906
Views
109
CrossRef citations to date
0
Altmetric
Original Research

Epidemiology of systemic sclerosis and systemic sclerosis-associated interstitial lung disease

, , &
Pages 257-273 | Published online: 18 Apr 2019

References

  • Avina-Zubieta JA, Sayre EC, Bernatsky S, et al. Adult Prevalence of Systemic Autoimmune Rheumatic Diseases (SARDs) in British Columbia, Canada [abstract]. Arthritis Rheum 2011;63(Suppl 10):S719
  • Hoffmann-Vold A-M, Midtvedt Ø, Molberg Ø, Garen T, Gran JT. Prevalence of systemic sclerosis in South-East Norway. Rheumatology (Oxford). 2012;51(9):1600–1605. doi:10.1093/rheumatology/ker15422539477
  • Ho YY, Lagares D, Tager AM, Kapoor M. Fibrosis—a lethal component of systemic sclerosis. Nat Rev Rheumatol. 2014;10(7):390–402. doi:10.1038/nrrheum.2014.5324752182
  • Subcommittee for Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum. 1980;23(5):581–590. doi:10.1002/art.17802305107378088
  • LeRoy EC, Black C, Fleischmajer R, et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol. 1988;15(2):202–205.3361530
  • LeRoy EC, Medsger TA Jr. Criteria for the classification of early systemic sclerosis. J Rheumatol. 2001;28(7):1573–1576.11469464
  • Arias-Nuñez MC, Llorca J, Vazquez-Rodriguez TR, et al. Systemic sclerosis in northwestern Spain: a 19-year epidemiologic study. Medicine (Baltimore). 2008;87(5):272–280. doi:10.1097/MD.0b013e318189372f18794710
  • Romanowska-Próchnicka K, Walczyk M, Olesinska M. Recognizing systemic sclerosis: comparative analysis of various sets of classification criteria. Reumatologia. 2016;54(6):296–305. doi:10.5114/reum.2016.6490628115780
  • Jaeger VK, Wirz EG, Allanore Y, et al. Incidences and risk factors of organ manifestations in the early course of systemic sclerosis: a longitudinal EUSTAR study. PLoS One. 2016;11(10):e0163894. doi:10.1371/journal.pone.016389427706206
  • McNearney TA, Reveille JD, Fischbach M, et al. Pulmonary involvement in systemic sclerosis: associations with genetic, serologic, sociodemographic, and behavioral factors. Arthritis Rheum. 2007;57(2):318–326. doi:10.1002/art.2253217330281
  • Siméon-Aznar CP, Fonollosa-Plá V, Tolosa-Vilella C, et al. Registry of the Spanish Network for Systemic Sclerosis: clinical pattern according to cutaneous subsets and immunological status. Semin Arthritis Rheum. 2012;41(6):789–800. doi:10.1016/j.semarthrit.2011.10.00422169458
  • Nihtyanova SI, Schreiber BE, Ong VH, et al. Prediction of pulmonary complications and long-term survival in systemic sclerosis. Arthritis Rheumatol. 2014;66(6):1625–1635. doi:10.1002/art.3839024591477
  • Mandhadi R, Lakshminarayanan S. Pulmonary involvement in systemic sclerosis at initial presentation: a single center experience. Arthritis Rheumatol. 2015;67(Suppl 10):S3575–3576.
