Systemic Sclerosis (SSc) Diagnosis & Management: 2013 ACR/EULAR Score, FIBROSIS Checklist, and Treat-the-Phenotype Approach

Mayta
Jun 14
4 min read

1. Confirming the Diagnosis

Step	How you do it	Exam pearls
1 A. Clinical suspicion	Look for skin thickening, Raynaud phenomenon, puffy fingers, digital ulcers, telangiectasia, dysphagia, dyspnoea or abrupt hypertension.	Skin that is distal-to-proximal and non-pitting is the giveaway.
1 B. Apply the 2013 ACR/EULAR Classification Criteria – need ≥ 9 points	Single sufficient item: skin thickening to MCPs = 9 ⇒ definite SSc. Other weighted items (take highest per domain): • Puffy fingers (2) → Thickening to PIP (4) • Digital tip ulcer (2) / pitting scar (3) • Telangiectasia (2) • Abnormal nail-fold caps (2) • PAH or ILD (2) • Raynaud (3) • Anti-centromere / anti-Scl-70 / anti-RNA-pol III (3) sclerodermainfo.org	Mnemonic “S C A R P I T” (Skin, Capillaries, Antibody, Raynaud, Pulmonary/ILD, Ischaemic tips, Telangiectasia).
1 C. Sub-type	Limited cutaneous (lcSSc) = skin distal to elbows/knees ± face; Diffuse cutaneous (dcSSc) = proximal limbs or trunk within 3 y of Raynaud onset. scleroderma.org	dcSSc carries higher early visceral risk.
1 D. Exclude mimics	Nephrogenic systemic fibrosis, eosinophilic fasciitis, morphea, POEMS, graft-vs-host, hypothyroidism.	Lack of Raynaud strongly argues against SSc.

2. Staging & Activity Assessment

Domain	Tool (how often)	Cut-offs you quote in viva
Skin	Modified Rodnan Skin Score (mRSS) every visit	≤14 mild, 15-30 moderate, >30 severe rheumcalc.com
Lung	HRCT (baseline) + PFTs (6-12 monthly)	FVC drop >10 % or DLCO <70 % = progressive ILD
Pulmonary vascular	Echo annually → right-heart cath if TR Vmax > 2.8 m/s	mPAP ≥20 mmHg = PAH per 2022 ESC/ERS publications.ersnet.org
Renal	BP & creatinine at every visit; urinalysis	Sudden ↑BP or creatinine ⇒ urgent ACE-i for possible SRC
Composite severity	Medsger Severity Scale (0–4 in nine organs)	≥2 in any organ → high-risk follow-up

3. Core Investigations (“FIBROSIS” checklist)

F – Full blood count / film I – Immune profile: ANA, anti-centromere, anti-Scl-70, anti-RNA-pol III B – Baseline metabolic: U&E (Urea and electrolytes), LFT, glucose, lipids R – Rheum imaging: hand X-ray (acro-osteolysis), HRCT chest O – Organ screening: Echo, PFTs, capillaroscopy, oesophageal manometry if dysphagia S – Skin score (mRSS) & photography I – Infection screen before immunosuppression (HBV/HCV, TB) S – Scan kidneys/renal Doppler if hypertensive

