Adults
Suspected systemic sclerosis (scleroderma)
Raynaud's with puffy fingers, skin thickening, digital ulcers or scleroderma antibodies.
What to look for
- Raynaud's with puffy or swollen fingers, sclerodactyly, digital pitting scars or ulcers
- Skin thickening proximal to the MCPs, telangiectasias, calcinosis
- Reflux, dysphagia, dyspnea, or a reduced DLCO
- ANA with an SSc-specific antibody: anti-centromere, anti-Scl-70 (topoisomerase I) or anti-RNA polymerase III
- Early disease can be just Raynaud's, puffy fingers and a positive ANA
What to do
- Send ANA with pattern, anti-centromere, anti-Scl-70, anti-RNA polymerase III, CBC, metabolic panel and urinalysis, and check blood pressure.
- Baseline PFTs with DLCO and HRCT are recommended for ILD screening (ACR/CHEST 2023). An echocardiogram helps screen for pulmonary hypertension.
- Avoid high-dose glucocorticoids, which raise the risk of renal crisis.
- Rapidly spreading skin thickening is urgent. New hypertension with AKI is emergent.
About this urgency level
Early specialist assessment changes outcomes. Send the usual workup with the referral, but don't hold the referral while waiting for results.
Timeframes are typical targets. Local pathways, capacity and clinical judgment come first.