  • Steele R, Hudson M, Lo E, Baron M. Group obotCSR. Clinical decision rule to predict the presence of interstitial lung disease in systemic sclerosis. Arthritis Care Res (Hoboken). 2012;64(4):519–524. doi:10.1002/acr.2158322213733
  • Strickland G, Pauling J, Cavill C, Shaddick G, McHugh N. Mortality in systemic sclerosis—a single centre study from the UK. Clin Rheumatol. 2013;32(10):1533–1539. doi:10.1007/s10067-013-2289-023812618
  • Vonk MC, Broers B, Heijdra YF, et al. Systemic sclerosis and its pulmonary complications in The Netherlands: an epidemiological study. Ann Rheum Dis. 2009;68(6):961–965. doi:10.1136/ard.2008.09171018511546
  • Kowal-Bielecka O, Landewé R, Avouac J, et al. EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR). Ann Rheum Dis. 2009;68(5):620–628. doi:10.1136/ard.2008.09667719147617
  • Adler S, Huscher D, Siegert E, et al. Systemic sclerosis associated interstitial lung disease - individualized immunosuppressive therapy and course of lung function: results of the EUSTAR group. Arthritis Res Ther. 2018;20(1):17. doi:10.1186/s13075-018-1517-z29382380
  • Silver KC, Silver RM. Management of systemic-sclerosis-associated interstitial lung disease. Rheum Dis Clin North Am. 2015;41(3):439–457. doi:10.1016/j.rdc.2015.04.00626210128
  • Tashkin DP, Roth MD, Clements PJ, et al. Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial. Lancet Respir Med. 2016;4(9):708–719. doi:10.1016/S2213-2600(16)30152-727469583
  • Kowal-Bielecka O, Fransen J, Avouac J, et al. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017;76(8):1327–1339. doi:10.1136/annrheumdis-2016-20990927941129
  • Airò P, Tabaglio E, Frassi M, Scarsi M, Danieli E, Rossi M. Prevalence of systemic sclerosis in Valtrompia in northern Italy. A collaborative study of rheumatologists and general practitioners. Clin Exp Rheumatol. 2007;25(6):878–880.18173923
  • Groseanu L, Saulescu I, Balanescu A, et al. Vitamin D status and clinical significance in a group of scleroderma patients. Ann Rheum Dis. 2013;72(Suppl 3):A647. doi:10.1136/annrheumdis-2013-eular.1922
  • Foti R, Converso G, Benenati A, et al. Iloprost as cyclic six-day per month. Long term efficacy in scleroderma patients. Clin Exp Rheumatol. 2014;32(2 Suppl 81):S113.
  • Furst DE, Fernandes AW, Iorga SR, Greth W, Bancroft T. Epidemiology of systemic sclerosis in a large US managed care population. J Rheumatol. 2012;39(4):784–786. doi:10.3899/jrheum.11110622382343
  • Bernatsky S, Joseph L, Pineau CA, Belisle P, Hudson M, Clarke AE. Scleroderma prevalence: demographic variations in a population-based sample. Arthritis Rheum. 2009;61(3):400–404. doi:10.1002/art.v61:319248123
  • Barnabe C, Joseph L, Edworthy S, et al. Scleroderma prevalence in Alberta: a population-based assessment [abstract]. J Rheumatol. 2011;38(6):1151. doi:10.3899/jrheum.100724
  • Fischer A, Molitor JA, Hummers LK, et al. Severe Interstitial Lung Disease within the PHAROS Registry: Baseline Characteristics and Clinical Features [abstract]. Arthritis Rheum. 2011;63(Suppl 10):S269–S270.
  • Alamanos Y, Tsifetaki N, Voulgari PV, et al. Epidemiology of systemic sclerosis in northwest Greece 1981 to 2002. Semin Arthritis Rheum. 2005;34(5):714–720. doi:10.1016/j.semarthrit.2004.08.00415846586
  • Herrick AL, Ennis H, Bhushan M, Silman AJ, Baildam EM. Incidence of childhood linear scleroderma and systemic sclerosis in the UK and Ireland. Arthritis Care Res (Hoboken). 2010;62(2):213–218. doi:10.1002/acr.2007020191520
  • Mayes MD, Lacey JV Jr, Beebe-Dimmer J, et al. Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum. 2003;48(8):2246–2255. doi:10.1002/art.1107312905479
  • Epailly E, Ikic A, Emmanuel C, et al. Heart transplantation in 8 patients with systemic sclerosis: results of a French national registry [abstract]. Transpl Int. 2015;28(Suppl S4):267.
  • Czirják L, Kumánovics G, Varjú C, et al. Survival and causes of death in 366 Hungarian patients with systemic sclerosis. Ann Rheum Dis. 2008;67(1):59–63. doi:10.1136/ard.2006.06634017519276
  • Bouros D, Wells AU, Nicholson AG, et al. Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Crit Care Med. 2002;165(12):1581–1586. doi:10.1164/ajrccm.165.8.210610412070056
  • Navaratnam V, Ali N, Smith CJP, McKeever T, Fogarty A, Hubbard RB. Does the presence of connective tissue disease modify survival in patients with pulmonary fibrosis?. Respir Med. 2011;105(12):1925–1930. doi:10.1016/j.rmed.2011.03.02021924888
  • Al-Dhaher FF, Pope JE, Ouimet JM. Determinants of morbidity and mortality of systemic sclerosis in Canada. Semin Arthritis Rheum. 2010;39(4):269–277. doi:10.1016/j.semarthrit.2008.06.00218706680
  • Ryerson CJ, O’Connor D, Dunne JV, et al. Predicting mortality in systemic sclerosis-associated interstitial lung disease using risk prediction models derived from idiopathic pulmonary fibrosis. Chest. 2015;148(5):1268–1275. doi:10.1378/chest.15-000325996795
  • Morisset J, Elicker BM, Hu X, et al. Predictors of mortality and risk prediction among patients with scleroderma related interstitial lung disease [abstract]. Eur Respir J. 2015;46(Suppl):59.
