Monitoring Scleroderma After Diagnosis
Here’s a breakdown of how care teams may monitor scleroderma progression:
How this presents
Swollen, painful fingers with a triphasic color change — white, then blue or purple, then red — triggered by cold or stress.
Baseline evaluation
Nailfold capillaroscopy (a non-invasive scope exam of the small blood vessels at the base of your fingernails) to confirm secondary RP — the form linked to scleroderma, as opposed to primary Raynaud’s.
Ongoing monitoring
Tracked through your medical history at follow-up visits.
Indicators of progression
Tissue damage from restricted blood flow, or ischemic ulcers (open sores caused by inadequate blood supply to the skin) that don’t heal even after rewarming.
How this presents
Puffy fingers, or tightened, thickened skin over the digits, which can later become contracted and hard to bend
Baseline evaluation
Measurement of skin thickness using a modified Rodnan Skin Score (mRSS), which is a hands-on exam where a clinician scores skin thickness at multiple body sites.
Ongoing monitoring
Repeat mRSS scoring alongside a hand function assessment.
Indicators of progression
Increasing skin thickness or a rising mRSS score.
How this presents
Fatigue, exercise intolerance, shortness of breath.
Baseline evaluation
High-resolution CT (HRCT) and pulmonary function tests (PFTs).
Ongoing monitoring
Repeat PFTs, followed by HRCT if lung function is declining or symptoms appear.
Indicators of progression
More fibrotic changes on HRCT, declining PFT results, or new or worsening respiratory symptoms
How this presents
Fatigue, exercise intolerance, shortness of breath, dizziness, swelling (edema), chest pain.
Baseline evaluation
Echocardiogram (an ultrasound of the heart) and PFTs.
Ongoing monitoring
Clinical assessment of dyspnea, NT-proBNP blood testing (a blood test that measures a hormone released when the heart is under strain), and repeat echocardiograms.
Indicators of progression
Worsening symptoms, hemodynamic changes (shifts in how blood is flowing through the heart and lungs, often picked up on echocardiogram), or an increased burden of telangiectasia (visible small blood vessels).
How this presents
Headache, blurred vision, shortness of breath, fatigue.
Baseline evaluation
Blood pressure check, plus urine or serum creatinine testing (a blood and urine test that measures how well the kidneys are filtering waste) if symptoms are present.
Ongoing monitoring
Blood pressure checks at home, plus urine or serum creatinine testing if symptoms appear.
Indicators of progression
Acute kidney injury or renal failure, hypertensive retinopathy (damage to blood vessels in the eye from severe high blood pressure), or hypertensive encephalopathy (brain swelling and dysfunction caused by a severe, rapid rise in blood pressure). This is a medical emergency. If you notice a sudden blood pressure spike alongside these symptoms, seek care immediately.
How this presents
Acid reflux (GERD), difficulty swallowing, unexplained weight loss, abdominal symptoms.
Baseline evaluation
If symptoms are present, GI tract imaging (upper endoscopy, a scope placed to look at the lining of the esophagus and stomach).
Ongoing monitoring
Repeat GI tract imaging if symptoms appear or change.
Indicators of progression
Worsening reflux, symptoms or lab tests indicating small intestinal bacterial overgrowth (SIBO) requiring antibiotic treatment, malabsorption (the body’s inability to properly absorb nutrients from food), or intestinal pseudo-obstruction (symptoms of a bowel blockage without an actual physical obstruction).
How this presents
Pain, stiffness, weakness, symptoms of carpal tunnel syndrome.
Baseline evaluation
Physical exam, creatine phosphokinase (CPK) bloodwork (a test that measures an enzyme released when muscle is damaged), and X-rays as needed.
Ongoing monitoring
Repeat physical exam, CPK, and X-rays as needed.
Indicators of progression
Joint contractures or tendon friction rubs.