Introduction

Many patients may not need any drug treatment at all because their symptoms are mild but in some cases the inflammation caused by the disease can lead to the development of granulomas. This can result in the reduction of function of the tissue affected. Where drug therapy is commenced the aim is to enable the lungs or affected organ to continue working to prevent permanent damage and alleviate symptoms. Because of the inflammatory nature of sarcoidosis the treatment of choice are the anti-inflammatory corticosteroids followed by other immune response modulating or suppressing drugs.

CORTICOSTEROIDS

Corticosteroid medications act in a similar way to the body’s natural steroid hormones such as cortisol. These hormones which are normally secreted by the adrenal glands which are situated at the top of the kidneys, are involved in a number of the body’s functions. These include immune response, stress response, regulation of inflammation, carbohydrate metabolism, the breakdown of protein, electrolyte levels in the blood and behaviour.

In sarcoidosis the body’s immune system can go into overdrive often causing inflammation, pain and impaired function of the affected organ. Synthetic corticosteroids are chemically similar to the body’s steroid hormones. When given at increased levels then the body normally produces they act to suppress the immune system and have an anti-inflammatory effect. These drugs therefore operate to counteract the effect of sarcoidosis and are often used in other autoimmune diseases such as inflammatory bowel disease, asthma and arthritis. Corticosteroid medications come in a number of different forms and preparations.

Corticosteroids Orally

Tablets including uncoated prednisolone tablets in strengths 5mg and 20mg and the enteric-coated (a barrier to control where in the digestive system the tablet is absorbed so it is not broken down by acid in the stomach) deltacortril tablets in strengths 2.5mg and 5mg. Initial dose of oral prednisalone is usually 30mg per day. Doses of up to 40mg should be taken as a single dose with or after food in the morning. Higher doses may be divided to morning and afternoon. Avoid taking late in the evening. High doses may be continued for three months. Most doctors will reduce the dose gradually as symptoms are controlled generally by about 5mg a month down to 15mg daily and then it is reduced more slowly to the lowest possible dose to control symptoms.

Corticosteroids Inhaled

For example via inhalers or nebulisers. The steroid is delivered directly to the lungs, with a much lower dose getting into the bloodstream. These should be used regularly each day with appropriate oral hygiene to prevent a dry mouth and sore throat. Good inhaler technique is important.

Corticosteroids Topical

For example creams and eye drops applied directly to the area to be treated, thereby minimising effects on other areas of the body. Because the treatment is direct the dose tends to be lower. Eye drops should be used as directed regarding timing. Always replace the cap and do not use if opened for longer then 4 weeks. Apply creams and ointments sparingly to reduce thinning of the skin and do not cover with dressings.

Corticosteroid by Injections

The treatment is injected into a vein or muscle. This means it has a quicker effect. As when taken orally it has effects throughout the body.

Oral and injected steroids tend to affect the whole body rather then a specific area. Because the treatment of sarcoidosis will probably be for a long time and initially at a high dose, the possible adverse effects of the treatment need to be considered prior to starting it.

Prolonged therapy may lead to adrenal suppression and the body may not be able to respond adequately when it needs to especially when under pressure from disease, trauma, and surgery for example. It is therefore important that patients on long term courses of corticosteroids are aware of the associated problems that may include:

Infections – You may become more susceptible to infection. Infections may be experienced with increased severity or atypical presentation. Special precautions must be taken with chicken pox and measles. If you have not previously had these illnesses avoid exposure to them and if exposed seek medical attention. Fungal or viral infections of the eye may be exacerbated.

Surgery – It is important to inform the anaesthetist prior to any operation.

Withdrawal of treatment – Corticosteroids should not be stopped suddenly if taken for more than a few days. This can result in the body’s own adrenal gland to stop working and may result in steroid deficiency, which can be most serious causing severe hypotension (a drop in blood pressure), fatigue, fever vomiting and diarrhoea, myalgia (muscle pain) and a possible relapse of disease symptoms. Your doctor will advise you on how to reduce the dosage safely.

Osteoporosis – Bone gradually loses its density with age, which makes it more likely to fracture spontaneously or following trauma. Most patients on long term steroids will develop osteoporosis. Ways to prevent it include a low maintenance dose of steroid, regular weight bearing exercise, diet, calcium and vitamin D supplements if tolerated, avoid smoking, reduced alcohol intake and treatment with bisphosphonate which reduces the breakdown of bone.

Diabetes – Corticosteroids act to reduce the effect of insulin a hormone produced from the pancreas to control blood sugar levels. This usually occurs in the first six weeks of treatment and symptoms include unintentional weight loss, thirst, frequent urination and fatigue.

Stomach Ulcers – The risk of stomach ulcers developing is small unless there are other mitigating factors for example a history of previous stomach ulcers or gastric bleeds and concurrent medication that may increase the risk such as non- steroidal anti-inflammatory such as aspirin, ibuprofen and diclofenac. If there is a risk tablets that protect the stomach lining must be considered for example proton pump inhibitors or H2 antagonists.

