans available at baseline (n = 135) and at follow-up (n = 119). The extent of disease was expressed as percentage of total lung volume (TLV). Results: ILD was present in 41% of MCTD patients at follow-up. Median (interquartile) extent (% of TLV) was 5 (8) at baseline and 7 (17) at follow-up, mean length 6.4 years later. The lung disease progressed in 19% of patients across the observation period. Predictors of ILD progression were elevated anti-RNP titre [hazard ratio (HR) 1.5, 95% CI: 1.1, 2.0; P = 0.008], presence of anti-ro52 antibodies (HR = 3.5, 95% CI: 1.2, 10.2; P = 0.023), absence of arthritis (HR = 0.2, 95% CI: 0.1, 0.6; P = 0.004) and male gender (HR = 4.0, 95% CI: 1.4, 11.5; P = 0.011) after age and baseline disease adjustments. The risk of death increased by 2.9 (95% CI: 1.1, 7.9; P = 0.038) in patients where disease involved ⩾5% of TLV. Conclusion: Lung disease extent and progression in MCTD are modest. Yet, the extension continues several years after MCTD diagnosis causing lung function decline and increasing the risk of mortality. The study identified male gender, elevated anti-RNP titre, presence of anti-ro52 antibodies and absence of arthritis as the strongest predictors of ILD progression.
Objectives: To assess the prevalence, extent, progression, functional impact and mortality of interstitial lung disease (ILD) in a nationwide unselected MCTD cohort. Methods: The study cohort included patients with high-resolution CT lung scans available at baseline (n = 135) and at follow-up (n = 119). The extent of disease was expressed as percentage of total lung volume (TLV). Results: ILD was present in 41% of MCTDpatients at follow-up. Median (interquartile) extent (% of TLV) was 5 (8) at baseline and 7 (17) at follow-up, mean length 6.4 years later. The lung disease progressed in 19% of patients across the observation period. Predictors of ILD progression were elevated anti-RNP titre [hazard ratio (HR) 1.5, 95% CI: 1.1, 2.0; P = 0.008], presence of anti-ro52 antibodies (HR = 3.5, 95% CI: 1.2, 10.2; P = 0.023), absence of arthritis (HR = 0.2, 95% CI: 0.1, 0.6; P = 0.004) and male gender (HR = 4.0, 95% CI: 1.4, 11.5; P = 0.011) after age and baseline disease adjustments. The risk of death increased by 2.9 (95% CI: 1.1, 7.9; P = 0.038) in patients where disease involved ⩾5% of TLV. Conclusion:Lung disease extent and progression in MCTD are modest. Yet, the extension continues several years after MCTD diagnosis causing lung function decline and increasing the risk of mortality. The study identified male gender, elevated anti-RNP titre, presence of anti-ro52 antibodies and absence of arthritis as the strongest predictors of ILD progression.
Authors: Eric L Matteson; Clive Kelly; Jörg H W Distler; Anna-Maria Hoffmann-Vold; James R Seibold; Shikha Mittoo; Paul F Dellaripa; Martin Aringer; Janet Pope; Oliver Distler; Alexandra James; Rozsa Schlenker-Herceg; Susanne Stowasser; Manuel Quaresma; Kevin R Flaherty Journal: Arthritis Rheumatol Date: 2022-05-02 Impact factor: 15.483
Authors: Kevin John John; Mohammad Sadiq; Tina George; Karthik Gunasekaran; Nirmal Francis; Ebenezer Rajadurai; Thambu David Sudarsanam Journal: Int J Rheumatol Date: 2020-01-29
Authors: Janine Schniering; Martina Benešová; Matthias Brunner; Stephanie Haller; Susan Cohrs; Thomas Frauenfelder; Bart Vrugt; Carol Feghali-Bostwick; Roger Schibli; Oliver Distler; Cristina Müller; Britta Maurer Journal: Front Immunol Date: 2019-11-22 Impact factor: 7.561