From the Guidelines
CREST syndrome is a limited form of systemic sclerosis that requires individualized symptom management to improve quality of life and prevent complications. The management of CREST syndrome focuses on treating its individual symptoms, including Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, telangiectasia, and calcinosis.
- For Raynaud's phenomenon, patients should avoid cold exposure, stop smoking, and may benefit from dihydropyridine-type calcium antagonists, usually oral nifedipine, as first-line therapy, as recommended by the EULAR guidelines 1.
- Esophageal symptoms can be managed with proton pump inhibitors (PPI) such as omeprazole or pantoprazole, which are recommended for the treatment of SSc-related gastroesophageal reflux disease (GERD) and prevention of oesophageal ulcers and strictures 1.
- Calcinosis may require surgical removal if painful or infected, although there is limited evidence to guide its management.
- Regular monitoring is essential as some patients may progress to diffuse systemic sclerosis, and early symptom management can significantly improve quality of life and prevent complications like digital ulcers or pulmonary hypertension. The condition results from autoimmune dysfunction causing excessive collagen production and vascular abnormalities, and while there's no cure, early symptom management with therapies such as calcium channel blockers, PPI, and potentially disease-modifying agents like mycophenolate mofetil or rituximab, can significantly improve outcomes 1.
From the Research
Definition and Characteristics of CREST Syndrome
- CREST syndrome is a form of progressive systemic sclerosis with limited skin involvement, characterized by calcinosis, Raynaud's phenomenon, esophageal dysfunction, and telangiectasia 2.
- The acronym CREST was coined in 1964, but the first case report was by French physicians Thibierge and Weissenbach in 1910 2.
- Antinuclear antibodies recognizing chromosomal centromere proteins are present in more than 50% of CREST syndrome cases 2.
Clinical Features and Diagnosis
- CREST syndrome is associated with systemic sclerosis and meets at least three of the five clinical features: calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia 3.
- Calcinosis is considered 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.
- The American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) 2013 criteria can be used to diagnose systemic sclerosis, which includes CREST syndrome 3.
Treatment and Management
- Botulinum toxin can be used to treat Raynaud phenomenon in CREST syndrome, as it has been shown to improve symptoms and promote healing of digital ulcers 4.
- Nursing care for CREST syndrome patients should follow a problem-oriented approach, addressing daily issues and providing diligent support from the healthcare team 5.
Prognosis and Complications
- The prognosis of CREST syndrome is relatively good, with a long disease duration (>10 years) 2.
- However, two complications are seldom associated with CREST syndrome: digital gangrene with finger losses and pulmonary hypertension (3 to 14% of CREST syndrome) 2.
- Pulmonary hypertension is a late event and has a severe prognosis, with a mortality rate of 50% after 2 years 2.