Sarcoidosis


What is it about ?

Sarcoidosis is a condition characterized by the appearance of spontaneous inflammation in one or more places of the body. It is a systemic disease, that is, a disease that can simultaneously affect several organs and joints. The organs that are most often affected are the lungs, lymph nodes, skin, liver and eyes.

The causes of the condition are not known. It is assumed that this is a autoimmune disease, which means that the immune system turns on its own cells.

The disease can be acute and transient in nature, but it can also become chronic. The form acute usually does not last more than two years; the chronic form can persist for many years. This form can cause permanent damage to organs.

What is its frequency?

Sarcoidosis is found all over the world. Its frequency is highest in northern Europe and among black-skinned people. In Scandinavia, 30 to 100 people in 100,000 suffer from sarcoidosis.

The condition manifests itself mainly between the ages of 20 and 40. Sarcoidosis can also occur at a later age, but in this case its course is rather chronic. The disease is very rare in children.

How to recognize it?

About half of all cases are discovered by chance, during an x-ray of the lungs prescribed for another condition. Symptoms, when present, are usually not severe. If so, they are reminiscent of a flu and consist of fatigue, fever, muscle pain and cough. Many people have sarcoidosis without knowing it.

Acute sarcoidosis

Acute sarcoidosis, on the other hand, is often accompanied by several serious symptoms. The disease begins as a severe flu, but other alarming symptoms appear after a few days, such as:

  • formation of painful, red-purple lumps on the skin of the shins (erythema nodosum): this symptom is observed mainly in women under 45 and may be the first and only symptom;
  • pain, redness, warmth and swelling in the joints, mainly fingers and toes;
  • fever ;
  • cough and shortness of breath following involvement of lung tissue;
  • swollen lymph nodes, mainly in the rib cage;
  • pain and redness in the eye ;
  • sometimes decreased visual acuity due to inflammation of the iris and sometimes also from the area around it;
  • dry eyes due to reduced tear production following damage to the tear glands;
  • swelling of the salivary glands ;
  • pain, redness and swelling of old scars.

Acute sarcoidosis usually resolves spontaneously within two years, especially when it is confined to the chest (lungs and lymph nodes in the rib cage) and when it begins with erythema nodosum.

Chronic sarcoidosis

About 15% of people with sarcoidosis develop the chronic form. This form varies in severity from person to person. There is no spontaneous healing, but permanent damage occurs to the affected organs; especially the lungs, skin and eyes:

  • some kind of scar tissue replaces normal lung tissue, which causes a form of chronic bronchitis mainly characterized by shortness of breath and cough;
  • raised and discolored skin lesions;
  • increased intraocular pressure (glaucoma), which can cause tubular vision or even blindness.

Rarer lesions:

  • kidney stones and decreased kidney function;
  • attainment of heart muscle (myocardium) associated with arrhythmias and heart failure;
  • enlarged spleen and bleeding disorders;
  • damage to the nervous system leading to paralysis.

A severe sarcoidosis with extra-pulmonary involvement (eg heart, nervous system, eyes or liver) occurs in only 4-7% of cases. The longer the presence of the disease in the lungs, the greater the risk of extra-pulmonary damage.

About 10% of people with sarcoidosis develop severe disability due to permanent organ damage. About 1 in 100 people with sarcoidosis die from the disease, usually from severe breathing problems, but also from heart failure or fatal heart rhythm disturbances.

How is the diagnosis made?

The doctor will always order an x-ray of the lungs and a blood test.

X-ray of the lungs

Of all people with sarcoidosis, 90-95% have changes on the x-ray of the lungs, such as swollen lymph nodes and / or abnormalities in the lung tissue itself. The different stages or types of sarcoidosis are established according to the changes observed.

Blood test

The blood test may be normal in case of sarcoidosis. Since the initial symptoms are often vague, the doctor will usually rule out other illnesses, such as rheumatism and tuberculosis. Sometimes there are abnormalities in blood cells, liver tests and inflammatory factors in the blood.

Additional tests

If these examinations have not made it possible to establish the diagnosis, a biopsy is done. A piece of tissue can be taken from each organ that may be affected by sarcoidosis and then subjected to microscopic examination.

The doctor will refer you to a specialist to confirm the diagnosis and perform further tests. It will be a pulmonologist or rheumatologist, depending on your symptoms. Most often, an examination at theophthalmologist is also needed.

What can you do ?

There really isn’t much you can do. If the existing symptoms worsen or if new symptoms appear, it is advisable to consult your general practitioner.

What can your doctor do?

Acute sarcoidosis usually resolves spontaneously. Your doctor may prescribe certain medications to relieve the symptoms. This is usuallyanti-inflammatory such as ibuprofen, naproxen and diclofenac, and paracetamol against pain and fever.

In severe cases, the specialist will start treatment with cortisone at a high dose, which it will then gradually reduce. If the iris is inflamed, the doctor may prescribe eye drops containing cortisone.

The specialist will continue to follow you up to about a year after your symptoms have resolved.

Want to know more?

Source

Foreign clinical practice guide ‘Sarcoidosis’ (2000), updated on March 21, 2017 and adapted to the Belgian context on March 22, 2019 – ebpracticenet