  • Hao Y, Husdon M, Carreira P, et al. Early mortality in Australian, Canadian and Spanish scleroderma patients: rationale for establishing a multi-national inception cohort of patients with systemic sclerosis [abstract]. Arthritis Rheum. 2014;66(Suppl 10):S316.
  • Le Guern V, Mahr A, Mouthon L, Jeanneret D, Carzon M, Guillevin L. Prevalence of systemic sclerosis in a French multi-ethnic county. Rheumatology (Oxford). 2004;43(9):1129–1137. doi:10.1093/rheumatology/keh25315213329
  • Assayag D, Hudson M, Tatibouet S, et al. Clinical correlates of lung scan abnormalities in patients with systemic sclerosis-associated interstitial lung disease [abstract]. Am J Respir Crit Care Med. 2012;185:A6616.
  • Mulla E, Shaffu S, Hassan W. A comparative study of the difference in clinical manifestations and disease outcomes between South Asian and Caucasian patients with systemic sclerosis in a large NHS trust, within the United Kingdom [abstract]. Ann Rheum Dis. 2015;74(Suppl 2):1128. doi:10.1136/annrheumdis-2015-eular.1996
  • Hoffmann-Vold AM, Molberg Ø, Midtvedt Ø, Garen T, Gran JT. Survival and causes of death in an unselected and complete cohort of Norwegian patients with systemic sclerosis. J Rheumatol. 2013;40(7):1127–1133. doi:10.3899/jrheum.12139023637316
  • Gaultier JB, Hot A, Cathébras P, Grange C, Ninet J, Rousset H. [Systemic sclerosis in men]. Rev Med Interne. 2008;29(3):181–186. doi:10.1016/j.revmed.2007.07.01017976873
  • Fransen J, Popa-Diaconu D, Hesselstrand R, et al. Clinical prediction of 5-year survival in systemic sclerosis: validation of a simple prognostic model in EUSTAR centres. Ann Rheum Dis. 2011;70(10):1788–1792. doi:10.1136/ard.2010.13983221784727
  • Tyndall AJ, Bannert B, Vonk M, et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010;69(10):1809–1815. doi:10.1136/ard.2009.11426420551155
  • Ovalles-Bonilla JG, Martínez-Barrio J, López-Longo F, et al. Survival, causes of death and mortality risk factors in systemic sclerosis [abstract]. Ann Rheum Dis. 2013;71(Suppl 3):402. doi:10.1136/annrheumdis-2012-eular.2717
  • Hachulla E, Carpentier P, Gressin V, et al. Risk factors for death and the 3-year survival of patients with systemic sclerosis: the French ItinérAIR-Sclérodermie study. Rheumatology (Oxford). 2009;48(3):304–308. doi:10.1093/rheumatology/ken48819174571
  • Abhishek A, Yazdani R, Pearce F, et al. Outcome of systemic sclerosis associated interstitial lung disease treated with intravenous cyclophosphamide. Clin Rheumatol. 2011;30(8):1099–1104. doi:10.1007/s10067-011-1734-121484227
  • Fernández-Codina A, Simeón-Aznar CP, Berastegui-García C, et al. Update on lung transplantation in systemic sclerosis in Spain [abstract]. Ann Rheum Dis. 2014;73(Suppl 2):560. doi:10.1136/annrheumdis-2014-205310
  • Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972–2002. Ann Rheum Dis. 2007;66(7):940–944. doi:10.1136/ard.2006.06606817329309
  • Mendoza F, Derk CT. Systemic sclerosis mortality in the United States: 1999–2002 implications for patient care. J Clin Rheumatol. 2007;13(4):187–192. doi:10.1097/RHU.0b013e318124a89e17762451
  • Hao Y, Hudson M, Carreira P, et al. Early mortality in Australian, Canadian and Spanish scleroderma patients: rationale for establishing a multi-national inception cohort of patients with systemic sclerosis In: ACR/ARHP Annual Meeting. Boston, MA, USA1114 19.723 ed2014
