Systemic sclerosis is a rare autoimmune disease characterized by vasculopathy and organ fibrosis
The condition is associated with genetic risks (18%) and environmental and workplace exposures, including respirable crystalline silica (e.g., from construction, mining) and organic solvents (e.g., degreasers, paint thinners, adhesives).1 Incidence in Canada is roughly 4 per 100 000 person-years and rising annually. 1 Females older than 50 years are most affected.1
Systemic sclerosis is the leading cause of secondary Raynaud phenomenon in adults2
Secondary Raynaud phenomenon should be considered if onset occurs after age 30 years, and asymmetry, digital ulcers or pits, puffy hands, or abnormal nailfold capillaries are present. Workup includes antinuclear antibody, complete blood count, C-reactive protein (CRP), and serum protein electrophoresis (to exclude paraproteinemia).2 Abnormal results should prompt rheumatology or dermatology referral.3
The disease affects multiple organs
Near universally involved systems include skin (telangiectasia, digital pits or ulcers, Raynaud phenomenon, sclerodactyly, calcinosis) and the gastrointestinal tract (reflux, dysphagia, and gastroparesis [90% of patients]).4–6 Pulmonary disease (interstitial lung disease [25% to 50%] and pulmonary arterial hypertension [5% to 10%]) are the most lethal manifestations. 4,5 Renal crisis is less common (1% to 14%) and has been associated with corticosteroid use.3,6
Blood pressure, creatinine, and urinalysis should be monitored annually after diagnosis
Baseline spirometry and echocardiography should be obtained at diagnosis. High-resolution computed tomography of the chest can be considered for high-risk patients (diffuse subtype, male, age > 50 yr, elevated CRP) or those with respiratory symptoms.4,5 Patients who develop worsening dyspnea, dry cough, rapid skin thickening, digital ulcers, new hypertension, or renal failure should be referred urgently.4,5
Supportive care includes smoking cessation, cardiovascular risk reduction, and nonpharmacologic measures to treat Raynaud phenomenon, such as heated mittens
First-line pharmacologic options such as nifedipine extended-release for Raynaud phenomenon and proton pump inhibitors for reflux may be initiated.3,4 Key early treatments, including dosing and practical tips, are summarized in Appendix 1, available at www.cmaj.ca/lookup/doi/10.1503/cmaj.251602/tab-related-content.
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Footnotes
Competing interests: None declared.
This article has been peer reviewed.
References
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