Sarcoidosis

Sarcoidosis

What is it ?

Sarcoidosis is an inflammatory disease that causes cell clusters to form in organs, preferably in the lungs. It is also referred to as Besnier-Boeck-Schaumann disease or by the acronym BBS. Its origin is unknown. Except in its severe and fatal form (less than 5% of cases), sarcoidosis heals spontaneously and does not reduce the life expectancy of the affected individual. Sarcoidosis is considered a rare but not exceptional disease. Its exact prevalence is unknown because the disease is not always associated with symptoms, but it is estimated that 1 in 5 to 000 in 1 people are affected in the total population. (20) 000 to 1 new cases are diagnosed annually in France. (6)

Symptoms

The clusters of inflammatory cells called “granulomas” can potentially affect many organs: the skin, eyes, heart, nervous system, kidneys, etc. Sarcoidosis can therefore take very different forms, depending on its extent: it can be completely asymptomatic (which explains why not all cases are diagnosed), and affect one or more organs.

Sarcoidosis often causes fatigue, fever, weight loss, and muscle and joint pain. In addition to these general signs, there are symptoms linked to organ involvement in particular: a persistent cough and shortness of breath linked to lung damage are the most common signs, as are lesions on the skin, ocular and cardiac arrhythmia.

There are two distinct forms:

  • An acute but benign form, corresponding to Löfgren Syndrome. It results in fever, joint pain and the appearance of inflammation in the legs (erythema). Healing is spontaneous and takes from a few weeks to a few months.
  • The disease can progress, but rarely, to chronic form and last for several years (usually 5 to 15 years).

The origins of the disease

The causes of sarcoidosis are still unknown. Current knowledge points to a disruption of the immune system causing its overreaction against a foreign aggressive agent not yet identified. It could be an allergenic environmental substance or a bacteria. This immune abnormality is believed to have a genetic cause.

Risk factors

Sarcoidosis appears preferentially in young adults aged 25 to 45 years, men and women. It is neither contagious nor hereditary. Family forms do develop, however, in very rare cases (again suggesting a genetic factor). Researchers cannot explain why this disease is much more present in black people and in more severe forms. Neither lifestyle nor environment seem to be risk factors.

Prevention and treatment

In the absence of knowledge of the causes of the disease, the symptoms of the disease are treated. Treatments include preventing the onset of pulmonary fibrosis (the main risk for the patient) and treating any complications (damage to the heart, brain, kidneys and eyes) with non-inflammatory anti-inflammatory drugs. steroids (corticosteroids). When corticosteroids are powerless, immunosuppressants are prescribed.

Remember that the disease often heals spontaneously after two or three years, in 30 to 70% of cases. Sarcoidosis is fatal only in its severe form, i.e. 0,5 to 5% of cases (1), most often from pulmonary fibrosis and to a lesser extent from cardiac involvement. People undergoing early and appropriate treatment have a life expectancy equal to that of the rest of the population. However, the psychological consequences of the disease in young people who have it should not be overlooked.

Leave a Reply