  • Chung L, Krishnan E, Chakravarty EF. Hospitalizations and mortality in systemic sclerosis: results from the Nationwide Inpatient Sample. Rheumatology (Oxford). 2007;46(12):1808–1813. doi:10.1093/rheumatology/kem04517986481
  • Nietert PJ, Silverstein MD, Silver RM. Hospital admissions, length of stay, charges, and in-hospital death among patients with systemic sclerosis. J Rheumatol. 2001;28(9):2031–2037.11550971
  • Su R, Bennett M, Jacobs S, et al. An analysis of connective tissue disease-associated interstitial lung disease at a US Tertiary Care Center: better survival in patients with systemic sclerosis. J Rheumatol. 2011;38(4):693–701. doi:10.3899/jrheum.10072421285162
  • Andréasson K, Saxne T, Bergknut C, Hesselstrand R, Englund M. 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. Ann Rheum Dis. 2014;73(10):1788–1792. doi:10.1136/annrheumdis-2014-20531023897770
  • Coral-Alvarado P, Pardo AL, Castaño-Rodriguez N, Rojas-Villarraga A, Anaya J-M. Systemic sclerosis: a world wide global analysis. Clin Rheumatol. 2009;28(7):757–765. doi:10.1007/s10067-009-1144-919277816
  • Allcock RJ, Forrest I, Corris PA, Crook PR, Griffiths ID. A study of the prevalence of systemic sclerosis in northeast England. Rheumatology (Oxford). 2004;43(5):596–602. doi:10.1093/rheumatology/keh12414872101
  • El Adssi H, Cirstea D, Virion JM, Guillemin F, de Korwin JD. Estimating the prevalence of systemic sclerosis in the Lorraine region, France, by the capture-recapture method. Semin Arthritis Rheum. 2013;42(5):530–538. doi:10.1016/j.semarthrit.2012.10.00123290691
  • Radic M, Martinovic Kaliterna D, Fabijanic D, Radic J. Prevalence of systemic sclerosis in Split-Dalmatia county in Southern Croatia. Clin Rheumatol. 2010;29(4):419–421. doi:10.1007/s10067-009-1341-620082237
  • Eaton WW, Pedersen MG, Atladóttir HO, Gregory PE, Rose NR, Mortensen PB. The prevalence of 30 ICD-10 autoimmune diseases in Denmark. Immunol Res. 2010;47(1–3):228–231. doi:10.1007/s12026-009-8153-220066507
  • Helmick CG, Felson DT, Lawrence RC, et al. Estimates of the prevalence of arthritis and other rheumatic conditions in the United States. Part I. Arthritis Rheum. 2008;58(1):15–25. doi:10.1002/art.2379418163481
  • Robinson D Jr, Eisenberg D, Nietert PJ, et al. Systemic sclerosis prevalence and comorbidities in the US, 2001–2002. Curr Med Res Opin. 2008;24(4):1157–1166. doi:10.1185/030079908X28061718430269
  • ACR 1980: Subcommittee for scleroderma criteria of the American Rheumatism Association diagnostic and therapeutics criteria committee. Arthritis Rheum. 1980;23:581–590
  • LeRoy EC, Black C, Fleischmajer R, et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol. 1988;15(2):202–2053361530
  • LeRoy EC, Medsger TA Jr. Criteria for the classification of early systemic sclerosis. J Rheumatol. 2001;28(7):1573–157611469464
  • Van Den Hoogen F, Khanna D, Fransen J, et al. 2013 Classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. Arthritis Rheum. 2013;65(11):2737–274724122180
  • Lo Monaco A, Bruschi M, La Corte R, Volpinari S, Trotta F. Epidemiology of systemic sclerosis in a district of northern Italy. Clin Exp Rheumatol. 2011;29(2 Suppl 65):S10–S14.21586212
  • Kaliterna DM, Radic M, Pavic A. [Incidence, prevalence and disease characteristics of systemic sclerosis in Split-Dalmatia County]. Reumatizam. 2010;57(2):94–98.21875011