SARCOIDOSIS FOR THE PATIENT

Norman T. Soskel, M. D.

Modified from D.G. James

Contents

Introduction Lungs Skin Eyes Other Systems Skin Tests Blood Tests Other Tests

Treatment Questions and Answers WASOG Useful Addresses

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INTRODUCTION

We do not know the cause of sarcoidosis but it is a form of inflammation which involves the lungs, eyes, skin and other internal organs in young adults. It is extremely rare in children and in the elderly. It is possible that certain persons are peculiarly susceptible to develop this inflammation because it is most frequently seen in blonde Scandinavians, red-headed Irish, Japanese, black Americans and West Indians. It is rare in the Chinese, Greeks or Cypriots. It used to be rare in India but it is now becoming more frequently seen there. Why is this so? Is it only recognized in sophisticated communities with modern diagnostic equipment? Will it be seen more often in Third World countries when chest x-rays are done on the majority of the population? Why is it that sarcoidosis develops in West Indians when they come to live in London, or Martiniques in Paris or Puerto Ricans in New York City? Any clues from patients may lead us to a better understanding of the cause of the disorder.

The term sarcoidosis is derived from the Greek root SARKO meaning flesh. The same source produces the word sarcasm, which means "to cut the flesh" or the word sarcophagus, which is a box to carry flesh. The "OID" comes from the Greek "like" so SARCOID is flesh-like or fleshy. Sarcoidosis is pronounced SAR-COY-DO-SIS.

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LUNGS

The lungs and the lymph glands inside the chest are the most frequently affected suggesting that the patient is reacting to something that he has inhaled from the air.

Most patients do not have any complaints but some experience difficulty in breathing particularly with heavy exercise. A few have a dry cough, muscle weakness, lethargy and listlessness. Whether these symptoms are present or absent the chest x-ray will almost always show evidence of sarcoidosis. There are many causes for this x-ray shadow so the doctor will do blood tests, analyze samples of fluid from the bronchial tubes and carry out lung function breathing tests. The chest x-ray will be repeated at intervals until it has cleared.

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SKIN

Erythema Nodosum consists of painful red blotches which develop on the skin in women most often in Springtime. Accompanying it are severe joint pans in knees and ankles. Although the red spots may frighten the patient it is harmless and will eventually subside with the help of pain-killing tablets.

Occasionally, there is burning or itching eyes, blurring of vision and increased tears.

It may occur during pregnancy or lactation; the patient should be reassured that it is harmless and it will not affect the unborn baby or infant at the breast. This form of sarcoidosis has an excellent outlook.

Lupus Pernio is a troublesome rash which develops slowly on the nose, face, ears and thereabouts.

The patient may complain of a stuffy nose because it is blocked by sarcoid tissue.

It is particularly common in West Indian and African women. It is a blemish which needs to be camouflaged with cosmetics. Various medicines, including once-weekly methotrexate, help to keep it under control.

Other Skin Plaques on the body may look like psoriasis. It may be necessary to remove a piece of skin to view it under the microscope for proof of sarcoidosis.

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EYES

The eyes may become red and inflamed so the patient is examined by an eye specialist who uses a special microscope to look into the eyes. This is called slit lamp examination of the eyes. He may also do another test called fluorescein angiography to get a better view of the back of the eye. A dye, fluorescein, is injected into a vein, which transports it to the eye. A special camera photographs the dye as it travels through the eye. It is a harmless but most valuable investigation because it shows up the presence of sarcoidosis in the back of the eye and alerts the doctor to give immediate steroid treatment to save the eyes from long-term damage.

Whenever the eyes are affected steroids are prescribed either as eye drops or as tablets by mouth or both in order to save vision.

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OTHER SYSTEMS

Sarcoidosis is sometimes described as multisystem. This means that it affects several different systems of the body. Mention has already been made of the lungs, skin and eyes. Sometimes but far less often it may involve liver, kidneys (including kidney stones), heart, brain, lymph glands, bone, salivary glands. The doctor may use various tests - x-rays, electrocardiogram, the use of radioactive tracers, brain scan or liver biopsy, to show up these various organs and to judge whether they have been affected. He is then able to give appropriate treatment and cure the disorder.

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SKIN TESTS

There are two skin tests which are commonly used in investigation.

Tuberculin Skin Test. An injection is made into the skin and the response is read after two or three days. It is commonly negative in sarcoidosis, and it is given the technical term of cutaneous anergy or depression of delayed-type hypersensitivity, or impaired cellular immunity. If this test shows a big red patch then it suggests that the body defenses are working well (not sluggish as in sarcoidosis) and this is a point against sarcoidosis.

