Abstract
Most cases of sarcoidosis are mild and self-limited, with a spontaneous cure. However,
in some patients, this disease may also be life-threatening, particularly when severe
manifestations induce vital organ dysfunction. Sarcoidosis may also severely impair
the quality of life through diverse, persistent disabling symptoms. To date, there
is no curative treatment for sarcoidosis, but only anti-inflammatory drugs limiting
the pathologic impact of sarcoidosis in reducing enhanced immunity reactions, granulomatous
formation, and their consequences. Current anti-inflammatory treatments for sarcoidosis
include corticosteroids as the first-line treatment; disease-modifying antisarcoid
drugs, mainly immunosuppressive and immunomodulatory drugs, as second-line treatment;
and finally tumor necrosis factor (TNF) inhibitors, as third-line treatment. Corticosteroids
are most effective; they give rapid results, sometimes with serious, incremental adverse
effects. A second-line treatment, mainly low-dose methotrexate and azathioprine, is
indicated in case of corticosteroid resistance, intolerance, or contraindication or
more often as a corticosteroid-sparing agent when a prolonged treatment of more than
10 mg/d equivalent prednisone is expected. TNF inhibitors are considered in severe
refractory sarcoidosis. Infliximab has been proven effective. Usually, treatment for
sarcoidosis lasts up to 1 year or longer. The usual drug regimen is made of an induction
and then a maintenance protocol before a step-by-step decrease and eventual withdrawal.
Contraindications may exist. Each therapeutic decision must follow a rigorous diagnostic
evaluation to determine the disease impact, its outcome (progression or not), and
its response to treatment in the long run. Pharmacogenetics is still in its infancy,
but could help develop a more personalized therapy. Non anti-inflammatory treatments,
such as implantable cardiac devices, are also useful, particularly for some organs.
In the end, persistent disabling symptoms are very frequent and call for an accurate
diagnosis, which may be difficult to treat.
Keywords
sarcoidosis - treatment - corticosteroids - immunosuppressive drugs - TNF inhibitors
