Tracking Impact of Interstitial Lung Disease in Systemic Sclerosis in a Complete Nationwide Cohort

被引:167
作者
Hoffmann-Vold, Anna-Maria [1 ,2 ]
Fretheim, Havard [1 ,2 ]
Halse, Anne-Kristine [3 ]
Seip, Marit [4 ]
Bitter, Helle [5 ]
Wallenius, Marianne [6 ,7 ]
Garen, Torhild [1 ]
Salberg, Anne [8 ]
Brunborg, Cathrine [9 ]
Midtvedt, Oyvind [1 ]
Lund, May Brit [2 ,10 ]
Aalokken, Trond M. [11 ]
Molberg, Oyvind [1 ,2 ]
机构
[1] Oslo Univ Hosp, Dept Rheumatol, Oslo, Norway
[2] Univ Oslo, Inst Clin Med, Oslo, Norway
[3] Haukeland Hosp, Dept Rheumatol, Bergen, Norway
[4] Univ Hosp North Norway, Dept Rheumatol, Tromso, Norway
[5] Hosp Southern Norway, Dept Rheumatol, Kristiansand, Norway
[6] Trondheim Reg & Univ Hosp, Dept Rheumatol, Norwegian Natl Advisory Unit Pregnancy & Rheumat, Trondheim, Norway
[7] Norwegian Univ Sci & Technol, Inst Neuromed & Movement Sci, Trondheim, Norway
[8] Lillehammer Hosp, Dept Rheumatol, Lillehammer, Norway
[9] Oslo Univ Hosp, Oslo Ctr Biostat & Epidemiol, Res Support Serv, Oslo, Norway
[10] Oslo Univ Hosp, Dept Resp Med, Oslo, Norway
[11] Oslo Univ Hosp, Dept Radiol & Nucl Med, Oslo, Norway
关键词
systemic sclerosis; epidemiology; pulmonary fibrosis; autoimmune disease; RESOLUTION COMPUTED-TOMOGRAPHY; EULAR SCLERODERMA TRIALS; FORCED VITAL CAPACITY; PULMONARY-HYPERTENSION; FIBROSING ALVEOLITIS; FUNCTION TESTS; MORTALITY; PREDICTOR; SURVIVAL; CYCLOPHOSPHAMIDE;
D O I
10.1164/rccm.201903-0486OC
中图分类号
R4 [临床医学];
学科分类号
1002 ; 100602 ;
摘要
Rationale: Interstitial lung disease (ILD) represents a major challenge in systemic sclerosis (SSc), but there are no precise, population-based data on its overall impact, limiting opportunities for screening and management strategies. Objectives: Evaluate impact of ILD in a unique, nationwide, population-based SSc cohort. Methods: ILD was assessed prospectively in the Norwegian SSc (Nor-SSc) cohort, including all 815 patients with SSc resident in the country from 2000 to 2012. Lung high-resolution computed tomography (HRCT) scans were available for fibrosis quantification at baseline (n = 650, 80%) and follow-up. Pulmonary function tests were assessed at baseline (n = 703, 86%) and follow-up. Vital status and standardized mortality ratios (SMRs) were estimated at study end (2018) in the 630 incident Nor-SSc cases and 15 individually matched control subjects. Cumulative survival rates were computed. Measurements and Main Results: At baseline, 50% of the subjects with SSc (n = 324) had ILD by HRCT and 46% displayed pulmonary function declines consistent with ILD progression. Mortality correlated with extent of lung fibrosis as SMR increased from 2.2 with no fibrosis to 8.0 with greater than 25% fibrosis. SMR was inversely related to baseline FVC% and increased at all FVC levels below 100%. In patients with normal-range baseline FVC (80-100%), the 5- and 10-year survival rates correlated with presence or absence of lung fibrosis, being 83% and 80%, respectively, with no fibrosis and 69% and 56%, respectively, with lung fibrosis (P = 0.03). Conclusions: The mere presence of ILD at baseline appears to affect outcome in SSc, suggesting that all patients with SSc should undergo a baseline pulmonary function test and lung HRCT screening to diagnose ILD early and tailor further management.
引用
收藏
页码:1258 / 1266
页数:9
相关论文
共 52 条
  • [1] Allanore Y, 2012, EULAR TXB RHEUMATIC
  • [2] Systemic sclerosis
    Allanore, Yannick
    Simms, Robert
    Distler, Oliver
    Trojanowska, Maria
    Pope, Janet
    Denton, Christopher P.
    Varga, John
    [J]. NATURE REVIEWS DISEASE PRIMERS, 2015, 1
  • [3] Prevalence and incidence of systemic sclerosis in southern Sweden: population-based data with case ascertainment using the 1980 ARA criteria and the proposed ACR-EULAR classification criteria
    Andreasson, K.
    Saxne, T.