Kveim-Siltzbach Skin Test. Kveim was a Norwegian dermatologist and Siltzbach was a New York chest physician. This test uses specially-prepared sarcoid tissue which is injected into the skin and the response is read after one month. If the patient has sarcoidosis a little lump of sarcoid tissue develops at the injection site in the course of one month.

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BLOOD TESTS

Serum angiotensin converting enzyme level is increased in many patients with sarcoidosis. It is a useful tests which is performed by many physicians when they want to know about the progress of the disease and the effectiveness of steroid therapy.

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OTHER TESTS

You will occasionally hear or read about other tests and techniques including Gallium lung scan, bronchoalveolar lavage, thallium heart scan and an open lung biopsy. Your doctor will explain to you, if any of these is necessary.

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TREATMENT See also Therapy

These are the four steps in the management of patients with suspected sarcoidosis:

1. The symptoms and physical signs notes when the patient is examined raises the possibility of sarcoidosis, so the doctor does a chest x-ray and lung function tests.

2. The next step is to obtain some tissue to look at under the microscope. This helps to clinch the diagnosis.

3. The next step is to decide whether the disorder is active or whether it has burnt itself out and is inactive. There are various tests which decide this point.

4. if the sarcoidosis is active, then it may need to be treated with steroids. Most patients make a complete recovery without treatment. Only the minority need treatment with the cortisone group of drugs. Steroid treatment is very effective in overcoming involvement of the eyes, lung and brain; and it may also be necessary for skin involvement.

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QUESTIONS AND ANSWERS

Q. Will it affect my pregnancy?

A. No. Carry on with your pregnancy. In fact, patients improve during pregnancy for they make their own steroids in the body and this helps to overcome sarcoidosis. However, three months after delivery, the doctor will be alert to the possibility of recurrence when the steroids of the body are once again at a lower level.

Q. Will it affect my life style?

A. No. Patients with sarcoidosis lead a normal life in every way. They carry on with normal employment and they are allowed a full social and athletic life. Sarcoidosis patients have won medals in the Olympic Games, become Judo champions, and sailed single-handed around the world.

Q. Will I pass it on to my children?

A. Almost certainly not. Familial sarcoidosis is extremely rare.

Q. Is my skin rash catching?

A. Never. Your friends and family must be reassured that they will not contract sarcoidosis either by close physical contact or by sharing intimate objects.

Q. Is sarcoidosis a form of skin or lung cancer?

A. Definitely not. The cause of sarcoidosis has so far eluded doctors but we know for certain that cancer is not a part of it.

Q. My doctor mentioned something about Hodgkin's Disease. Is sarcoidosis a form of cancer or Hodgkin's lymphoma?

A. No. Sarcoidosis is not related to lymphoma, Hodgkin's Disease, leukemia or cancer.

Q. Do I have to get rid of my cat?

A. No. You need not get rid of your pets.

Q. Do I have to observe dietary precautions?

A. Occasionally, calcium and vitamin-D rich foods including milk, cheese, other dairy products, vitamins and calcium supplements may cause the blood calcium level to rise which, if not corrected, may produce stones and gravel in the kidneys. If your calcium level is high, avoid calcium rich substances and too much direct sunshine.

Q. Should I restrict my exercise?

A. No, you should lead a normal life. If your lungs cannot keep up with you, you will be breathless. In that case, you should stop and rest.

Q. How long will it take to get better?

A. If you got ill quickly (for example, with the sudden onset of erythema nodosum), then you can expect to be completely better within one year. If your illness came on very gradually, it may grumble on for much longer.

Q. Don't these steroid tablets have side effects?

A. Yes, in some people. You are more likely to develop them if the dose is high and the tablets are taken for a long time. Whenever your doctor suggests steroids, he will weigh the benefits against the possible disadvantages.

Discuss them with him - they may include weight gain, indigestion, raised blood pressure, diabetes and loss of calcium from bones.

Q. Can I travel by air? Will it have any effect on my lungs?

A. Most people have no problems at all. Only a few patients may have advanced scarring of their lungs. Such patients might need supplementary oxygen.

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WASOG

World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) was formed to encourage research and information on sarcoidosis. Its members include sarcoidosis specialists in thirty countries and all continents of the world. It holds Sarcoidosis Conferences at frequent intervals, and it publishes a scientific journal, Sarcoidosis Vasculitis and Diffuse Lung Diseases, all designed to keep the world informed of the latest news on the disorder.

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