Sarcoidosis (orbital manifestations)

Changed by Bruno Di Muzio, 23 Apr 2015

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Orbital manifestations of sarcoidosis is common among patients with systemic sarcoidosis and can involve the lacrimal gland, the orbit, soft tissues of the orbit, and the optic nerve. Uveitis is by far the most common manifestation and is typically bilateral 5.

For a general discussion of the underlying condition, please refer to the article sarcoidosis

Epidemiology

Orbital involvement in systemic sarcoidosis is common (up to 80% of patients) and anterior uveitis is the most common manifestation 1, 3-5. Lacrimal glandThe manifestations may coexist with asymptomatic systemic disease and can precede systemic involvement is rare 6by several years

Clinical presentation 

Anterior uveitis has a rapid onset of symptoms characterised by blurred vision, photophobia, and excessive lacrimation 3.

Conjunctival involvement with small, pale, yellow nodules is frequently present 3

Pathology

Histopathological analysis of the biopsied lacrimal glands reveals diffuse chronic granulomatous inflammation with non-caseating granulomas.

Radiographic features

There is no real indication for imaging studies in patients with known sarcoidosis 6

CT/MRI
  • lacrimal gland
    • contrast-enhanced CT or MR imaging typically demonstrates enlarged, asymmetric, enhancing lacrimal glands 5 
    • the glands typically present hypointensity both in T1 and T2 6

Treatment and prognosis 

The manifestations can be self-limited or chronic, with episodic recrudescence and remissions 3. Acute uveitis usually resolves spontaneously or responds to local corticosteroid therapy 5.

Differential diagnosis

  • lacrimal gland
    • lymphoma of lacrimal gland: both are radiologically indistinguishable 
  • -<p>Orbital manifestations of sarcoidosis is common among patients with systemic sarcoidosis and can involve the lacrimal gland, the orbit, soft tissues of the orbit, and the optic nerve. Uveitis is by far the most common manifestation and is typically bilateral <sup>5</sup>.</p><p>For a general discussion of the underlying condition, please refer to the article <a href="/articles/sarcoidosis-1">sarcoidosis</a>. </p><h4>Epidemiology</h4><p>Orbital involvement in systemic sarcoidosis is common (up to 80% of patients) and anterior uveitis is the most common manifestation <sup>1, 3-5</sup>. Lacrimal gland involvement is rare <sup>6</sup>. </p><h4>Clinical presentation </h4><p>Anterior uveitis has a rapid onset of symptoms characterised by blurred vision, photophobia, and excessive lacrimation <sup>3</sup>.</p><p>Conjunctival involvement with small, pale, yellow nodules is frequently present <sup>3</sup>. </p><h4>Radiographic features</h4><p>There is no real indication for imaging studies in patients with known sarcoidosis <sup>6</sup>. </p><h5><span style="line-height:1.6">CT/MRI</span></h5><ul><li>lacrimal gland<ul>
  • +<p>Orbital manifestations of sarcoidosis is common among patients with systemic sarcoidosis and can involve the lacrimal gland, the orbit, soft tissues of the orbit, and the optic nerve. Uveitis is by far the most common manifestation and is typically bilateral <sup>5</sup>. </p><p>For a general discussion of the underlying condition, please refer to the article <a href="/articles/sarcoidosis-1">sarcoidosis</a>. </p><h4>Epidemiology</h4><p>Orbital involvement in systemic sarcoidosis is common (up to 80% of patients) and anterior uveitis is the most common manifestation <sup>1, 3-5</sup>. The manifestations may coexist with asymptomatic systemic disease and can precede systemic involvement by several years.  </p><h4>Clinical presentation </h4><p>Anterior uveitis has a rapid onset of symptoms characterised by blurred vision, photophobia, and excessive lacrimation <sup>3</sup>.  </p><p>Conjunctival involvement with small, pale, yellow nodules is frequently present <sup>3</sup>. </p><h4>Pathology</h4><p>Histopathological analysis of the biopsied lacrimal glands reveals diffuse chronic granulomatous inflammation with non-caseating granulomas.</p><h4>Radiographic features</h4><p>There is no real indication for imaging studies in patients with known sarcoidosis <sup>6</sup>. </p><h5>CT/MRI</h5><ul><li>lacrimal gland<ul>
  • -</li></ul><h4>Treatment and prognosis </h4><p>The manifestations can be self-limited or chronic, with episodic recrudescence and remissions <sup>3</sup>. Acute uveitis usually resolves spontaneously or responds to local corticosteroid therapy <sup>5</sup>.</p><h4>Differential diagnosis</h4><ul><li>
  • -<span style="line-height:1.6">lacrimal gland</span><ul><li><span style="line-height:1.6">lymphoma of lacrimal gland: both are radiologically indistinguishable </span></li></ul>
  • +</li></ul><h4>Treatment and prognosis </h4><p>The manifestations can be self-limited or chronic, with episodic recrudescence and remissions <sup>3</sup>. Acute uveitis usually resolves spontaneously or responds to local corticosteroid therapy <sup>5</sup>.</p><h4>Differential diagnosis</h4><ul><li>lacrimal gland<ul><li>lymphoma of lacrimal gland: both are radiologically indistinguishable </li></ul>

References changed:

  • 6. Vogl TJ. Differential Diagnosis in Head and Neck Imaging. Thieme. (1999) ISBN:0865778116. <a href="http://books.google.com/books?vid=ISBN0865778116">Read it at Google Books</a> - <a href="http://www.amazon.com/gp/product/0865778116">Find it at Amazon</a><span class="auto"></span>
  • 5. Koyama T, Ueda H, Togashi K et-al. Radiologic manifestations of sarcoidosis in various organs. Radiographics. 2004;24 (1): 87-104. <a href="http://radiographics.rsna.org/content/24/1/87.full">Radiographics (full text)</a> - <a href="http://dx.doi.org/10.1148/rg.241035076">doi:10.1148/rg.241035076</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/14730039">Pubmed citation</a><span class="ref_v3"></span>
  • 3. Mafee MF, Dorodi S, Pai E. Sarcoidosis of the eye, orbit, and central nervous system. Role of MR imaging. Radiol. Clin. North Am. 1999;37 (1): 73-87, x. <a href="http://www.ncbi.nlm.nih.gov/pubmed/10026730">Pubmed citation</a><span class="auto"></span>
  • 4. Simon EM, Zoarski GH, Rothman MI et-al. Systemic sarcoidosis with bilateral orbital involvement: MR findings. AJNR Am J Neuroradiol. 1998;19 (2): 336-7. <a href="http://www.ncbi.nlm.nih.gov/pubmed/9504490">Pubmed citation</a><span class="auto"></span>
  • 1. Prabhakaran VC, Saeed P, Esmaeli B et-al. Orbital and adnexal sarcoidosis. Arch. Ophthalmol. 2007;125 (12): 1657-62. <a href="http://dx.doi.org/10.1001/archopht.125.12.1657">doi:10.1001/archopht.125.12.1657</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/18071118">Pubmed citation</a><span class="auto"></span>
  • 2. Shaikh ZA, Bakshi R, Greenberg SJ et-al. Orbital involvement as the initial manifestation of sarcoidosis: magnetic resonance imaging findings. J Neuroimaging. 2000;10 (3): 180-3. <a href="http://www.ncbi.nlm.nih.gov/pubmed/10918747">Pubmed citation</a><span class="auto"></span>

Systems changed:

  • Head & Neck

Tags changed:

  • cases

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