Sarcoidosis for Clinicians

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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

  1. Baughman RP, Culver DA, Judson MA. A concise review of pulmonary sarcoidosis. Am J Respir Crit Care Med. 2011;183(5):573-581.
  2. Gerke AK, Hunninghake G. The immunology of sarcoidosis. Clin Chest Med. 2008;29(3):379-390.
  3. Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med. 2007;357(21):2153-2165.
  4. 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.
  5. 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.
  6. 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

Foundation for Sarcoidosis Research

WASOG

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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)

Educational Materials

How to treat Sarcoidosis, Illustrated by a Case History.

The Role of HRCT in the Diagnosis of Interstitial Lung Disease (ILD)

In this video Professor J.A. Verschakelen (CV) presents the current role of high-resolution CT (HRCT) in the diagnostic work-up of diffuse and interstitial lung disease (DILD).

Presentations by WASOG:
  • BAL introduction
  • BAL EAA
  • BAL sarcoidosis
  • Many faces of sarcoidosis: Eye involvement
  • Many faces of sarcoidosis: Radiology

WASOG Task Force Papers

These documents include articles and clinical practice guidelines, policy statements, and research statements, developed by multi disciplary experts on ILD collaborating in WASOG Task Forces.

CME Online: None

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