Epidemiology of Systemic Sclerosis

Abstract and Introduction

Abstract

Purpose of review To identify the recent data regarding prevalence, incidence, survival, and risk factors for systemic sclerosis (SSc) and to compare these data to previously published findings.
Recent findings SSc disease occurrence data are now available for Argentina, Taiwan, and India and continue to show wide variation across geographic regions. The survival rate is negatively impacted by older age of onset, male sex, scleroderma renal crisis, pulmonary fibrosis, pulmonary arterial hypertension, cancer, and antitopoisomerase and anti-U1 antibodies. It appears that silica exposure confers an increased risk for developing scleroderma, but this exposure accounts for a very small proportion of male patients. Smoking is not associated with increased SSc susceptibility. Malignancies are reported in scleroderma at an increased rate, but the magnitude of this risk and the type of cancer vary among reports.
Summary Prevalence and incidence of SSc appears to be greater in populations of European ancestry and lower in Asian groups. Exposure to silica dust appears to be an environmental trigger, but this only accounts for a small proportion of male cases. Evidence for increased risk of neoplasia is suggestive, but the magnitude of the risk and the types of malignancies vary among reports.

Introduction

Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by small vessel vasculopathy, autoantibody production, and excessive collagen deposition in the skin and internal organs.^[1] The classification of SSc is divided into two groups: limited and diffuse cutaneous disease.^[2] The limited form is characterized by thickening of the skin confined to the areas distal to the elbows and knees, and is associated with less severe internal organ involvement. The diffuse form involves skin thickening proximal to the elbows and knees in addition to distal area involvement and is associated with more severe organ damage. This review will focus on the epidemiology of SSc and discuss the recently published articles related to this topic. The incidence and prevalence, survival, risk factors, malignancy, and putative environmental triggers will be reviewed.

Table 1. Incidence and prevalence in past decades
Publication	Region	Study period	Incidence per million	Prevalence per million
USA
Steen et al. [4]	Pennsylvania	1963–1972, 1973–1982, 1963–1982	13.9 (entire period)
Maricq et al. [5]	South Carolina	1989	–	286
Mayes et al. [6]	Michigan	1989–1991	21	276
Arnett et al. [7]	Oklahoma	1996		658.6
Bernatsky et al. [8]	Quebec	2003	–	443
Australia
Englert et al. [9]	Sydney	1974–1988	12	45.2 (1975) 86.2 (1988)
Chandran et al. [10]	South Australia	1987–1993	–	208
Roberts-Thompson et al. [11]	South Australia	1993	15.1	200
		1999	22.8	233
Roberts-Thompson et al. [12]	South Australia	1993–2002	20.4	232.2
Japan
Tamaki et al. [13]	Tokyo	1987	7.2	38
UK and Europe
Silman et al. [14]	England (West Midlands)	1986	3.7	31
Allock et al. [15]	England (Newcastle)	2000	–	88
Geirsson et al. [16]	Iceland	1975–1990	3.8	71
Kaipiainen-Seppanen	Finland	1990	3.7
and Aho [17]
Le Guern et al. [18]	France (Seine-St-Denis	2001	–	158
Alamanos et al. [19]	Greece (Northwest)	1981–2002	11	154
Arias-Nunez et al. [20]	Spain (Northwest)	1988–2006	23	277

Table 2. Incidence and prevalence: updates in last year
Publication	Region	Study period	Incidence per million	Prevalence per million
Monaco et al. [21•]	Northeast Italy	1991–2007	43	341
Rosa et al. [22•]	Buenos Aires	1999–2004	21.2	296
Kuo et al. [23•]	Taiwan	2002–2007	10.9	56
Minz et al. [24•]	North India	2006–2007	–	120

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