Skin tightening, Raynaud, telangiectasia
Confirm: anti-Scl-70 (diffuse) / anticentromere (limited/CREST)
Organ-directed: Raynaud → nifedipine (PDE5 inhibitor for refractory/digital ulcers); reflux → PPI; ILD → MMF/CYC ± nintedanib or tocilizumab/rituximab; digital ulcers → sildenafil ± bosentan (EULAR SSc 2023)
Order set
- ANA, anti-Scl-70, anticentromere
- BP + renal monitoring
- Echo + PFTs (PAH/ILD)
- Nailfold capillaroscopy
Criteria
AdmitRenal crisis, PAH, or severe ILD
ICURenal crisis with malignant HTN, respiratory failure
DialysisRenal crisis with AEIOU
DischargeOrgan crises stabilised, ACEi (renal crisis), surveillance
Never
- Use anything but an ACE inhibitor for scleroderma renal crisis
- Use high-dose glucocorticoids (≥15 mg/day prednisolone) — they precipitate scleroderma renal crisis
Clinical detail — differentials, red flags, pitfalls, disposition
Red flags
- Scleroderma renal crisis (↑BP, AKI)
- New dyspnoea (PAH/ILD)
Differentials
- Raynaud (primary)
- Mixed CTD
- Eosinophilic fasciitis
Common mistakes
- Anything but ACEi for renal crisis
- Missing PAH/ILD screening
Disposition & follow-up
Organ-based therapy; annual PAH/ILD surveillance.
Discharge package
MedicationsOrgan-directed (ACEi for renal crisis, PPI, vasodilators)
Follow-upRheumatology; annual PAH/ILD screen
Warning symptomsRising BP, breathlessness, digital ulcers
💊 Treatment detail — doses & preparation
Nifedipinedihydropyridine CCB
DoseMR 10–30 mg OD (Raynaud, HTN); short-acting capsules avoided (precipitous drops)
PreparationMR tablets 10/20/30/60 mg — swallow whole
MonitorAnkle oedema, headache; MR only in hypertension
OmeprazolePPI
Dose20–40 mg OD 30 min before breakfast ×4–8 wk
Preparation20/40 mg capsules (MUPS dispersible for NG)
MonitorLong-term: Mg²⁺, B12, C. diff risk
LisinoprilACE inhibitor
DoseStart 2.5–5 mg OD, target 20–40 mg OD
Preparation2.5/5/10/20 mg tablets
MonitorCreatinine + K⁺ 1–2 wk after each titration
Mycophenolate mofetilimmunosuppressant
Dose500 mg BD titrate to 1–1.5 g BD (SSc-ILD; also lupus nephritis)
Preparation250/500 mg tablets/capsules
MonitorFBC weekly ×4 then monthly; teratogenic — contraception essential
📖 EULAR Systemic Sclerosis (2023 update)Reviewed July 2026