What is Scleroderma?
Scleroderma is an umbrella term that refers to the various different types of this rare and complex condition.
General Questions
Systemic sclerosis is a chronic disease but is variable in severity, between patients and over time. Some forms of the disease are more active in the early stages but stabilise or improve later. The term ‘remission’ generally describes a phase when the disease is no longer active. It is an area of ongoing research to better define remission in systemic sclerosis, but it is certainly possible for the activity of the disease to reduce, and for skin and other aspects to improve, particularly after intensive treatments such as stem cell transplantation.
Fatigue is a frequent symptom in systemic sclerosis and many other autoimmune rheumatic diseases. The cause for fatigue is unclear and may involve different mechanisms. There is emerging evidence that regular exercise and some prescription treatments used for connective tissue disease can be helpful, such as hydroxychloroquine. It is important to discuss symptoms of fatigue so that other medical causes can be excluded such as abnormal thyroid function tests, anaemia or iron deficiency. Correcting these associated causes can be extremely helpful. People with systemic sclerosis sometimes report benefit from nutritional supplements and vitamins.
Localised Scleroderma
Yes, often. Achy joints and exhaustion is very common when it’s inflamed. Is a feature of inflammatory disease generally.
The Lungs
Patients with lung fibrosis should generally continue to take as much exercise as is possible within the limits of their symptoms. Even if breathlessness occurs it is still usually safe to exercise at your own pace, but situations that reduce blood oxygen levels severely should be avoided. This can be tested in the hospital during a standard six-minute walk test. Regular exercise may improve “deconditioning” that is a common problem in systemic sclerosis where the heart, lungs and muscles are less efficient at delivering increased oxygen to tissues during exercise.
Because a cough has a number of causes, like acid reflux, heartburn or post nasal drip, it doesn’t necessarily mean your lung function is getting worse. Dr. Elizabeth Renzoni recommends looking out for breathlessness. Breathlessness on exertion. Checking if you are still able to do as much as you were able to do 6 months before? For instance while using a flight of stairs or walking a certain length. A cough can be a cause for concern for worsening, but breathlessness is worth contacting your treatment team about.
G.I. Tract
Although there is no specific diet recommended in systemic sclerosis many patients describe improvement in symptoms with dietary adjustments. Spicy and hot foods may aggravate symptoms of heartburn and all food should be chewed well and consumed with fluids if there are swallowing difficulties. Some patients with bloating, diarrhoea, or constipation report that their symptoms improve from excluding wheat; whereas others may benefit from a reduction in dairy or red meat. However, it is important to maintain a good dietary intake to avoid becoming malnourished. Many scleroderma patients have a reduced appetite and so there is the risk of losing weight. If that is severe then advice from a dietician may be helpful.
Vomiting can occur in systemic sclerosis due to inflammation in the stomach (gastritis), or slow emptying of the stomach as part of the general slowing of intestinal motility in the disease. It can be treated by anti-sickness mediation and adjusting the portion size, timing and frequency of meals. However, any new or severe symptoms in systemic sclerosis should be discussed with your clinical team in case further investigation, such as an endoscopy (gastroscopy), is required.
The Skin
Skin itching can occur in scleroderma and in the early stages of the disease can reflect inflammation in the skin. Later, dryness of the skin can be a major problem. Applying moisturisers can be helpful and avoiding strong soaps. Sometimes medication can help such as antihistamines or other approaches, but it remains one of the most difficult symptoms to deal with, and is an area that is being actively researched to see if some of the drugs being developed for eczema or other diseases might also be helpful.
