Determinants of Mortality in Systemic Sclerosis: a Focused Review

Overview

Journal Rheumatol Int

Specialty Rheumatology

Date 2017 Nov 9

PMID 29116439

Citations 30

Authors

Dilli Ram Poudel

Divya Jayakumar

Abhijeet Danve

Shiv Tej Sehra

Chris T Derk

Affiliations

Soon will be listed here.

Abstract

Scleroderma (systemic sclerosis) is an autoimmune rheumatic disorder that is characterized by fibrosis, vascular dysfunction, and autoantibody production that involves most visceral organs. It is characterized by a high morbidity and mortality rate, mainly due to disease-related complications. Epidemiological data describing mortality and survival in this population have been based on both population and observational studies. Multiple clinical and non-clinical factors have been found to predict higher likelihood of death among thepatients. Here, we do an extensive review of the available literature, utilizing the PubMed database, to describe scleroderma and non-scleroderma related determinants of mortality in this population. We found that even though the mortality among the general population has declined, scleroderma continues to carry a very high morbidity and mortality rate, however we have made some slow progress in improving the mortality among scleroderma patients over the last few decades.

Citing Articles

Altered X-chromosome inactivation of the TLR7/8 locus and heterogeneity of pDCs in systemic sclerosis.

Du Y, Faz-Lopez B, Ah Kioon M, Cenac C, Pierides M, Lakin K J Exp Med. 2024; 222(3).

PMID: 39670995 PMC: 11639950. DOI: 10.1084/jem.20231809.

Prevalence of hybrid TLR4M2 monocytes/macrophages in peripheral blood and lung of systemic sclerosis patients with interstitial lung disease.

Gotelli E, Soldano S, Feghali-Bostwick C, Montagna P, Campitiello R, Contini P Front Immunol. 2024; 15:1488867.

PMID: 39635531 PMC: 11615060. DOI: 10.3389/fimmu.2024.1488867.

PIK-III exerts anti-fibrotic effects in activated fibroblasts by regulating p38 activation.

Sanchez S, McDowell-Sanchez A, Al-Meerani S, Cala-Garcia J, Waich Cohen A, Ochsner S PLoS One. 2024; 19(9):e0306624.

PMID: 39240940 PMC: 11379285. DOI: 10.1371/journal.pone.0306624.

Unraveling the immunometabolism puzzle: Deciphering systemic sclerosis pathogenesis.

Masoumi M, Bodaghi A, Khorramdelazad H, Ebadi E, Houshmandfar S, Saeedi-Boroujeni A Heliyon. 2024; 10(15):e35445.

PMID: 39170585 PMC: 11336762. DOI: 10.1016/j.heliyon.2024.e35445.

Overlapping Case of Advanced Systemic Sclerosis and IgG4-Related Disease after Autologous Hematopoietic Stem Cell Transplantation.

Dulko A, Butrimiene I, cypiene A, Peceliunas V, Petroska D, Stankeviciene E Medicina (Kaunas). 2024; 60(3).

PMID: 38541223 PMC: 10972323. DOI: 10.3390/medicina60030496.

References

Mathai S, Hummers L, Champion H, Wigley F, Zaiman A, Hassoun P . Survival in pulmonary hypertension associated with the scleroderma spectrum of diseases: impact of interstitial lung disease. Arthritis Rheum. 2009; 60(2):569-77. DOI: 10.1002/art.24267. View

Boin F, Rosen A . Autoimmunity in systemic sclerosis: current concepts. Curr Rheumatol Rep. 2007; 9(2):165-72. DOI: 10.1007/s11926-007-0012-3. View

Kahan A, Allanore Y . Primary myocardial involvement in systemic sclerosis. Rheumatology (Oxford). 2006; 45 Suppl 4:iv14-7. DOI: 10.1093/rheumatology/kel312. View

Nihtyanova S, Schreiber B, Ong V, Rosenberg D, Moinzadeh P, Coghlan J . Prediction of pulmonary complications and long-term survival in systemic sclerosis. Arthritis Rheumatol. 2014; 66(6):1625-35. DOI: 10.1002/art.38390. View

