Sarcoidosis is a rare inflammatory disease characterized by the formation of clumps of inflammatory immune cells called granulomas. These build up in different tissues and organs in the body affecting their ability to function properly. They can cause permanent damage if untreated.

Although there is currently no cure for sarcoidosis, there are several therapies that can help manage the symptoms of the condition.

Corticosteroids are commonly the first medication prescribed for sarcoidosis patients. However, some patients do not respond to these or may experience severe side effects that prevent them from taking these. In such cases, other therapies like immune-modulating therapies may help as a second-line therapy.

What are immune-modulating therapies?

Immune-modulating therapies are medications that alter the way that the immune system works.

Because sarcoidosis is associated with an overreaction of the immune system, suppressing part or all of the immune response can help manage symptoms and prevent permanent damage.

Types of immune-modulating therapies

There are different types of immune-modulating therapies that act in a variety of different ways. Non-specific therapies act by suppressing the immune system as a whole and are very effective but tend to have more side effects. Targeted therapies inhibit specific cells or proteins in the immune system and usually cause fewer side effects.

Immune-modulating therapies may be prescribed off-label to treat sarcoidosis. Some commonly used examples are discussed here.

CellCept (mycophenolate mofetil) acts by preventing the growth of immune cells such as T-cells and B-cells, which are involved in the formation of granulomas. CellCept may be particularly effective in treating neurosarcoidosis, alone or in combination with corticosteroids. The therapy may also be beneficial in ocular sarcoidosis.

Anti-malarial agents such as Plaquenil (chloroquine) and Aralen (hydroxychloroquine) may also be effective in treating sarcoidosis. These are thought to act by blocking the release of cytokines or immune proteins that can trigger damage responses such as inflammation. They may also inhibit antigen presentation, a process which activates T-cells. Anti-malarials may be effective in treating cutaneous sarcoidosis, sarcoidosis-related arthritis, hypercalcemia (related to renal sarcoidosis), and neurosarcoidosis. Plaquenil may also be beneficial in treating pulmonary sarcoidosis.

Cyclosporine inhibits the production of a chemical-signaling molecule called interleukin-2 (IL-2), which is involved in T-cell signaling. IL-2 inhibition reduces the levels and activity of T-cells and therefore suppresses the immune response. Cyclosporine may be used to treat ocular sarcoidosis. It may also be used to reduce the dose of corticosteroids needed in neurosarcoidosis.

Other types of immune-modulating therapies include cytotoxic and cytostatic medications that are non-specific therapies and act to suppress the immune system as a whole, or TNF-alpha inhibitors targeted to a specific component of the immune system. Stelara (ustekinumab), which inhibits the immune proteins IL-12 and IL-23, may be prescribed, but its benefit in sarcoidosis is uncertain. Rituxan (rituximab), which can reduce levels of B-cells, may be effective in treating ocular sarcoidosis.

Other information

Immune-modulating therapies can increase the risk of infections because the immune system is less able to respond to actual threats.

The range of side effects can vary based on the type of medication, but common side effects include diarrhea and nausea. Anti-malarial medications may cause retinopathy, or damage to the retina, and lead to vision loss.

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