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CHORIORETINAL INFLAMMATORY DISEASES - Noninfectious Causes

Results 1-8 of 15
   
Sarcoidosis  
 

Sarcoidosis is an inflammatory multisystem granulomatous disease of unknown etiology.

Sarcoidosis frequently manifests initially as an ocular problem. It is...

Sarcoidosis is an inflammatory multisystem granulomatous disease of unknown etiology.

Sarcoidosis frequently manifests initially as an ocular problem. It is a granulomatous panuveitis with retinal vasculitis, vascular sheathing, periphlebitis, vitreous snowballs or string of pearls, yellow-white retinal/choroidal granulomas, anterior chamber cells and flare, mutton fat keratic precipitates, Koeppe/Busacca iris nodules, and macular edema.

Anterior uveitis is the most common ocular manifestation of sarcoidosis, affecting 25% of patients with the disorder.

Systemic findings include hilar adenopathy, pulmonary fibrosis, pulmonary parenchymal involvement, erythema nodosum, subcutaneous nodules, and lymphadenopathy.

Serpiginous Choroiditis  
 

Serpiginous choroiditis is a bilateral inflammatory disease of unknown cause that represents a pathologic affliction of the retinal pigment epithelium,...

Serpiginous choroiditis is a bilateral inflammatory disease of unknown cause that represents a pathologic affliction of the retinal pigment epithelium, choriocapillaris, and inner choroid. There is a disruption of the overlying neurosensory retina, marked by periods of activity with intervening intervals of quiescence.

It typically occurs in young to middle-aged white people.

Acute lesions have a gray-white or yellow appearance that begin around the optic nerve or the posterior pole and advance centrifugally by recurrences, to the midperiphery, in an irregular serpentine fashion, leading to scarring and choroidal atrophy. They may result in choroidal neovascular membrane in as many 25% of patients.

Given the unpredictability of the disease progression, the fovea is never truly safe from eventual involvement.

Diferential Diagnosis

Acute posterior multifocal placoid pigment epitheliopathy

Infectious disorders

Presumed ocular histoplasmosis syndrome

  • Syphilis
  • Toxoplasmosis
  • Tuberculosis

Inflammatory diseases

  • Multifocal choroiditis
  • Posterior scleritis
  • Sarcoidosis

Degenerative/dystrophic disorders

  • Best disease
  • Central areolar choroidal dystrophy
  • Fundus flavimaculatus
  • Sorsby´s pseudo-inflammatory dystrophy

Infiltrative diseases of the choroid

  • Choroidal osteoma
  • Metastatic tumor
  • Non-Hodgkin’s lymphoma (reticulum cell sarcoma)

Choroidal neovascularization

  • Age-related macular degeneration
  • Angioid streaks Drusen of optic nerve head
  • Idiopathic choroidal neovascularization
  • Pathologic myopia
  • Peripapillary subretinal neovascularization
Birdshot Retinochoroidopathy  
 

Birdshot retinochoroidopathy is a clinically distinct pattern of posterior segment inflammation of presumed autoimmune etiology.

The characteristic spots of depigmentation,...

Birdshot retinochoroidopathy is a clinically distinct pattern of posterior segment inflammation of presumed autoimmune etiology.

The characteristic spots of depigmentation, usually cream-colored at the level of the retinal pigment epithelium or deeper, are the most distinctive sign of birdshot retinochoroidopathy. The lesions radiate outwards from the disc in a linear pattern that seems to follow the choroidal vessels.

The differential diagnosis includes other entities that produce a chronic mild to moderate bilateral panuveitis with choroidal infiltrates.

References

Chang B., Lumbroso L, Rabb MF, Yannuzzi LA. Birdshot chorioretinopathy. In: Yannuzzi LA, Flower RW, Slakter JS, eds. Indocyanin Green Angiography, 1st ed.St.Louis: Mosby, 1997:231-238.

Acute Retinal Pigment Epithelitis  
 

Acute retinal pigment epithelitis (Krill’s Disease) is a rare, idiopathic and self-limiting benign inflammatory disorder of the retinal pigment epithelium...

Acute retinal pigment epithelitis (Krill’s Disease) is a rare, idiopathic and self-limiting benign inflammatory disorder of the retinal pigment epithelium in the macular area that affects healthy young adults.

