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

Management Approach for CREST Syndrome

The management of CREST syndrome requires organ-specific treatment targeting each component of the syndrome (Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia) to reduce morbidity and improve quality of life. 1

Overview of CREST Syndrome

CREST syndrome is a limited cutaneous form of systemic sclerosis (SSc), characterized by:

• Calcinosis: Calcium deposits in soft tissues
• Raynaud's phenomenon: Vasospasm of digital arteries
• Esophageal dysmotility: Impaired esophageal function
• Sclerodactyly: Skin thickening of fingers
• Telangiectasia: Dilated blood vessels visible on skin

Management Algorithm by Manifestation

1. Raynaud's Phenomenon

• First-line: Dihydropyridine calcium channel blockers (especially nifedipine) 1
• Second-line: Phosphodiesterase 5 inhibitors (sildenafil) 1
• Severe cases: Intravenous iloprost 1
• For digital ulcers: Bosentan to reduce development of new digital ulcers 1
• Alternative therapy: Botulinum toxin injections (10 units into each webspace) for recalcitrant cases 2

2. Calcinosis

• Conservative management: Pain control with analgesics
• Surgical options:
  • Simple removal for minor outpatient cases
  • Radical debridement for major, painful calcifications
  • Cover flap reconstruction (kite flap recommended for thumb) 3
  • Complete resection for symptomatic lesions to improve quality of life 4

3. Esophageal Dysmotility

• Dietary modifications:
  • Thickened liquids based on severity of dysphagia
  • Nectar-thick liquids for mild aspiration risk
  • Honey-thick liquids for more severe aspiration risk
  • Spoon delivery rather than cup drinking to reduce aspiration risk
• Postural techniques:
  • Chin-down position during swallowing
  • Head rotation or tilt as needed
• Pharmacologic therapy:
  • Proton pump inhibitors for gastroesophageal reflux
  • Prokinetic agents for dysmotility

4. Sclerodactyly

• Immunosuppressive therapy for early diffuse cutaneous SSc:
  • Mycophenolate mofetil as first-line therapy 1
  • Cyclophosphamide as alternative
• Physical therapy to maintain hand function and prevent contractures
• Moisturizers to manage skin dryness

5. Telangiectasia

• Cosmetic interventions:
  • Laser therapy for visible telangiectasias
  • Camouflage makeup

6. Associated Conditions

• Interstitial Lung Disease (ILD):
  • Screening with pulmonary function tests and HRCT
  • Mycophenolate mofetil as first-line therapy 1
  • Nintedanib for progressive fibrotic ILD 1
• Pulmonary Arterial Hypertension (PAH):
  • Regular screening with echocardiography
  • Combination therapy with phosphodiesterase-5 inhibitors and endothelin receptor antagonists 1
  • Addition of prostacyclin analogues if necessary 1
• Sjögren's Syndrome (if present):
  • Saliva secretion stimulators
  • Salivary substitutes
  • Topical fluoride for dental protection 5

Monitoring and Follow-up

• Regular blood pressure monitoring, especially in early disease
• Pulmonary function tests every 6-12 months
• Echocardiography annually for PAH screening
• Endoscopy as needed for esophageal symptoms

Common Pitfalls and Caveats

• Delayed recognition: CREST syndrome can develop gradually over years, but early recognition and treatment are crucial to prevent complications 6
• Undertreatment of Raynaud's: Aggressive management is needed to prevent digital ulcers and tissue loss
• Overlooking pulmonary complications: Regular screening for ILD and PAH is essential as these are major causes of mortality
• Surgical risks with calcinosis: Despite size and muscle invasion, complete resection of calcinosis is possible with appropriate reconstruction techniques 4
• Dehydration risk: Patients on thickened liquids for esophageal dysmotility have higher risk of dehydration, especially with honey-thick liquids

By addressing each component of CREST syndrome with targeted therapies and regular monitoring for complications, morbidity can be reduced and quality of life improved for patients with this challenging condition.