Crest Syndrome

Overview

CREST syndrome is a long-term condition that mainly affects the skin, the blood vessels and the food pipe (the tube that carries food from your mouth to your stomach). It is a form of scleroderma - an autoimmune condition. Autoimmune means your immune system, the body's defence system, gets confused and turns against your own tissues. In CREST syndrome this leads to extra scar-like tissue building up in the skin and sometimes in internal organs. The name CREST comes from the first letters of five features doctors often see: Calcinosis (small chalky lumps under the skin), Raynaud's (fingers and toes that change colour in the cold), Esophageal problems (the food pipe not moving food along well), Sclerodactyly (tight, hard skin on the fingers), and Telangiectasia (tiny red spots on the skin). Many doctors now call it 'limited cutaneous systemic sclerosis', but CREST is still widely used and understood.

CREST syndrome is rare. It affects only a few people in every 100,000, and new cases are uncommon. Because it is rare, it can take time for anyone - including a doctor - to think of it. That is not a sign that something has been done wrong.

Anyone can develop CREST syndrome, but it is much more common in women, usually first noticed between the ages of 30 and 50. It is more common, and tends to be more serious, in people of African or Native American heritage. It is not infectious and it is not caused by anything you did or did not do.

Symptoms

Common symptoms

• Raynaud's: fingers or toes that go white, then blue, then red in the cold or with stress, often with numbness, tingling or pain
• Skin on the fingers becoming tight, shiny and hard, so the fingertips look tapered
• Tiny red spots (telangiectasia) on the face, lips, hands or inside the mouth
• Small hard lumps under the skin, usually on the fingers, sometimes leaking a white chalky material (calcinosis)
• Heartburn or acid reflux, especially at night or when lying down
• Food feeling stuck when swallowing, or needing to drink to wash food down
• Dry eyes or a dry mouth
• Tiredness that does not improve with rest
• Aching, stiff joints, particularly in the hands
• The mouth opening becoming smaller, with fine wrinkles around the lips
• Sores or small ulcers on the fingertips that are slow to heal

Causes

Main causes

• The exact cause is not known. CREST syndrome is an autoimmune condition, which means the immune system mistakenly targets the body's own tissues.
• The immune system produces antibodies that seem to switch on cells called fibroblasts. These cells then make too much collagen, a protein that normally gives skin its strength - too much of it makes skin tight and hard.
• The same process can narrow small blood vessels, which is why fingers react strongly to cold and why internal organs can be affected.
• It is not caused by diet, stress, lifestyle or anything you did. It is not contagious, and you cannot pass it on to other people.

Risk factors

• Being a woman - about four out of five people with the condition are women
• Being between 30 and 50 years old when symptoms first appear
• A family history of autoimmune conditions - the genetic link is small, and most relatives are completely unaffected
• Being of African or Native American heritage, where the condition is more common and can be more serious
• Long-term contact with silica dust, certain solvents or industrial chemicals in some workplaces
• Possibly certain infections that trigger immune changes - this link is not proven

When to see a doctor

See a doctor urgently if:

• A fingertip that is turning dark, or a finger sore or ulcer that is not healing
• New or quickly worsening breathlessness, or a cough that will not settle
• Food sticking when you swallow, or losing weight without trying
• Heartburn that is very painful, or that comes with vomiting
• A red, hot, swollen and painful finger, particularly with a fever
• Swelling of the hands or feet, or putting on weight quickly

Book a routine appointment if:

• Fingers that go white, blue and red in the cold, with pain, numbness or tingling
• Skin on the fingers becoming tight, shiny or hard
• Tiny red spots on the face, lips or hands
• Small hard lumps under the skin, or chalky material coming from the skin
• Heartburn most days, or a sour taste in your mouth at night
• Dry eyes or a dry mouth that bothers you
• Ongoing tiredness, or aching and stiffness in the hands and joints

Diagnosis

There is no single test for CREST syndrome. A doctor - often a rheumatologist, a specialist in joints, muscles and autoimmune conditions - builds the diagnosis from your story, an examination, blood tests and other checks. Because the condition develops slowly, it can take more than one appointment, and sometimes months, to be sure. Being referred to a specialist does not mean something terrible is happening; it means your doctor wants the right team involved.

Tests that may be done

• Blood tests looking for antibodies, including antinuclear antibody and anti-centromere antibody tests
• Nailfold capillaroscopy - a magnifying test that looks at the tiny blood vessels at the base of your fingernails
• Lung function tests, where you breathe into a machine, to check how well your lungs work
• A CT scan of the chest to look for lung changes
• An echocardiogram (ultrasound of the heart) to check the heart and blood pressure in the lungs
• Blood pressure checks and urine or blood tests to check the kidneys
• A swallowing study, such as a barium swallow or pressure test of the food pipe
• Occasionally a small skin sample (biopsy), though this is often not needed

What to expect at your appointment

You will usually have a first appointment with a specialist who asks detailed questions, examines your hands, face and skin, and arranges tests. Some tests are done the same day, while others need a separate visit or a short wait for results. After that, most people are seen regularly - often once or twice a year - to keep an eye on the lungs, heart, gut and blood pressure. Keep a note of your symptoms, particularly how often your fingers change colour and how swallowing and heartburn are going, as this helps your doctor a lot.

Treatment

CREST syndrome cannot be cured, but a great deal can be done to control symptoms, protect your organs and help you feel better day to day. Treatment is tailored to your symptoms and is reviewed regularly, because what you need may change over time. Most of the care happens at home, supported by your GP and a specialist team.

