CREST Syndrome

What is CREST Syndrome?

CREST syndrome is the former name for limited cutaneous systemic sclerosis, a rare connective tissue disease in the scleroderma family with an unknown cause.^[1] The letters stand for calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia.^[1] This is a serious autoimmune condition that needs specialist medical care.

Common symptoms

The first sign is often Raynaud phenomenon, in which the fingers and toes turn white or blue in cold or under stress and then red as blood flow returns.^{[1, 2]} Over time the skin of the fingers thickens and tightens (sclerodactyly), and small red vessel marks (telangiectasia) and calcium deposits under the skin (calcinosis) may appear.^[1]

Esophageal dysmotility means the esophagus moves food poorly, causing heartburn and trouble swallowing.^[1] In limited disease the skin thickening stays below the elbows and knees and on the face, but the disease can still involve the lungs and other organs.^[1]

Causes and risk factors

The cause is not known. It is an autoimmune disease in which the immune system, blood vessel injury and scarring (fibrosis) all play a part.^[3] It is more common in women and usually begins in adulthood.^[3] Anticentromere antibodies are the typical antibody in this limited form.^[1]

How it is diagnosed and treated conventionally

Diagnosis rests on the pattern of symptoms and examination, supported by antibody blood tests, and a rheumatologist guides this.^{[1, 3]} Early and accurate diagnosis matters, as does regular checking for organ involvement.^[3]

Treatment is matched to each organ affected. European treatment recommendations cover Raynaud phenomenon, digital ulcers, pulmonary arterial hypertension, kidney crisis, skin fibrosis, lung disease, gut involvement and arthritis, and they include immune-suppressing and other targeted medicines for the more serious manifestations.^[2] In a systematic review of secondary Raynaud phenomenon (the type seen with diseases like systemic sclerosis), calcium channel blockers significantly reduced the frequency and severity of attacks.^[4]

Why CREST Syndrome calls for a whole-person approach

CREST syndrome affects blood vessels, skin, gut and sometimes lungs, so no single measure covers it. Reviews describe a combined process of inflammation, blood vessel disease and fibrosis, and say that treatment must be tailored to the person and the organs involved.^[3]

Supportive therapies may help people cope with symptoms and daily life. They do not act on the disease process, and they do not replace screening or medical treatment.

Blood vessels and cold sensitivity

Vascular disease is a core feature, and Raynaud phenomenon affected 97 of 100 patients with systemic sclerosis in one survey.^[5] Drug treatment is directed by your rheumatologist, and calcium channel blockers have the clearest evidence for reducing the frequency and severity of attacks in the secondary form.^[4] Keeping warm and avoiding triggers are the usual self-care measures.

The gut

The esophagus and bowel are commonly involved, producing reflux, swallowing difficulty and bowel symptoms.^[1] These need medical evaluation, because they can lead to complications.

Skin and joints

Skin tightening limits movement in the fingers and hands, and joint involvement is part of the disease picture.^[2] Physical and occupational therapy are usual parts of care.

Stress, pain and quality of life

In a survey of patients with lupus, Sjogren syndrome or systemic sclerosis, more than half used complementary approaches, most often to relieve pain, for well-being and to reduce fatigue, and those with poorer quality of life and higher anxiety scores used them more.^[6]

Acupuncture as supportive care in CREST Syndrome

What the research shows

No clinical trials of acupuncture for CREST syndrome or limited systemic sclerosis were found. A review of complementary therapies in systemic sclerosis noted that acupuncture may be beneficial in care, but that no large randomized trials have been done.^[7]

The nearest evidence concerns Raynaud's phenomenon. A meta-analysis of 6 randomized trials with 272 participants found that acupuncture was associated with more remission and slightly fewer daily attacks, but the authors warned that the studies were small, at high risk of bias and of very low quality.^[8] These trials were in Raynaud syndrome generally, not specifically in scleroderma. An earlier systematic review of secondary Raynaud's phenomenon found only one acupuncture trial and judged the evidence for interventions other than calcium channel blockers and iloprost to be limited, conflicting or absent.^[4]

How acupuncture may work

Researchers propose that acupuncture may influence nerve reflexes and immune signaling.^[9] This is mostly laboratory research, and it has not been shown to alter the course of scleroderma or restore damaged blood vessels.

What treatment involves

Treatment uses fine, sterile, single-use needles, usually left in place for about 20 to 40 minutes, with a short course of visits and a reassessment. Because skin can be tight, fragile or ulcerated, especially on the fingers, we ask about skin and circulation first and avoid needling ulcers, infected or very tight skin.

