Sarcoidosis – MAX Today

About 20 out of 100,000 Dutch people develop sarcoidosis every year. This means that our country has a total of around 8,000 patients with sarcoidosis. That makes it a rare condition, but one that King Willem-Alexander, for example, has also had to deal with in the past. What exactly is it and can something be done about it? Doctor Ted van Essen explains.

Granulomas

Sarcoidosis, also known as Besnier-Boeck’s disease after its discoverers, is a disease in which inflammation can occur in various organs and joints in the body. In those inflammations many immune cells, white blood cells, are created that accumulate in certain places. These accumulations are also called granulomas and can sometimes be felt under the skin as small nodules. Granulomas can disappear on their own over time, but sometimes they continue to exist and then they can endanger the functioning of organs. Scar tissue or fibrosis may also develop at the site of the granulomas, which can also damage the organs. Because sarcoidosis is caused by a response from the immune system, it used to be called an autoimmune disease. These days, people prefer to call it multi-system disease because it can occur in so many different organs.

2 forms

Sarcoidosis can occur throughout the body, but most often in the lungs. Granulomas are also common in sarcoidosis patients in the lymph nodes, skin, eyes, and joints. It is most dangerous when the accumulations of immune cells occur in the heart or nervous system, but that is fortunately rare. Sarcoidosis is divided into 2 forms, the acute form and the chronic form. In the acute form, which is approximately 30 percent of the cases, patients suddenly develop violent symptoms in a short time. In the chronic form, in 70 percent of the cases, the symptoms arise insidiously and the condition has been around for a long time with vague symptoms before the patient finally goes to the doctor. Then it can take years before the diagnosis is made.

Complaints

The symptoms can be very non-specific with, for example, only fatigue. If sarcoidosis suddenly arises, the symptoms initially resemble those of flu: fever, fatigue, listlessness, coughing and painful joints. How the symptoms develop further depends mainly on the places in the body where the accumulation of immune cells occurs. If that is in the lungs there are symptoms such as stuffiness and coughing. In a more advanced stage, countless scars arise in the lung tissue and the lung capacity and lung function can deteriorate. About 10 percent of patients develop severe lung damage, lung fibrosis, due to sarcoidosis.

Logrgren

Sometimes painful purplish red and thickened spots appear on, for example, both shins or forearms, which is also called erythema nodosum. This also occurs with other disorders. Skin sarcoidosis can also cause specific skin symptoms. If the joints are affected, they can become inflamed and painfully thick and warm. Sometimes eye problems are a first symptom of sarcoidosis (uveitis): decreased tear fluid production, conjunctivitis, blurred vision. Deafness and arrhythmias can also occur. It can also happen that a large number of these complaints occur at the same time, we are talking about Löfgren’s syndrome.

Sometimes the disease also proceeds without clear symptoms and is then discovered by chance. “That is how it allegedly went with King Willem-Alexander. In the past this often happened at the military service inspection, where a photo of the lungs was always taken. The RVD announced in 1999 that crown prince Willem-Alexander was then diagnosed with sarcoidosis in 1991 after a diving examination where spots on the lungs of a prince were found on the lungs. It is said that he had a mild form of the disease, received no medication and was cured by himself. The fact that he ran the New York marathon the following year, in 1992, also seems to indicate that. In 2012, Willem-Alexander opened the Sarcoidosis Center in the Erasmus MC. ”

Cause a mystery

The real cause is still a mystery. The symptoms with sarcoidosis vary widely per patient and also occur with other diseases. And that is precisely why sarcoidosis is sometimes difficult to recognize. There is no specific blood test that can detect sarcoidosis. Blood tests can show whether there are inflammatory values ​​in the blood and whether there are antibodies, proteins that have to do with the immune system, in the blood. With sarcoidosis, an increased level of a certain enzyme (angiotensin converting enzyme, ACE) can sometimes be detected in the blood, and sometimes the level of calcium in the blood or urine is increased. The diagnosis is also likely to be made with a PET scan; with a lung biopsy that is certain. Although not much is known about the disease, it is certain that it is not contagious and that it sometimes occurs more often within one family, so that there may be a hereditary factor. And it mainly occurs between 20 – 40 years, the same in men and women, and very rarely in children.

Cannot heal or treat

At this time it is not possible to cure sarcoidosis with medication or with other treatment. Any medication is only able to suppress the inflammation, not to prevent it forever. Fortunately, it is true that this disease often resolves itself within a few months to a few years. That chance is greatest with sarcoidosis that has arisen acutely: around 80 percent of these patients heal without lasting complaints within 1 to 2 years. With this acute sarcoidosis, medication is therefore often not necessary and it is sufficient to have you checked regularly to monitor the situation of the affected organs.

Suppress

If, on the other hand, the disease has developed gradually, the prospects for spontaneous recovery are less favorable. For this reason, this form is also called chronic. In such cases, periods in which the symptoms are worse and less severe alternate. When the symptoms are very annoying or painful, there is, of course, medication available to combat the pain and to suppress the inflammation. In such a case, the doctor may prescribe the painkiller paracetamol, or anti-inflammatory drugs such as NSAIDs or corticosteroids such as prednisone. Heavier drugs are sometimes required, such as methotrexate or so-called biologicals (TNF-alpha inhibitors). Incidentally, it is true that sarcoidosis may eventually return even after spontaneous recovery. If sarcoidosis is chronic, sometimes only fatigue can persist. Then it is often very difficult to convince medical examiners that there really is a disease. A third of sarcoidosis patients are incapacitated for work.