Psychiatric Side Effects – Mental disturbance may occur – paranoia, depression with risk of suicide, euphoria. Symptoms usually in the first weeks of therapy and if they occur the dose may need to be reduced or stopped.

Cushing’s Syndrome – Symptoms include rapid weight gain, particularly of the trunk and face, a round face often referred to as a “moon face”, excess sweating, thinning of the skin (which causes easy bruising) and other mucous membranes, purple or red striate (also caused by thinning of the skin) and hirsutism (facial male-pattern hair growth). Effects are usually reversible when treatment is stopped.

Other Side Effects – Hypertension, fluid retention, increased appetite, weight gain, thinning and bruising of the skin, difficulty sleeping, nightmares, cataracts and occasionally glaucoma.

AZATHIOPRINE

Azathioprine belongs to a group of drugs called immunosupressants. It works by inhibiting the cells involved in the immune response, thereby suppressing the body’s own defence system. Immunosupressants can be used in the therapy of autoimmune diseases such as sarcoidosis where the immune system is reacting against the body itself. They are also used to treat and prevent the rejection of transplanted organs. Immunosuppressants can be prescribed in some patients in combination with steroid treatment, so that their steroid dose can be kept at a lower level This is known as a ‘steroid sparing’ effect thereby reducing the toxicity associated with high dosage and prolonged usage of corticosteroids.

Azathioprine may be taken in tablet form once or twice a day, with or after food. The dose you take may depend on your weight, and on the results of blood tests carried out before you start the treatment. The dose may change again later with regular monitoring of your blood.

The therapeutic effect may be evident only after weeks or months of taking the drug. As with corticosteroids treatment should not be stopped suddenly and will need to be withdrawn carefully over a period of time following your doctor’s advice.

There are potential risks with taking azathioprine especially bone marrow depression. Therefore it should only be prescribed if you can be adequately monitored for toxic effects throughout the duration of therapy. It is generally suggested that during the first few weeks of therapy that you have your blood cell count checked at least weekly or as recommended by your doctor. This monitoring may be less frequent later in therapy but should be continued. It is important that whilst taking azathioprine that you inform the doctor immediately if you experience any of the following:

  • Unexplained bleeding or bruising
  • Abdominal pain and /or diarrhoea
  • Signs of infection or fever
  • Dizziness or fatigue
  • Bone and muscle pain
  • Kidney problems (ie. a change in colour/frequency with urination)
  • Jaundice (yellowing of skin and whites of the eyes)
  • Any serious skin reaction (ie. blistering or peeling)

People taking azathioprine should be aware that there is an increased risk of skin tumours and so should avoid sun exposure and use high protection sunscreens. Excessive handling of tablets should also be avoided. It should also be avoided during pregnancy. Alopecia (hair loss) can also occur although this usually resolves during treatment.

METHOTREXATE

Methotrexate is an immunosuppressant and anti-metabolite drug affecting the production and growth of cells. It is referred to as cytotoxic which means that it is commonly used to kill tumour cells (please remember sarcoidosis is not a form of cancer), it also helps to reduce inflammation by altering the body’s defences. It is administered orally or via injection. Tablets are available in 2 strengths 2.5mg and 10mg. Tablets are taken once a week on the same day each week, the prescribed dose is usually between 7.5mg and 15mg as a single dose or divided 12 hourly, taken with food.

As with azathioprine treatment may take time to bring about improvement. Full blood tests and kidney and liver function tests will need to be done prior to starting and repeated weekly until well established. This will then be reduced to every 2-3 months. It is very important not to miss any appointment. Again methotrexate has a steroid sparing affect and can be given on its own or in conjunction with low dose corticosteroids. Side effects include:

  • Bone marrow depression: symptoms may include Infection – flu, fever, sore throat.
  • Anaemia – tiredness, shortness of breath or low platelets – bruising, bleeding gums.
  • Pulmonary fibrosis – breathlessness, persistent, unproductive cough
  • Hepatotoxicity – jaundice, deranged liver function tests (it is therefore important to avoid excessive alcohol)
  • Gastro intestinal upset, – nausea, vomiting, diarrhoea
  • Others – sore mouth, rash, alopecia, constipation, dry cough

If you develop these symptoms you should seek medical attention. Taking folic acid supplements or eating more folic acid in your diet can alleviate some of the side effects of methotrexate.

Pregnancy should be avoided during treatment and for 3-6 months after its completion. This applies to both men and women as it affects both sperm and egg production.

HYDROXYCHLOROQUINE

(Plaquenil)

This is best known as a treatment for malaria but can also be used for inflammatory diseases such as sarcoidosis rheumatoid arthritis and lupus.

The mode of action of hydroxychloroquine in suppressing the disease process is unknown but can have a good effect in sarcoidosis with those with skin symptoms or high levels of calcium. It may take several weeks for the beneficial effects to become obvious. It is important prior to therapy to have baseline eye test plus liver and renal function tests. Initial dose 400mg, in divided doses with food or milk. Once the disease improves it may be reduced to a maintenance dose of 200-400mg.

Side effects include rash, diarrhoea and retinal toxicity. Any reduction in night vision or peripheral vision should be reported and caution should be taken with regards to driving.

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