Difference between revisions of "Sarcoid Uveitis"

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(Created page with 'Article summary goes here. = Disease Entity = Sarcoid Uveitis == Disease == Sarcoidosis is a systemic inflammatory disease characterized by the formation of noncaseating gr…')
 
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== Physical examination  ==
 
== Physical examination  ==
  
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Granulomatous anterior uveitis is the most common ocular manifestation of sarcoidosis, with less than 1/3 presenting with posterior uveitis without anterior involvement. 
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In 2009, an international group of uveitis specialists met for the International Workshop On Ocular Sarcoidosis (IWOS).  They determined the following criteria for the diagnosis of ocular sarcoid:
  
== Signs  ==
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Seven Signs of Intraocular Sarcoidosis
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1. mutton-fat keratic precipitates (KPs)/small granulomatous KPs and/or iris nodules (Koeppe/Busacca)
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2. trabecular meshwork (TM) nodules and/or tent-shaped peripheral anterior synechiae (PAS)
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3. vitreous opacities displaying snowballs/strings of pearls
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4. multiple chorioretinal peripheral lesions (active and/or atrophic)
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5. nodular and/or segmental peri-phlebitis (+/- candlewax drippings) and/or retinal macroaneurism in an inflamed eye
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6. optic disc nodule(s)/granuloma(s) and/or solitary choroidal nodule
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7. bilaterality
  
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== Symptoms  ==
 
== Symptoms  ==
  
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Ocular sarcoid can manifest itself with blurred vision, photophobia, floaters, redness, and pain from uveitis. Cranial nerve palsies causing diplopia and decreased vision from optic nerve infiltration have also been reported.  Lacrimal gland involvement may cause proptosis and diplopia secondary to mass affect. 
  
== Clinical diagnosis  ==
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== Diagnostic Criteria ==
  
Add text here
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The IWOS refers to definitive ocular sarcoidosis as biopsy-supported diagnosis with a compatible uveitis.  Presumed ocular sarcoidosis was defined as a chest x-ray showing bilateral hilar lymphadenopathy associated with a compatible uveitis.  If a biopsy was not done and the chest x-ray did not show BHL but there were 3 of the above intraocular signs and 2 positive laboratory tests, the condition was labeled as probable ocular sarcoidosis.  Finally, if lung biopsy was done and the result was negative but at least 4 of the above signs and 2 positive laboratory investigations were present, the condition was labeled as possible ocular sarcoidosis.
  
 
== Diagnostic procedures  ==
 
== Diagnostic procedures  ==
  
Add text here
+
When the diagnosis of  sarcoid uveitis is suspected, serum angiotensin converting enzyme (ACE), chest x-ray and gallium scan are noninvasive diagnostic tools. 
 +
 
 +
According to the IWOS, individuals with clinical signs of ocular sarcoidosis should receive the following diagnostic tests:
 +
 
 +
1. negative tuberculin skin test in a BCG-vaccinated patient or in a patient having had a positive tuberculin skin test previously
 +
2. elevated serum ACE levels and/or elevated serum lysozyme
 +
3. chest x-ray revealing bilateral hilar lymphadenopathy (BHL)
 +
4. abnormal liver enzyme tests
 +
5. chest CT scan in patients with a negative chest x-ray result
 +
 
 +
In individuals with suspected sarcoid uveitis, chest x-ray evidence of hilar adenopathy is seen in up to 80%.  If the chest x-ray is negative but sarcoidosis is strongly suspected, a high-resolution chest CT should be considered.
 +
 
 +
Definitive diagnosis of sarcoidosis requires a biopsy.  Typically, the most accessible site with the lowest associated morbidity is chosen, such as a palpable lymph node.  Conjuntival biopsies can also be performed.  Bilateral blind conjunctival biopsies are more likely to be positive in biopsy-proven sarcoidosis from other sites.  Conjunctival biopsies are particularly useful in the presence of conjunctival follicles, ocular abnormalities consistent with sarcoidosis, and in patients with pulmonary infiltrates on chest x-ray.  If lacrimal gland involovement is noted on physical exam or gallium scan, a lacrimal gland biopsy can also be performed.  In cases of active pulmonary disaese, fiberoptic bronchoscopy can be biopsy positive in 63-80%, depending on the stage of disease.   
 +
 
