Sarcoidosis is an inflammatory disease of the connective tissue, the cause of which is still not definitively understood. Sarcoidosis affects the entire body, but is particularly common in the lymph nodes and lungs. Also known as Boeck’s disease, sarcoidosis is a relatively rare disease.
Sarcoidosis (Boeck’s disease).
The disease sarcoidosis occurs worldwide. Sarcoidosis is particularly common in Sweden and Iceland. Exact numbers of this disease in Germany are unknown; estimates are 40,000 affected persons. Since sarcoidosis does not always cause symptoms, experts assume that the actual rate of the disease is even much higher.
Nevertheless, sarcoidosis is one of the rare diseases. Other names are – after the first descriptors of this clinical picture – Boeck’s disease and Schaumann-Besnier’s disease, for the acute course form also Löfgren’s syndrome.
Lungs, lymph nodes, and liver often affected
Sarcoidosis is a systemic disease, meaning it affects the entire body. Characteristics are granulomas – small connective tissue nodules as signs of inflammatory reactions. In principle, these can occur in any organ tissue, but are almost always found at least in the lymph nodes and lungs. The liver is also relatively frequently affected, and sarcoidosis occurs somewhat less frequently in the eyes, skin or bones.
The type and extent of sarcoidosis symptoms depend on the affected region, and general symptoms are not always present either. The likelihood of developing the disease is particularly high between the ages of 20 and 40.
Sarcoidosis: progression of forms
In principle, a distinction is made between an acute and a chronic form of progression. The former sets in suddenly, but usually regresses (in 80-90%) after a few weeks to months without further impairment. Chronic sarcoidosis develops slowly and is usually unnoticed at first. Symptoms that lead sufferers to the doctor are often discomfort in the eye or changes in the skin. In about half of cases, chronic sarcoidosis comes to a halt; in the others, it progresses.
Sarcoidosis: causes unknown
The causes of sarcoidosis remain unclear. In principle, it is assumed that there is a pathological defense reaction of the immune system that leads to the formation of the numerous connective tissue nodules (granulomas). However, the exact trigger is not known; substances that are absorbed via the lungs and an infection similar to tuberculosis are discussed, among others. A hereditary component is also suspected, which at least increases susceptibility to sarcoidosis.
Although the nodules are benign, their number and location can lead to impaired organ function. As a result, the granulomas can regress completely, but they can also scar over to become functionless tissue – however, it is not possible to predict the course of the disease. The drug interferon is also discussed as a possible trigger of sarcoidosis, which is why it should not be used in cases of known sarcoidosis.
