Sarcoidosis for Clinicians
Sarcoidosis
Introduction
Sarcoidosis is a systemic disease that involves many immune pathways. It is believed that granuloma formations result from an exaggerated immune response to unknown antigens.1-3
- Granulomas are typically composed of macrophages, epithelioid cells, multinucleated giant cells, T cells, mast cells, and fibroblasts1-3
- Various cytokines, such as IL-2, IFN-gamma, and TNF-alpha, are released throughout the process, leading to sarcoid granuloma formation, inflammation, and progression1-3
- B cells may be directly involved in local inflammatory processes in granulomatous tissue in patients with sarcoidosis4
- Serum BAFF levels were significantly elevated in sarcoidosis patients when compared with healthy controls5,6
Hypothesized Immunopathagens of Sarcoidosis3
Sarcoidal granulomas may persist, resolve, or lead to fibrosis3
APCs=antigen-presenting cells. BAFF=B-cell activating factor. GM-CSF=granulocyte-macrophage colony-stimulating factor. IFN=interferon. IL=interleukin. TNF=tumor necrosis factor.
References
- Baughman RP, Culver DA, Judson MA. A concise review of pulmonary sarcoidosis. Am J Respir Crit Care Med. 2011;183(5):573-581.
- Gerke AK, Hunninghake G. The immunology of sarcoidosis. Clin Chest Med. 2008;29(3):379-390.
- Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med. 2007;357(21):2153-2165.
- Kamphius LS, van Zelm MC, Lam KH, et al. Perigranuloma localization and abnormal maturation of B cells: emerging key players in sarcoidosis? Am J Respir Crit Care Med. 2013;187(4):406-416.
- Saussine A, Tazi A, Feuillet S, et al. Active chronic sarcoidosis is characterized by increased transitional blood B cells, increased IL-10-producing regulatory B cells and high BAFF levels. PLoS One. 2012;7(8):e43588.
- Ueda-Hayakawa I, Tanimura H, Osawa M, et al. Elevated serum BAFF levels in patients with sarcoidosis: association with disease activity. Rheumatology (Oxford). 2013;52(9):1658-1666
Decision to Treat
Treatment Algorithm:
Here we present two treatment algorithms to help with therapy selection.
The FSR suggests the following:
This is fully explained in FSR treatment Protocol (see below)
CME Online: None

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