  
 
== Laboratory test  ==
 
== Laboratory test  ==
  
Add text here
+
Serum level of ACE (Angiotensin Converting Enzyme) is often elevated in sarcoidosis and can be used to monitor disease activity.  Higher serum ACE levels indicate more active disease.  Elevated serum ACE is 73% sensitive and 83% specific for sarcoid when used alone.  The specificity increases to almost 100% when used in combination with whole-body gallium (67GA) scanning.  This has also been shown in studies of ocular sarcoidosis with normal or equivocal chest radiographs.  Serum lysozyme also plays a role in the diagnosis of ocular sarcoid.  Serum lysozyme greater than 8mg/l was found to be 60% sensitive and 76% specific for sarcoid uveitis.
  
 
== Differential diagnosis  ==
 
== Differential diagnosis  ==
  
Add text here
+
The differential includes autoimmune and infectious causes of granulomatous uveitis, including syphilis, Lyme disease, tuberculosis, Behcet's disease, Vogt-Koyanagi-Harada disease, and sympathetic ophthalmoplegia.
  
 
= Management  =
 
= Management  =
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{{Article
 
{{Article
|Authors=Parvathy.Pillai,  
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|Authors=Parvathy.Pillai,
 
|Category=Uveitis
 
|Category=Uveitis
 
}}
 
}}

Revision as of 12:10, July 5, 2011

Article summary goes here.

Disease Entity

Sarcoid Uveitis

Disease

Sarcoidosis is a systemic inflammatory disease characterized by the formation of noncaseating granulomas in affected organs, most commonly the lungs, lymph nodes, skin, and eyes. The disease was first described in 1878 by noted surgeon Sir Jonathan Hutchinson as a dermatologic disorder. It was not until 1909 that a Danish ophthalmologist, Heerfordt Christian Frederik1, described uveitis as part of the disease process. His eponymous “Heerfordt Sydrome” consisted of uveitis, parotitis and fever (uveoparotid fever) +/- facial palsy.


Etiology

While the etiology of sarcoidosis is unknown, several hypothesis regarding genetic and environmental factors have been studied. Some hypothesize that it is a clinical syndrome consisting of different diseases with different underlying etiologies. Associations between sarcoid and HLA-DRB1 and M. tuberculosis DNA are currently being evaluated.

Risk Factors

In the United States, the incidence of systemic sarcoidosis ranges from 5-40 per 100,000 population. This prevalence is 10 times greater for African Americans when compared to white Americans. African Americans are also more likely to have an acute presentation of the disease and a more severe clinical course whites.

A large retrospective chart review from the University of Illinois uveitis service indicated that in biopsy-proved sarcoidosis, African-American patients were more likely to be diagnosed as having uveitis than whites. African-Americans younger than 50 years were more likely to present with uveitis.

Several studies have noted a slight female preponderance, with most symptomatic patients being between 20-40 years old.


Pathophysiology

The exact etiology of sarcoidosis is uinknown, making it largely a diagnosis of exclusion. It is characterized by the appearance of noncaseating granulomas in affected tissue, most frequently seen in the lungs as bilateral hilar lymphadenopathy or pulmonary infiltration. Granulomas can also be seen on the skin, in the eyes, liver, spleen, salivary glands, heart, bones and nervous system. Ocular involvement occurs in approximately 25% of patients with sarcoidosis. The uveal tract is the most common site of ocular involvement by sarcoidosis.


Diagnosis

Sarcoid is a systemic inflammatory disease and can manifest itself in multiple ways. Therefore, clinical suspicion is often the driving force behind correct diagnosis. A thourough review of systems if necessary in all patients with recurrent uveitis. An individual with pumlonary or dermatologic complaints consistent with sarcoid should be further evaluated. Uveitis can also precede pulmonary symptoms by several years.

History

Clinical presentation of sarcoidosis depends on the severity and organ involved. A history of pulmonary disease in an individual with granulomatous uveitis should raise the suspicion of sarcoidosis.

Physical examination

Granulomatous anterior uveitis is the most common ocular manifestation of sarcoidosis, with less than 1/3 presenting with posterior uveitis without anterior involvement.