Self-care at home

• Keep hands and feet warm - gloves, warm socks, hand warmers, and warm water for washing up. Warm up slowly rather than all at once.
• Stop smoking and avoid smoky places, because nicotine and smoke narrow blood vessels and make Raynaud's worse.
• Eat smaller meals more often, and avoid big meals late at night. Try to stay upright for a couple of hours after eating.
• Raise the head of your bed slightly, or use extra pillows, if heartburn troubles you at night.
• Moisturise your skin daily and use gentle, fragrance-free soap. Protect your skin from knocks and cuts.
• Do gentle hand and face exercises to keep movement and skin suppleness - a hand therapist can show you how.
• Use sunscreen and cover up in strong sun, as the tiny red spots can be sensitive to sunlight.
• Check your fingers and toes regularly for sores, and tell your doctor about any that appear.
• Keep up regular dental check-ups, as a dry mouth and a smaller mouth opening can affect your teeth and gums.
• Pace yourself and build rest into your day if tiredness is a problem.

Medical treatments

Doctors use several broad groups of treatment, chosen to match your symptoms. Medicines that help blood vessels stay open and improve circulation can reduce how often and how badly Raynaud's attacks happen. Medicines that lower the amount of acid in the stomach help heartburn and protect the food pipe. Some people are offered medicines that calm the immune system when the condition is active. Creams, ointments and dressings help with tight or dry skin and small sores. Calcium lumps may be treated with minor procedures if they become painful or infected. Blood pressure medicines are also used, mainly to protect the kidneys. Because internal organs can be affected quietly, most people have regular check-ups of the lungs, heart and blood pressure even when they feel completely well. Your doctor will talk through the options, the benefits and the possible side effects, and choose what suits you.

When is surgery considered?

Surgery is not usually needed. It may occasionally be considered for a very painful calcium lump, for severe Raynaud's that has not improved with other treatment (an operation that interrupts the nerve signals to the fingers), or for a badly narrowed food pipe. Decisions like these are made with your specialist team, and you would be given time to weigh up the pros and cons.

Living with this condition

CREST syndrome is a long-term condition, and most people settle into a routine that keeps symptoms manageable. Small daily habits make a real difference - a pair of gloves by the front door, a warm scarf and hat, a flask of a warm drink on cold days, and a bag with your usual essentials. Plan demanding tasks for when you have the most energy, and build in breaks. Keep a simple symptom diary so you can see patterns and share them at appointments.

Lifestyle tips

• Stop smoking, or ask for help to stop - this is one of the single most helpful things you can do for your circulation
• Keep physically warm, with layers, gloves hats and warm footwear, and avoid sudden changes in temperature
• Stay active in a gentle, regular way - walking, swimming, stretching or tai chi all help circulation and stiffness
• Protect your hands: use tools with padded grips and avoid gripping cold metal or carrying heavy bags by the fingers
• Eat regular, smaller meals and stay upright after eating to reduce reflux
• Keep up your dental care and tell your dentist about your condition
• Limit alcohol, which can worsen heartburn, and avoid very hot, very cold or very spicy food if it triggers symptoms
• Look after your sleep and build in rest, as tiredness is common

Diet and exercise

There is no special diet that cures CREST, but eating well supports your energy, skin and gut. Smaller, more frequent meals are often easier than three large ones, and chewing slowly helps if swallowing is tricky. If heartburn is a problem, try cutting back on very rich, fatty or spicy meals, and avoid eating close to bedtime. Soft, moist foods can be easier if your mouth feels dry. Gentle exercise keeps joints supple and helps circulation - aim for something you enjoy and can keep up, and ask your doctor about a physiotherapist or hand therapist if you would like a tailored plan.

Mental health and emotional wellbeing

Living with a long-term condition can be tiring, and it is very common to feel low, worried or frustrated at times. Fingers that hurt in the cold, a diagnosis that takes time, or simply the effort of managing appointments can all take a toll. These feelings are understandable, and support is available - talking to family, a counsellor, or your specialist team often helps a lot. If you ever have thoughts of harming yourself, please get help straight away: call your local emergency number, go to your nearest emergency department, or contact a local crisis line.

Prevention

There is no proven way to prevent CREST syndrome, and it is not caused by anything you did. What you can do is reduce how often symptoms flare and protect your body: keep warm, stop smoking, eat and rest well, and attend your check-ups. For people already diagnosed, noticing changes early and reporting them promptly is the most useful kind of prevention, because small problems are easier to treat.

Screening programmes

There is no general screening test for CREST syndrome in people without symptoms. Once the condition has been diagnosed, however, regular check-ups act as screening: lung function tests, a chest scan, an ultrasound of the heart, blood pressure checks and kidney tests, usually every year or so. These are done even when you feel well, because they can spot problems early when they are easiest to manage.

Complications

If left untreated

• Fingertip ulcers and scarring, which can be painful and slow to heal
• Severe Raynaud's attacks, rarely leading to damage to the fingertip tissue
• Troublesome heartburn and narrowing or tightening of the food pipe, which can make eating difficult
• Poor nutrition and weight loss if swallowing becomes hard
• Scarring of the lung tissue (pulmonary fibrosis) or high blood pressure in the lungs (pulmonary hypertension)
• Heart rhythm problems or heart strain
• Kidney problems, which are less common in this form of scleroderma but can occur
• Dental problems and dry eyes or mouth that affect daily comfort
• Low mood, anxiety and reduced quality of life if symptoms are not well supported

Long-term outlook

The outlook for most people with CREST syndrome is encouraging. Serious internal organ problems are less common than in the other, more widespread form of scleroderma, and most people live a normal or close to normal length of life. The condition usually moves slowly, and regular check-ups mean changes can be caught early, when they are easier to treat. Many people find that with a good routine, warmth, and a team they trust, they are able to carry on with the work, hobbies and relationships that matter to them.

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