Serious problems from acupuncture are rare. In prospective studies, about 9 in 100 patients had a minor reaction such as brief soreness, bleeding or bruising at a needle site, and serious events occurred in about 1 in 10,000 patients.^[10] Tell the practitioner first if you have a bleeding disorder, take blood thinners, are pregnant, or have a pacemaker (relevant to electroacupuncture).

Ayurvedic medicine as supportive care in CREST Syndrome

The Ayurvedic view

Ayurveda has no exact counterpart to scleroderma. Skin hardening is traditionally discussed under skin disorders such as Kushtha, and cold, pale fingers may be described as an imbalance of Vata, with impaired digestion (agni) contributing. This is a traditional framework, not a biomedical diagnosis.

Therapies that may be used

Care is limited to gentle, supportive measures chosen for the individual:

• Warm, regular meals suited to your swallowing and reflux, and a steady daily routine
• Gentle warm oil massage of intact skin on the hands and feet, avoiding ulcers and open skin
• Stress-reducing practices such as breathing exercises and gentle yoga within your limits
• Individually prescribed herbs, only after review of your medicines and the cautions below

Panchakarma, the traditional cleansing program, is not appropriate during pregnancy, acute illness or frailty, and we would not offer it as a treatment for this condition, particularly with swallowing problems or lung involvement.

What the research shows

No clinical trials or systematic reviews of Ayurvedic treatment for scleroderma were found in PubMed. A review of complementary therapies lists turmeric among options that some studies suggest may help in systemic sclerosis, but notes that large randomized trials have not been done.^[7]

Product quality is a concern: about one fifth of Ayurvedic medicines bought online in one study contained detectable lead, mercury or arsenic.^[11]

Herbal medicine as supportive care in CREST Syndrome

Herbs and formulas that have been studied

No trials of herbal medicine for CREST syndrome or systemic sclerosis were found. Herbs have been studied for primary Raynaud's phenomenon, including the Chinese formulas Buyang Huanwu Tang and Danggui Sini Tang.^[12] In a survey of 100 patients with systemic sclerosis, 55 had used herbal products, most often green tea, St John's wort and turmeric.^[5]

What the research shows

A meta-analysis of 8 randomized trials with 674 participants found that some Chinese herbal formulas improved overall symptoms of primary Raynaud's phenomenon more than nifedipine in single trials. The methodological quality was generally poor, none reported attack frequency or adverse events, and the authors called the benefit inconclusive.^[12] Primary Raynaud's is a different condition from the secondary form seen in CREST syndrome, so these results may not apply. Survey patients said herbs helped, but the authors noted that studies of effectiveness are insufficient.^[5]

Safety and herb-drug interactions

Herbs can interact with medicines, and CREST syndrome is often treated with several. A literature review documented that St John's wort lowered blood levels of several drugs, including cyclosporine and tacrolimus, and bleeding with warfarin and several herbs.^[13] Tell us about every medicine you take, including calcium channel blockers, immunosuppressants and acid reducers, and have herbs reviewed before use.

Herbal and dietary supplements are a recognized cause of liver injury, which matters if your medicines are processed by the liver.^[14] Tell us if you are pregnant or breastfeeding, or have liver or kidney disease.

Translating traditional medicine into modern biological language

The comparisons here are interpretation, not equivalence. Chinese medicine would describe cold, pale fingers and tight skin using patterns such as cold congealing in the channels, or stagnation of qi and blood. Ayurveda would speak of Vata imbalance and weak digestion. These do not map onto the autoimmune, vascular and fibrotic processes described by researchers.^[3]

Researchers studying acupuncture look at nerve reflexes and immune signaling as possible ways it might affect blood flow and pain.^[9] Whether this applies to scleroderma has not been tested.

How this care fits with your medical care

CREST syndrome needs treatment and monitoring by a rheumatologist and often other specialists. For a condition like this, we ask that you are under a physician's care for it and that your treating team knows you are receiving complementary care.

Do not stop or change prescribed medicines without your prescriber. We do not diagnose or manage the disease, order its tests or replace your physicians.

Please bring your diagnosis, a list of medicines and supplements, recent lung, heart and swallowing test results, and note any finger sores or ulcers.

When to seek urgent medical care

Scleroderma can cause sudden, serious complications, including in the kidneys, lungs and fingers.^{[2, 3]}

• New or fast-worsening shortness of breath, chest pain or fainting
• A sudden severe headache, high blood pressure or reduced urination, which can signal kidney crisis
• A painful finger or toe that stays white, blue or black, or a digital ulcer with pus, spreading redness or fever
• Choking, food stuck when swallowing, or vomiting blood
• Black stools or severe abdominal pain

If any of these occur, call 911 or go to the nearest emergency department.