    Bergknut, C.
    Hesselstrand, R.
    Englund, M.
    [J]. ANNALS OF THE RHEUMATIC DISEASES, 2014, 73 (10) : 1788 - 1792
  • [4] Predictors of interstitial lung disease in early systemic sclerosis: a prospective longitudinal study of the GENISOS cohort
    Assassi, Shervin
    Sharif, Roozbeh
    Lasky, Robert E.
    McNearney, Terry A.
    Estrada-Y-Martin, Rosa M.
    Draeger, Hilda
    Nair, Deepthi K.
    Fritzler, Marvin J.
    Reveille, John D.
    Arnett, Frank C.
    Mayes, Maureen D.
    [J]. ARTHRITIS RESEARCH & THERAPY, 2010, 12 (05)
  • [5] Major lung complications of systemic sclerosis
    Denton, Christopher P.
    Wells, Athol U.
    Coghlan, John G.
    [J]. NATURE REVIEWS RHEUMATOLOGY, 2018, 14 (09) : 511 - 527
  • [6] Systemic sclerosis
    Denton, Christopher P.
    Khanna, Dinesh
    [J]. LANCET, 2017, 390 (10103) : 1685 - 1699
  • [7] Mapping and predicting mortality from systemic sclerosis
    Elhai, Muriel
    Meune, Christophe
    Boubaya, Marouane
    Avouac, Jerome
    Hachulla, Eric
    Balbir-Gurman, Alexandra
    Riemekasten, Gabriela
    Airo, Paolo
    Joven, Beatriz
    Vettori, Serena
    Cozzi, Franco
    Ullman, Susanne
    Czirjak, Laszlo
    Tikly, Mohammed
    Mueller-Ladner, U. L. F.
    Caramaschi, Paola
    Distler, Oliver
    Iannone, Florenzo
    Ananieva, Lidia P.
    Hesselstrand, Roger
    Becvar, Radim
    Gabrielli, Armando
    Damjanov, Nemanja
    Salvador, Maria J.
    Riccieri, Valeria
    Mihai, Carina
    Szucs, Gabriella
    Walker, Ulrich A.
    Hunzelmann, Nicolas
    Martinovic, Duska
    Smith, Vanessa
    Mueller, Carolina de Souza
    Montecucco, Carlo Maurizio
    Opris, Daniela
    Ingegnoli, Francesca
    Vlachoyiannopoulos, Panayiotis G.
    Stamenkovic, Bojana
    Rosato, Edoardo
    Heitmann, Stefan
    Distler, Joerg H. W.
    Zenone, Thierry
    Seidel, Matthias
    Vacca, Alessandra
    De langhe, Ellen
    Novak, Srdan
    Cutolo, Maurizio
    Mouthon, Luc
    Henes, Joerg
    Chizzolini, Carlo
    von Muhlen, Carlos Alberto
    [J]. ANNALS OF THE RHEUMATIC DISEASES, 2017, 76 (11) : 1897 - 1905
  • [8] Challenges in Pulmonary Hypertension: Controversies in Treating the Tip of the Iceberg A Joint National Institutes of Health Clinical Center and Pulmonary Hypertension Association Symposium Report
    Elinoff, Jason M.
    Agarwal, Richa
    Barnett, Christopher F.
    Benza, Raymond L.
    Cuttica, Michael J.
    Gharib, Ahmed M.
    Gray, Michael P.
    Hassoun, Paul M.
    Hemnes, Anna R.
    Humbert, Marc
    Kolb, Todd M.
    Lahm, Tim
    Leopold, Jane A.
    Mathai, Stephen C.
    McLaughlin, Vallerie V.
    Preston, Ioana R.
    Rosenzweig, Erika B.
    Shlobin, Oksana A.
    Steen, Virginia D.
    Zamanian, Roham T.
    Solomon, Michael A.
    [J]. AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, 2018, 198 (02) : 166 - 174
  • [9] Prognostic implications of physiologic and radiographic changes in idiopathic interstitial pneumonia
    Flaherty, KR
    Mumford, JA
    Murray, S
    Kazerooni, EA
    Gross, BH
    Colby, TV
    Travis, WD
    Flint, A
    Toews, GB
    Lynch, JP
    Martinez, FJ
    [J]. AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, 2003, 168 (05) : 543 - 548
  • [10] Impaired quality of life in systemic sclerosis and patient perception of the disease: A large international survey
    Frantz, Camelia
    Avouac, Jerome
    Distler, Oliver
    Amrouche, Fazia
    Godard, Dominique
    Kennedy, Ann Tyrrell
    Connolly, Kerry
    Varga, John
    Matucci-Cerinic, Marco
    Allanore, Yannick
    [J]. SEMINARS IN ARTHRITIS AND RHEUMATISM, 2016, 46 (01) : 115 - 123