Limited systemic sclerosis (limited scleroderma): a patient guide

Systemic sclerosis is an autoimmune condition in which the immune system attacks the body's own cells, leading to thickening and hardening of the skin and connective tissue. Scleroderma and Raynaud's UK (SRUK) explains that systemic sclerosis has two main types, limited and diffuse. Arthritis UK describes the limited type as a milder form that only affects the skin on the face, lower arms, hands, lower legs and feet, which normally progresses slowly, although it can affect the lungs and digestive system too.

The older term scleroderma literally means hard skin. Arthritis UK points out that systemic sclerosis is not the same as localised scleroderma (morphoea), which only affects the skin, and the NHS lists localised scleroderma as a separate main type. The two should not be confused, because the monitoring and outlook are different.

The NHS says systemic sclerosis mostly affects women and usually develops between 30 and 50 years of age, while Arthritis UK gives 25 to 55 as the usual range. Children are rarely affected, according to the NHS.

For many people the first sign is Raynaud's. The NHS describes Raynaud's as blood flow stopping properly to the fingers and toes, with the skin turning white, then blue, then red as flow returns, and says it is common and usually not severe, but can sometimes be a sign of a more serious condition. Arthritis UK says many people have Raynaud's without ever having systemic sclerosis, but most people with systemic sclerosis will have it at some time.

Skin and other changes then develop gradually. Arthritis UK says most people find systemic sclerosis affects just a few parts of the body and comes on gradually, may slowly get worse, but usually settles to become stable after a few years. For others the digestive problems cause the most trouble. Because the picture differs so much, your specialist team is best placed to say what your own pattern is.

The NHS lists the typical symptoms of limited cutaneous systemic sclerosis. Arthritis UK adds detail on the common skin and other changes.

• Raynaud's: fingers and toes turning white or blue in the cold, sometimes years before other symptoms.

• Thickening and hardening of the skin on the hands, feet and face, with swelling of the hands and feet especially in the morning.

• Small red spots on the skin of the face, hands and arms, which Arthritis UK calls telangiectasia and says are harmless.

• Hard lumps under the skin, including white chalky calcium deposits, often on the fingertips.

• Heartburn and problems swallowing, which most people with systemic sclerosis have to some extent.

• Open sores or cracking over the fingertips because of poor blood supply, in later disease.

• Pain or stiffness in joints and, sometimes, muscle weakness.

The NHS advises seeing a GP if you have Raynaud's that is very bad, getting worse or affecting daily life, symptoms on only one side of the body, joint pain, skin rashes or muscle weakness alongside it, or if you are over 30 and have Raynaud's for the first time. A GP may arrange tests if Raynaud's could be a sign of a more serious condition.

Once diagnosed, bring new breathlessness, a persistent dry cough, worsening swallowing difficulty or non-healing fingertip sores to your specialist team promptly, because these are the features that monitoring is designed to catch. For severe difficulty breathing, a tight or heavy chest or sudden confusion, the NHS says to call 999 or go to A&E.

There is no single test. Arthritis UK says the thickening of the skin is often the key reason a doctor makes the diagnosis, and that tests help find out whether other parts of the body are affected, including blood tests, X-rays and CT scans, breathing tests, a heart scan, stomach tests, skin biopsy and capillaroscopy of the nailfold vessels.

Ongoing monitoring is a core part of limited disease. The NHS says you will need regular blood pressure checks and other tests to check for problems with organs, including pulmonary hypertension. Arthritis UK describes pulmonary hypertension, narrowing of the blood vessels of the lungs leading to high blood pressure in the lungs, as a rare complication for which you have regular tests of lung and heart function.

The NHS lists medication to improve circulation, medicines that reduce the activity of the immune system and slow progression, steroids for joint and muscle problems, moisturising the skin, and medicines to control other symptoms such as pain, heartburn and high blood pressure. Arthritis UK names nifedipine and sildenafil as drugs that improve circulation in Raynaud's, iloprost by infusion for painful ulcers that will not heal, and antacids or proton pump inhibitors for heartburn.

Self-management is substantial. The NHS advises keeping the hands and feet warm with thick gloves and socks, eating healthily, exercising regularly and stopping smoking. Arthritis UK suggests keeping warm from top to toe, wearing a hat, using layers, protecting broken skin with dressings, moisturising after hands have been in water, and avoiding strong detergents and soaps containing lanolin. It recommends small, frequent meals, eating slowly and raising the head of the bed for heartburn.

Red spots can be covered with make-up and, in some hospitals' specialist dermatology units, treated with laser, as Arthritis UK describes. Stress can reduce blood flow to the hands, so it advises talking about stress or low mood with a healthcare professional or a rheumatology nurse.

The NHS says there are many therapies and lifestyle changes that can reduce the impact of scleroderma on your life. Physiotherapy and stretching exercises can help keep muscles supple and loosen tight skin, and an occupational therapist can advise on changes to the home and equipment. Arthritis UK describes aids and gadgets and mentions that people in England may be entitled to support from their local authority with everyday tasks, depending on eligibility.

The NHS says women with systemic sclerosis may find it harder to get pregnant and may have a slightly higher risk of miscarriage and premature birth, but that a pregnancy planned with a doctor during a stable period can go well. SRUK is the UK charity that supports people with scleroderma and Raynaud's.