What is the management approach for Crest (Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia) syndrome?

Management of CREST Syndrome

CREST syndrome requires a systematic, manifestation-specific treatment approach prioritizing early screening for life-threatening complications (ILD and PAH), aggressive management of Raynaud's phenomenon with calcium channel blockers or PDE-5 inhibitors, and surgical intervention for symptomatic calcinosis when medical therapy fails. 1

Immediate Screening and Risk Stratification

All patients must undergo comprehensive cardiopulmonary screening at diagnosis, as these complications determine mortality 1:

• Perform pulmonary function tests, high-resolution CT, and echocardiography to screen for interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) 1
• Institute regular blood pressure monitoring, particularly in patients with anti-RNA polymerase III antibodies, to detect scleroderma renal crisis early 1
• Recognize that while ILD occurs in 40-75% of systemic sclerosis patients, only 15-18% have progressive disease requiring treatment 1

Manifestation-Specific Treatment Algorithm

Raynaud's Phenomenon (First Manifestation to Address)

Start with dihydropyridine calcium channel blockers (nifedipine specifically) as initial therapy 1:

• PDE-5 inhibitors are equally appropriate as first-line therapy 1
• Escalate to intravenous iloprost for severe Raynaud's phenomenon when oral therapies fail 1

Digital Ulcers

• Use PDE-5 inhibitors and/or intravenous iloprost for active digital ulcer treatment 1
• Add bosentan specifically for prevention of new digital ulcer formation, not for healing existing ulcers 1

Esophageal Dysmotility

• Initiate proton pump inhibitors (PPIs) for gastroesophageal reflux disease and prevention of esophageal ulcers and strictures 1
• Add prokinetic drugs for symptomatic motility disturbances 1
• Aggressively address nutritional support, as malnutrition from gastrointestinal involvement is the leading cause of mortality 1

Sclerodactyly and Skin Fibrosis

Treatment is most effective within 2-5 years from onset of first non-Raynaud's features 1:

• Use methotrexate, mycophenolate mofetil (MMF), or rituximab for early disease with significant skin involvement 1
• Consider tocilizumab specifically for early, inflammatory diffuse cutaneous disease 1

Interstitial Lung Disease

Mycophenolate mofetil (MMF) is the first-line therapy for ILD 1:

• Cyclophosphamide or rituximab serve as alternative first-line options 1
• Add nintedanib alone or in combination with MMF for progressive fibrotic ILD 1

Pulmonary Arterial Hypertension

Initiate combination therapy with PDE-5 inhibitors and endothelin receptor antagonists as first-line treatment 1:

• Use intravenous epoprostenol for advanced PAH (WHO functional class III and IV) 1
• Consider other prostacyclin analogues or riociguat as alternatives 1
• Do NOT use anticoagulants (warfarin) for SSc-PAH—this differs critically from idiopathic PAH management 1

Calcinosis

No proven medical therapy exists for calcinosis—consider surgical intervention early for symptomatic cases rather than prolonged ineffective medical management 1:

• Simple excision is adequate for minor outpatient cases 2, 3
• Radical debridement with flap reconstruction is required for major, painful cases, particularly involving the thumb or functionally important areas 3
• The kite flap provides optimal tissue quality, dimensions, and sensory recovery for thumb reconstruction 3
• Complete resection with adequate reconstruction is possible even for large invasive tumors, providing acceptable postoperative results and quality of life improvement 2
• Spinal calcinosis may require minimally invasive surgical approaches with decompression and fusion 4

Scleroderma Renal Crisis

• Start ACE inhibitors immediately at diagnosis of scleroderma renal crisis 1
• Monitor blood pressure regularly in patients on glucocorticoids to detect renal crisis early 1

Critical Pitfalls to Avoid

• Never delay screening for ILD and PAH—these complications determine mortality and early intervention changes natural history 1
• Do not use anticoagulation routinely for SSc-PAH as you would for idiopathic PAH 1
• Do not assume typical disease progression—many patients with diffuse disease do not improve after 4 years and may worsen later 1
• Never overlook nutritional status—malnutrition from gastrointestinal involvement is a leading cause of mortality 1
• Avoid prolonged ineffective medical management of symptomatic calcinosis when surgical options can improve quality of life 1, 2