What is the treatment for digital gangrene in scleroderma?

Treatment of Digital Gangrene in Scleroderma

Digital gangrene in scleroderma requires immediate surgical consultation for amputation consideration while simultaneously initiating aggressive intravenous prostacyclin therapy (iloprost). 1

Immediate Management Priorities

Surgical Assessment

• Gangrene presence is an absolute indication for surgical evaluation and likely amputation 1
• Do not delay surgical consultation—this represents a medical emergency requiring prompt evaluation 1
• Assess for underlying osteomyelitis, which occurs in 11% of scleroderma digital ulcer cases and necessitates surgical intervention 1
• Surgery is reserved for threatened digits with gangrene, underlying calcinosis, or failure of medical therapy 2

Concurrent Intravenous Therapy

• Initiate intravenous iloprost (prostacyclin analogue) immediately for severe digital ischemia with gangrene 1
• Iloprost is administered as continuous infusion over 6 hours daily for 5 consecutive days, with dosing adjusted to individual tolerability within 0.5 to 2.0 ng/kg/min 3
• This therapy has demonstrated efficacy in healing digital ulcers and reducing new ulcer formation 1
• Common adverse effects include headache, flushing, palpitations/tachycardia, nausea, vomiting, dizziness, and hypotension 3

Systemic Pharmacologic Management

First-Line Oral Vasodilators

• Add PDE5 inhibitors (sildenafil or tadalafil) to the regimen, as they show beneficial effects in improving and reducing digital ulcers 1
• These agents improve healing of existing digital ulcers (strength of recommendation: A) 4
• Continue or initiate dihydropyridine calcium channel blockers (nifedipine) as first-line therapy for underlying Raynaud's phenomenon 4

Prevention of Further Ulceration

• Consider bosentan (endothelin receptor antagonist) for prevention of new digital ulcers, particularly in patients with multiple (≥4) digital ulcers 1, 4
• Bosentan reduces new digital ulcer formation by 48% but does not improve healing of existing ulcers 4
• This should be added after failure of calcium channel blockers and prostanoid therapy 4

Wound Care and Infection Management

Local Wound Management

• Specialized wound care by trained nurses and physicians is required 1
• Apply silver sulfadiazine topical ointment to ulcerated areas 5
• Antibiotics should only be added when infection is clinically suspected—avoid prophylactic antibiotics 1

Critical Pitfall to Avoid

• Do not use prophylactic antibiotics routinely; reserve antibiotics for clinically suspected infection only 1, 6

Procedural Interventions

Digital Sympathectomy

• Consider palmar digital sympathectomy if medical management fails 1, 5
• This procedure has evidence supporting both healing and prevention of digital ulcers 1
• In one series, chronic digital ulcerations healed within 6 weeks after surgery, with 6 of 9 hands remaining ulcer-free at 26-64 month follow-up 5
• Reserve this intervention for patients who fail 12 weeks of aggressive medical management 5

Treatment Algorithm Summary

1. Immediate: Surgical consultation for amputation evaluation + IV iloprost 1
2. Concurrent: PDE5 inhibitors + calcium channel blockers 1, 4
3. Add if multiple ulcers: Bosentan for prevention 1, 4
4. Local care: Specialized wound management + antibiotics only if infection suspected 1
5. If medical failure: Digital sympathectomy 1, 5

Additional Supportive Measures

• Cessation of all vasoconstrictive agents (beta-blockers, ergot alkaloids, bleomycin, clonidine) 7, 5
• Smoking cessation and cold avoidance 7
• Monitor blood pressure carefully when combining vasodilatory therapies due to additive hypotensive effects 6