In 2009, an international group of uveitis specialists met for the International Workshop On Ocular Sarcoidosis (IWOS). They determined the following criteria for the diagnosis of ocular sarcoid:

Seven Signs of Intraocular Sarcoidosis 1. mutton-fat keratic precipitates (KPs)/small granulomatous KPs and/or iris nodules (Koeppe/Busacca) 2. trabecular meshwork (TM) nodules and/or tent-shaped peripheral anterior synechiae (PAS) 3. vitreous opacities displaying snowballs/strings of pearls 4. multiple chorioretinal peripheral lesions (active and/or atrophic) 5. nodular and/or segmental peri-phlebitis (+/- candlewax drippings) and/or retinal macroaneurism in an inflamed eye 6. optic disc nodule(s)/granuloma(s) and/or solitary choroidal nodule 7. bilaterality


Symptoms

Ocular sarcoid can manifest itself with blurred vision, photophobia, floaters, redness, and pain from uveitis. Cranial nerve palsies causing diplopia and decreased vision from optic nerve infiltration have also been reported. Lacrimal gland involvement may cause proptosis and diplopia secondary to mass affect.

Diagnostic Criteria

The IWOS refers to definitive ocular sarcoidosis as biopsy-supported diagnosis with a compatible uveitis. Presumed ocular sarcoidosis was defined as a chest x-ray showing bilateral hilar lymphadenopathy associated with a compatible uveitis. If a biopsy was not done and the chest x-ray did not show BHL but there were 3 of the above intraocular signs and 2 positive laboratory tests, the condition was labeled as probable ocular sarcoidosis. Finally, if lung biopsy was done and the result was negative but at least 4 of the above signs and 2 positive laboratory investigations were present, the condition was labeled as possible ocular sarcoidosis.

Diagnostic procedures

When the diagnosis of sarcoid uveitis is suspected, serum angiotensin converting enzyme (ACE), chest x-ray and gallium scan are noninvasive diagnostic tools.

According to the IWOS, individuals with clinical signs of ocular sarcoidosis should receive the following diagnostic tests:

1. negative tuberculin skin test in a BCG-vaccinated patient or in a patient having had a positive tuberculin skin test previously 2. elevated serum ACE levels and/or elevated serum lysozyme 3. chest x-ray revealing bilateral hilar lymphadenopathy (BHL) 4. abnormal liver enzyme tests 5. chest CT scan in patients with a negative chest x-ray result

In individuals with suspected sarcoid uveitis, chest x-ray evidence of hilar adenopathy is seen in up to 80%. If the chest x-ray is negative but sarcoidosis is strongly suspected, a high-resolution chest CT should be considered.

Definitive diagnosis of sarcoidosis requires a biopsy. Typically, the most accessible site with the lowest associated morbidity is chosen, such as a palpable lymph node. Conjuntival biopsies can also be performed. Bilateral blind conjunctival biopsies are more likely to be positive in biopsy-proven sarcoidosis from other sites. Conjunctival biopsies are particularly useful in the presence of conjunctival follicles, ocular abnormalities consistent with sarcoidosis, and in patients with pulmonary infiltrates on chest x-ray. If lacrimal gland involovement is noted on physical exam or gallium scan, a lacrimal gland biopsy can also be performed. In cases of active pulmonary disaese, fiberoptic bronchoscopy can be biopsy positive in 63-80%, depending on the stage of disease.


Laboratory test

Serum level of ACE (Angiotensin Converting Enzyme) is often elevated in sarcoidosis and can be used to monitor disease activity. Higher serum ACE levels indicate more active disease. Elevated serum ACE is 73% sensitive and 83% specific for sarcoid when used alone. The specificity increases to almost 100% when used in combination with whole-body gallium (67GA) scanning. This has also been shown in studies of ocular sarcoidosis with normal or equivocal chest radiographs. Serum lysozyme also plays a role in the diagnosis of ocular sarcoid. Serum lysozyme greater than 8mg/l was found to be 60% sensitive and 76% specific for sarcoid uveitis.

Differential diagnosis

The differential includes autoimmune and infectious causes of granulomatous uveitis, including syphilis, Lyme disease, tuberculosis, Behcet's disease, Vogt-Koyanagi-Harada disease, and sympathetic ophthalmoplegia.

Management

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General treatment

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Medical therapy

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Medical follow up

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Surgery

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Surgical follow up

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Complications

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Prognosis

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Additional Resources

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References

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