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Laatst geupdate op 14 October, 2021

What is sarcoidosis?

Sarcoidosis is a rare disease in which inflammation occurs due to an unknown cause. During such inflammation, white blood cells accumulate. These accumulations are called granulomas. The inflammations can occur almost anywhere in the body. The most common complaint is fatigue, which many people with sarcoidosis experience as the main limitation.

There is no medicine that can cure sarcoidosis; any medication that may be prescribed is aimed at suppressing the inflammation. Most patients are between 20 and 40 years old when sarcoidosis is discovered.

Clinical picture

Sarcoidosis is most commonly found in the lungs, liver, lymph nodes, eyes and skin. Depending on the location of the granulomas, many different complaints can occur. However, there are also many general symptoms such as fatigue, fever, muscle aches, concentration problems,sleeping problems, weight loss and night sweats.

In most patients, the granulomas disappear within two to three years, sometimes even without medication. However, it is not uncommon for complaints to persist even after that, which we call residual complaints. In some patients the granulomas remain despite the medication. Their complaints persist as well.

The breadth and diversity of symptoms also apply to the course and consequences of sarcoidosis. These differ greatly from person to person. There are people who have hardly any symptoms, who can continue to work full-time and who are able to fulfil their family life. On the other hand, there are sarcoidosis patients who become completely disabled before the age of 30 and can barely maintain a social life.

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More facts and figures about the disease sarcoidosis and it’s treatment (Dutch, pdf)

Progression

The course of sarcoidosis is difficult to predict. The disease is characterised by an irregular course. Periods in which the patient feels relatively well can alternate with periods in which the symptoms increase or the disease gets (temporarily) worse. Sarcoidosis with an acute onset generally has a more favourable course than sarcoidosis with a gradual onset.

In 70% of all patients with sarcoidosis, the disease disappears within a period of two to three years, sometimes even without medication. Some 20-30% of patients have permanent damage to the lungs, for example, and for a very small proportion of patients (1-5%) sarcoidosis is the cause of death.

Every form is different

Not every form of sarcoidosis follows a similar course. For example, the prognosis for cardiac sarcoidosis and neurosarcoidosis is less favourable than for sarcoidosis of the eyes. Of course, the degree of scarring is also closely related to the course of sarcoidosis.

Rare disease

How common is sarcoidosis?

Anyone can get sarcoidosis: it occurs in all ethnic groups and in both men and women. The prevalence is estimated at about 50 per 100,000. Estimates of the number of people in the Netherlands who have the disease range from 5,000 to 8,000 (figures from 2012-2013). The broadness of this range is due to a lack of good epidemiological research. However, sarcoidosis is more common in some regions, in some ethnic groups and in some families than in others. For example, negroid young women (40 in 100,000), as well as Scandinavian (64 in 100,000), Asian, German, Irish and Puerto Rican populations are more frequently affected by sarcoidosis. The course of the disease is also often more aggressive in these groups. This suggests that, although clear hereditary factors are lacking, a relationship with certain genes may be assumed, even though there is no hard evidence for this yet. It is estimated that 20 people per 100,000 inhabitants develop sarcoidosis in the Netherlands each year.

Age

In almost all cases, sarcoidosis patients are between 20 and 40 years old when the disease is diagnosed. Patients who are younger than 10 or older than 60 at the time of diagnosis are the exceptions. Though sarcoidosis can manifest itself in this group, it’s course is almost always general and usually limited to general malaise and fatigue. In the Netherlands, the ratio of men to women is equal. The average age at diagnosis in men is about 32, while in women sarcoidosis is discovered at the average age of 36.

History

Before the end of the 19th century, two other dermatologists published on skin disorders in sarcoidosis. They were Ernest Besnier and Caesar Boeck. For a long time the disease was therefore called Besnier-Boeck. This name is still used today. In 1914, the ophthalmologist Jörgen Schaumann observed that Besnier Boeck is a disorder involving several organs. In some publications that followed the disorder was referred to as Besnier-Boeck-Schaumann.

The name Besnier-Boeck has since been replaced by ‘sarcoidosis’. This comes from the Greek language in which ‘sarcoid’ means fleshy and ‘-ose’ means disease. Literally translated, it is a fleshy disease.

1869 A patient is diagnosed with skin lesions on the fingers, back of the hand and shin.

1877 Jonathan Hutchinson described these lesions in the Illustrations of Clinical Surgery.

1898 This condition was named ‘Mortimer’s malady’.

1889 Ernest Besnier described skin lesions which he called ‘lupus pernio’.

1899 Caesar Boeck published an article describing not only skin lesions but also lymph node swellings. Boeck was also the first to perform histological examinations, introducing the term ‘multiple benign sarcoid of the skin’.

1909 Christian Heerfordt, a Danish ophthalmologist, described a syndrome he called ‘febris uveo-parotidea subchronica’, which he saw as a manifestation of tuberculosis. It was not until 1936 that this syndrome was ‘recognised’ as sarcoidosis.

1914 Jörgen Schaumann was the first to recognise that it was a disease affecting several organs and tissues and proposed the term ‘lymphogranulomatosis benigna’.

1920 Otto Jüngling named bone cysts in sarcoidosis as ‘ostitis tuberculosa simplex’. It should be noted that these bone lesions were also described by Karl Kreibich in 1904 in a patient with ‘lupus pernio’.

1941 The Kveim test was introduced, the importance of which lay particularly in the initiation of immunological examination in sarcoidosis. Later, Siltzbach conducted many studies on the Kveim reaction.

1946 Sven Löfgren described an acute form of sarcoidosis accompanied by ‘erythema nodosum’. Remarkably, sarcoidosis was then still regarded as a form of tuberculosis.

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