From the Guidelines
CREST syndrome is a limited form of systemic sclerosis, an autoimmune disorder that affects the skin and internal organs, characterized by Calcinosis, Raynaud's phenomenon, Esophageal dysfunction, Sclerodactyly, and Telangiectasia. The acronym CREST stands for the five main features of the condition. Treatment typically focuses on managing individual symptoms rather than curing the disease. For Raynaud's, calcium channel blockers like nifedipine (10-30 mg daily) or amlodipine (5-10 mg daily) may help improve circulation, as recommended by the EULAR guidelines 1. Proton pump inhibitors such as omeprazole (20-40 mg daily) or pantoprazole (40 mg daily) can address esophageal issues and prevent acid reflux, also in line with EULAR recommendations 1. Immunosuppressants like methotrexate (7.5-25 mg weekly) might be prescribed for more severe manifestations, as suggested by studies 1. The condition occurs because the immune system mistakenly attacks healthy tissues, causing inflammation and excessive collagen production that leads to hardening and scarring. While CREST syndrome progresses more slowly and is generally less severe than diffuse systemic sclerosis, regular medical follow-up is essential to monitor for potential complications involving the lungs, heart, or kidneys, as highlighted in the EULAR guidelines 1.
Some key points to consider in the management of CREST syndrome include:
- The use of dihydropyridine-type calcium antagonists, such as nifedipine, as first-line therapy for Raynaud's phenomenon 1
- The consideration of PDE-5 inhibitors for the treatment of digital ulcers and Raynaud's phenomenon 1
- The use of proton pump inhibitors for the treatment of gastroesophageal reflux disease (GERD) and prevention of esophageal ulcers and strictures 1
- The potential use of immunosuppressants, such as methotrexate, for the treatment of skin fibrosis and other manifestations of CREST syndrome 1
- The importance of regular monitoring for potential complications, including pulmonary arterial hypertension, interstitial lung disease, and scleroderma renal crisis 1.
Overall, the management of CREST syndrome requires a comprehensive approach that takes into account the individual patient's symptoms and needs, and is guided by the latest evidence-based recommendations, such as those provided by the EULAR guidelines 1.
From the Research
Definition of CREST Syndrome
- CREST syndrome is a clinical entity associated with systemic sclerosis, characterized by the presence of at least three of the five clinical features: calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia 2.
- It is a form of limited systemic sclerosis, manifesting as fibrotic skin changes restricted to the hands and face, with vascular, musculoskeletal, and visceral involvement 3.
Clinical Features
- The five clinical features of CREST syndrome are:
- 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 2.
Associations and Complications
- CREST syndrome can overlap with both limited and diffuse subsets of systemic sclerosis 2.
- It can be associated with other conditions, such as primary sclerosing cholangitis 4 and pulmonary hypertension 5.
- Patients with CREST syndrome may experience digital ulceration, persistent pain, and recalcitrant Raynaud's phenomenon, which can be treated with botulinum toxin 3.