4. Management — “Treat the phenotype, monitor the organ”

(2023 EULAR update + 2024 BSR guideline) nature.com

Manifestation	1st-line	Escalate / adjunct	Key monitoring & exam-favourite complications
Raynaud / digital ulcers	Nifedipine 30–60 mg SR od	PDE-5i (sildenafil), topical nitrates; IV iloprost for refractory ulcers; bosentan to prevent new ulcers	Watch for hypotension, ankle oedema (CCB); LFTs (bosentan).
Skin fibrosis (early, progressive)	Methotrexate 15–25 mg weekly (lcSSc) or Mycophenolate 1 g bid (dcSSc/ILD overlap)	Rituximab*, abatacept, or tocilizumab if inflammatory; HSCT in selected aggressive dcSSc	MTX – hepatotoxicity/fibrosis; MMF – cytopenia, infections.
Interstitial Lung Disease	Mycophenolate (preferred) or IV cyclophosphamide 6–12 months → azathioprine/MMF maintenance	Nintedanib 150 mg bid antifibrotic add-on if FVC falling	GI upset & ↑LFTs with nintedanib; haematuria with cyclo.
Pulmonary Arterial Hypertension	Endothelin-RA (bosentan/macitenan) ± PDE-5i ± prostacyclin analogues; follow 2022 ESC/ERS algorithm	Triple therapy or lung transplant referral if WHO FC III/IV	Monthly LFTs on ERA; echo every 6 m.
Scleroderma Renal Crisis (SRC)	Immediate Captopril 25 mg q8h titrate (any rise in BP ≥140/90 mmHg or creatinine)	Add other rapid anti-hypertensives; dialysis if needed	ACE-i dramatically ↓mortality (<10 %) amjmed.com. High-dose steroids (>15 mg pred) increase SRC risk – avoid.
Gastro-oesophageal reflux	PPI (omeprazole 20–40 mg od)	Add domperidone or metoclopramide; endoscopic dilatation strictures	Screen for Barrett oesophagus.
Calcinosis cutis	Conservative → low-dose diltiazem, minocycline; surgical removal	Still an unmet need; mention in OSCE as “research/clinical trial area”.

*Rituximab showed mRSS improvement in the DESIRES trial 2021 nature.com

5. Lifestyle, Preventive & Follow-up Essentials

Cold-avoidance gloves, smoking cessation.
Annual influenza, pneumococcal, zoster & COVID boosters.
DEXA + calcium/vit D if on steroids or immobile.
Pregnancy counselling: pause mycophenolate, cyclophosphamide, ERA; continue nifedipine for Raynaud.

6. Putting it into Practice (OSCE script)

“Mrs B, your skin score today is 18 (moderate) and your lung tests are stable at FVC 85 %. That tells us you’re in the early-diffuse phase but without lung fibrosis yet.”
Order HRCT + echo + labs per FIBROSIS.
Start mycophenolate 500 mg bid, uptitrate to 1 g bid over 4 weeks, plus nifedipine LA 30 mg for Raynaud.
Explain red-flag symptoms (headache, blurred vision → come in for BP check).
Follow treat-to-phenotype algorithm: repeat PFT/echo at 6 months; escalate to nintedanib if FVC drops >10 %.

7. Check-Yourself Question

Why is captopril favoured over other ACE-i in scleroderma renal crisis? Answer: Rapid titratability (short half-life) lets you adjust doses hourly to regain BP control, critical during SRC.

Remember: SSc is heterogeneous. The art is matching drug to organ trajectory early while vigilantly screening for silent complications. Master the 2013 score, know the big-ticket organ therapies, and you’re viva-proof!

General doctor vs Rheumatologist — who does what?

Task	GP / Internist	Rheumatology referral?
Initial recognition & baseline work-up	✓ – order ANA & antibodies, basic labs, chest X-ray, ECG, start cold-avoidance & PPI if reflux.	Refer all confirmed or strongly suspected SSc—early disease benefits from organ-screening & DMARD timing.
Management of *uncomplicated* Raynaud, reflux, skin care	✓ – prescribe CCB, PPI, give vaccine boosters, counsel lifestyle.	If digital ulcers, critical ischaemia, or refractory Raynaud → Rheum ± vascular.
Starting immunosuppressants (MTX, MMF) or biologics	✗ – requires Rheumatology supervision for dosing, monitoring & insurance approval.
Monitoring PFTs / echo yearly	GP can arrange tests, but results need Rheum interpretation for drug escalation.
Scleroderma renal crisis or suspected ILD/PAH	Initiate captopril and urgent BP control, admit; simultaneously call Rheum & Nephrology/Respiratory.
Pregnancy counselling	Coordinate obstetric care, but disease-modifying plan from Rheum/fetal-medicine.

Bottom line:

Any patient beyond simple Raynaud or reflux should be co-managed with, or promptly referred to, a rheumatologist. Early specialist input improves survival, especially for diffuse cutaneous disease, ILD, PAH and renal crisis. The general doctor’s continuing roles are prevention (vaccines, smoking cessation), comorbidity care, and rapid detection of organ complications.