A 54 yo F with HTN and GERD presents with puffy digits, hand tightening, and new shortness of breath.
Questions to Ask
- Do puffy digits look like skin tightening (loss of wrinkles) or look like arthritis (localized swelling at joints)?
- Is the skin tightening proximal to the wrist? Is there truncal involvement (look for tightening at upper chest – can have “salt and pepper” appearance of skin)?
- Is there Raynaud’s?
- Any new cough? Fevers? Lower extremity swelling? Choking episodes?
Physical Exam
- Patient has puffy digits with loss of wrinkles and no swelling localized over the joints
- Also some tightening at the upper chest and difficulty opening mouth all the way
- ++Raynaud’s
- Crackles on pulmonary exam
- No fevers
- Trace new LE edema, symmetrical bilaterally
Diagnostic Workup
Initial Labs, Imaging, & Procedures
- Recommended labs:
- Recommended imaging/procedures:
- CXR → High-resolution CT chest
- PFTs
- TTE
- Swallow study
Further Workup
- If ANA positive, recommend:
- SCL-70
- Centromere antibody
- ENA panel (anti-Smith, dsDNA, etc.)
- RNA polymerase III antibody
- If ANA negative, referral to dermatology for biopsy of skin to further elucidate if different diagnosis
- CXR → regardless of remarkable or not, recommend CT chest interstitial lung disease (ILD) protocol (high res, no contrast)
- Ideally imaging in prone
- PFTs may clue in re: pHTN, ILD
- TTE recommended
Consults/Referrals
- Rheumatology
- Dermatology
- GI
- Pulmonology +/- cardiology
Treatment
- Caution AGAINST start of systemic steroids – they do not help with the skin tightening
- Recommend CCB for Raynaud’s therapy if no contraindications
- MMF can be considered after confirming systemic sclerosis (SSc) diagnosis
- Start ACEi immediately if labs and urinalysis show concern for scleroderma renal
crisis