Draeger H, Assassi S, Sharif R, Gonzalez E, Harper B, Arnett F . Right bundle branch block: a predictor of mortality in early systemic sclerosis. PLoS One. 2013; 8(10):e78808. PMC: 3814983. DOI: 10.1371/journal.pone.0078808. View

Hunninghake G, Kalica A . Approaches to the treatment of pulmonary fibrosis. Am J Respir Crit Care Med. 1995; 151(3 Pt 1):915-8. DOI: 10.1164/ajrccm.151.3.7881692. View

Meier F, Frommer K, Dinser R, Walker U, Czirjak L, Denton C . Update on the profile of the EUSTAR cohort: an analysis of the EULAR Scleroderma Trials and Research group database. Ann Rheum Dis. 2012; 71(8):1355-60. DOI: 10.1136/annrheumdis-2011-200742. View

Scotton C, Chambers R . Molecular targets in pulmonary fibrosis: the myofibroblast in focus. Chest. 2007; 132(4):1311-21. DOI: 10.1378/chest.06-2568. View

Chu S, Chen Y, Liu C, Tseng W, Lin M, Hwang C . Increased risk of acute myocardial infarction in systemic sclerosis: a nationwide population-based study. Am J Med. 2013; 126(11):982-8. DOI: 10.1016/j.amjmed.2013.06.025. View

10.

Walsh S, Fenster J . Geographical clustering of mortality from systemic sclerosis in the Southeastern United States, 1981-90. J Rheumatol. 1998; 24(12):2348-52. View

11.

Mukerjee D, St George D, Coleiro B, Knight C, Denton C, Davar J . Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis. 2003; 62(11):1088-93. PMC: 1754353. DOI: 10.1136/ard.62.11.1088. View

12.

DeMarco P, Weisman M, Seibold J, Furst D, Wong W, Hurwitz E . Predictors and outcomes of scleroderma renal crisis: the high-dose versus low-dose D-penicillamine in early diffuse systemic sclerosis trial. Arthritis Rheum. 2002; 46(11):2983-9. DOI: 10.1002/art.10589. View

13.

Becker M, Riemekasten G . Risk factors for severity and manifestations in systemic sclerosis and prediction of disease course. Expert Rev Clin Immunol. 2015; 12(2):115-35. DOI: 10.1586/1744666X.2016.1115717. View

14.

Fisher M, Mathai S, Champion H, Girgis R, Housten-Harris T, Hummers L . Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum. 2006; 54(9):3043-50. DOI: 10.1002/art.22069. View

15.

Steen V, Medsger Jr T . Long-term outcomes of scleroderma renal crisis. Ann Intern Med. 2000; 133(8):600-3. DOI: 10.7326/0003-4819-133-8-200010170-00010. View

16.

Kohno K, Katayama T, Majima K, Fujisawa M, Iida S, Fukami K . A case of normotensive scleroderma renal crisis after high-dose methylprednisolone treatment. Clin Nephrol. 2000; 53(6):479-82. View

17.

Meune C, Vignaux O, Kahan A, Allanore Y . Heart involvement in systemic sclerosis: evolving concept and diagnostic methodologies. Arch Cardiovasc Dis. 2010; 103(1):46-52. DOI: 10.1016/j.acvd.2009.06.009. View

18.

Rubio-Rivas M, Royo C, Simeon C, Corbella X, Fonollosa V . Mortality and survival in systemic sclerosis: systematic review and meta-analysis. Semin Arthritis Rheum. 2014; 44(2):208-19. DOI: 10.1016/j.semarthrit.2014.05.010. View

19.

Nietert P, Silverstein M, Silver R . Hospital admissions, length of stay, charges, and in-hospital death among patients with systemic sclerosis. J Rheumatol. 2001; 28(9):2031-7. View

20.

Hoeper M, Bogaard H, Condliffe R, Frantz R, Khanna D, Kurzyna M . Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol. 2013; 62(25 Suppl):D42-50. DOI: 10.1016/j.jacc.2013.10.032. View