From the Guidelines
CREST syndrome management should prioritize treating individual symptoms, with a focus on Raynaud's phenomenon, esophageal dysfunction, and other organ involvements, as outlined in the 2023 EULAR recommendations for systemic sclerosis treatment 1.
Key Features of CREST Syndrome
- Calcinosis (calcium deposits in the skin)
- Raynaud's phenomenon (blood vessel constriction in fingers and toes)
- Esophageal dysfunction
- Sclerodactyly (skin thickening on fingers)
- Telangiectasia (dilated blood vessels on the skin)
Management of CREST Syndrome
- For Raynaud's phenomenon, dihydropyridine-type calcium antagonists, such as nifedipine, should be used as first-line therapy, with PDE5 inhibitors also considered for treatment 1.
- Esophageal symptoms can be managed with proton pump inhibitors (PPI), such as omeprazole or pantoprazole, for the treatment of SSc-GERD and prevention of oesophageal ulcers and strictures 1.
- Calcinosis may require surgical removal if painful or infected.
- Regular monitoring is essential as patients have an increased risk of pulmonary hypertension, which may require specific treatments like sildenafil or bosentan 1.
- Methotrexate, mycophenolate mofetil, and/or rituximab should be considered for treatment of SSc skin fibrosis, with tocilizumab also considered for the treatment of skin fibrosis in patients with early, inflammatory dcSSc 1.
Organ Involvement and Treatment
- Digital ulcers: PDE5 inhibitors and/or intravenous iloprost should be considered for treatment, with bosentan also considered for reduction of new digital ulcers 1.
- SSc-PAH: combination of PDE5i and endothelin receptor antagonists should be considered as first-line treatment, with intravenous epoprostenol considered for advanced PAH 1.
- Renal crisis: ACE inhibitors should be used immediately at diagnosis, with regular monitoring of blood pressure to detect scleroderma renal crisis 1.
- Gastrointestinal involvement: PPI should be considered for treatment of SSc-GERD, with prokinetic drugs considered for symptomatic motility disturbances related to SSc 1.
From the Research
Definition and Characteristics of CREST Syndrome
- CREST syndrome is a variant of systemic sclerosis (scleroderma) that affects a relatively small number of patients 2.
- It is also known as limited cutaneous scleroderma and has a more favorable prognosis than the diffuse form of scleroderma 2.
- CREST syndrome is characterized by the presence of at least three of the five clinical features: calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia 3.
Clinical Manifestations and Management
- The clinical manifestations of CREST syndrome include fibrotic skin changes restricted to the hands and face, with vascular, musculoskeletal, and visceral involvement 4.
- Patients with CREST syndrome often experience daily issues that are difficult to manage and require diligent support from the healthcare team 2.
- Management of CREST syndrome may involve a problem-oriented approach for nursing management 2 and various treatments such as botulinum toxin for Raynaud phenomenon 4.
Comparison with Systemic Sclerosis
- CREST syndrome is closely related to systemic sclerosis and seems to be part of the spectrum of systemic sclerosis 5.
- Patients with CREST syndrome tend to have a lower frequency of arthralgia and arthritis compared to those with systemic sclerosis 5.
- Calcinosis appears to be a key element in the diagnosis of CREST syndrome, as it is less common in systemic sclerosis and its association with other clinical features is characteristic of CREST syndrome 3.
Dental Management and Other Considerations
- CREST syndrome can have dentofacial complications, and prosthodontists should be aware of the clinical manifestations and management of the disease 6.
- The syndrome can overlap with both limited and diffuse subsets of systemic sclerosis, and future studies may contribute to a reassessment of systemic sclerosis classification 3.