ABSTRACT
Systemic sclerosis (SSc) is a rare multisystem autoimmune disease characterized by fibrosis, vasculopathy, and autoimmunity. There are multiple complications inherent to SSc and its management. One of these complications is increased infection risk, which can lead to decreased quality of life and increased morbidity and mortality. Patients with SSc have lower vaccination rates and decreased vaccine seroconversion secondary to immunosuppressive medications compared with the general population. The purpose of this review is to provide clinicians with an approach to vaccinations in SSc.
Subject(s)
Quality of Life , Scleroderma, Systemic , Humans , Scleroderma, Systemic/complications , Fibrosis , Autoimmunity , Vaccination/adverse effectsABSTRACT
Systemic sclerosis (SSc) is a rare multisystem autoimmune disease characterized by fibrosis, vasculopathy, and autoimmunity. Lesser known complications inherent to SSc, such as malignancies and osteoporosis, can lead to decreased quality of life and increased morbidity and mortality. Patients with SSc have a greater risk of developing malignancies than the general population. In addition, they are more likely to be vitamin D deficient and are at great risk of osteoporosis-related fractures. However, these complications can be addressed through preventative measures. The purpose of this review is to provide clinicians with an approach to bone health and cancer screening in SSc.
Subject(s)
Neoplasms , Osteoporosis , Osteoporotic Fractures , Scleroderma, Systemic , Humans , Bone Density , Early Detection of Cancer , Quality of Life , Scleroderma, Systemic/complications , Scleroderma, Systemic/diagnosis , Osteoporosis/diagnosis , Osteoporosis/etiology , Osteoporosis/prevention & control , Neoplasms/diagnosis , Neoplasms/epidemiology , Neoplasms/prevention & controlABSTRACT
PURPOSE OF REVIEW: Systemic sclerosis (scleroderma, SSc) is a rare multisystem autoimmune disease characterized by autoantibodies, vasculopathy, and fibrosis of the skin and internal organs. This review aims to provide an overview and summary of the recent epidemiological studies in systemic sclerosis. RECENT FINDINGS: Global trends of scleroderma demonstrate greater prevalence of SSc in European, North, and South American patients compared with East Asian patients. However, the greatest prevalence (47 in 100â000), was found among the indigenous peoples in Canada. Phenotypical differences exist depending on the age of presentation with greater internal organ involvement and disease acceleration present in older patients. Sex differences include greater severity of disease expression, relative prevalence of diffuse cutaneous SSc, and organ involvement in males versus females. New studies conflict with previous data reporting greater proportion of pulmonary arterial hypertension in females. Furthermore, the effect of low median household income is demonstrated as a factor increasing risk of death in SSc patients. SUMMARY: Understanding the epidemiological factors in SSc enables patient care through patient classification, prognostication, and monitoring. Future research may emphasize enrichment of SSc patients in randomized trials who are more likely to progress or be treatment responsive, focused screening, and personalized patient care through the creation and validation of new SSc criteria and subsets.