In 75% of cases the disease is unilateral, without any signs or symptoms of intraocular inflammation. It has a good prognosis without treatment.

This disease is characterized by small lesions at the level of the retinal pigment epithelium in the macular area of one or both eyes. The pigmented spots may be surrounded by a hypo-pigmented halo that transmits fluorescence without leakage in fluorescein angiography.

The etiology of acute retinal pigment epithelitis remains unknown, but some authors suggest it may have a viral etiology, while others have suggested a correlation between ARPE and central serous chorioretinopathy.

Uveitis  
 

Uveitis is an intraocular inflammation and may be classified anatomically into anterior, intermediate, posterior and panuveitic forms.

The symptoms include...

Uveitis is an intraocular inflammation and may be classified anatomically into anterior, intermediate, posterior and panuveitic forms.

The symptoms include eye pain, redness of the eye, blurred vision, floating spots, and photophobia.

Uveitis is associated with several disorders: ankylosing spondylitis, juvenile rheumatoid arthritis, multiple sclerosis, sarcoidosis, systemic lupus erythematosus, Vogt-Koyanagi-Harada syndrome, HLA-B27, arthritis and other autoimmune diseases. It also appears as an immune response to infections inside the eye.

The presentation in the eye, in some cases, is characteristic of some syndromes such as serpiginous choroiditis, multifocal choroiditis, punctate inner choroidopathy, birdshot retinochoroidopathy, acute posterior multifocal placoid pigment epitheliopathy, and multiple evanescent white dot syndrome. Several masquerade syndromes that clinically present anterior and posterior uveitis includes retinal detachment, intraocular foreign body, leukemia, malignant melanoma, and lymphoma.

Early diagnosis and treatment of uveitis is important to prevent permanent loss of vision. Uveitis is typically treated with corticosteroids and/or immunomodulators.

Eales Disease  
 

Eales disease is characterized by periphlebitis, predominantly in the periphery.

This idiopathic peripheral obliterative vasculopathy leads to areas of capillary...

Eales disease is characterized by periphlebitis, predominantly in the periphery.

This idiopathic peripheral obliterative vasculopathy leads to areas of capillary non-perfusion which may lead to the development of neovascularization in the periphery.

Recurrent vitreous hemorrhage is common.

Eales disease affects mostly healthy young males, usually between 20 and 30 years of age.

Multifocal Choroiditis  
 

Multifocal choroiditis is an inflammatory disease that causes visual loss in 70 % of patients. It usually occurs in 30...

Multifocal choroiditis is an inflammatory disease that causes visual loss in 70 % of patients. It usually occurs in 30 to 60-year-old healthy myopic females. Most cases are bilateral or become bilateral over time.

Fundoscopic findings include small, round, discrete and gray-yellowish spots located at the level of the retinal pigment epithelium and choriocapillaris, most often in the mid-periphery. As these lesions age and become inactive, they become atrophic and “punched-out”.

Patients may have variable amounts of anterior segment inflammation and vitritis.

Peripapillary and macular choroidal neovascularization may occur in 25-32 % of the patients.

Differential Diagnosis

  • Birdshot retinochoroidopathy and myopic degeneration
  • Ocular histoplasmosis syndrome
  • Sarcoidosis
  • Serpiginous choroiditis
  • Subretinal fibrosis and uveitis syndrome
  • Sympathetic uveitis
  • Vogt-Koyanagi-Harada disease
Multiple Evanescent White Dot Syndrome  
 

Multiple evanescent white dot syndrome is an acute, multifocal, usually unilateral retinopathy that is predominantly seen in 20 to30-year-old healthy...

Multiple evanescent white dot syndrome is an acute, multifocal, usually unilateral retinopathy that is predominantly seen in 20 to30-year-old healthy females.

Patients present blurred central vision, bothersome photopsias, paracentral scotomas or enlargement of the blind spot, and headaches.

This syndrome is characterized by multiple small gray-white spots at the level of the retinal pigment epithelium in the posterior pole, sparing the fovea.

The white dots disappear spontaneously within 2 months.

Differential Diagnosis

  • Acute macular neuroretinopathy
  • Acute posterior multifocal placoid pigment epitheliopathy
  • Acute retinal pigment epithelitis
  • Acute zonal occult outer retinopathy
  • Birdshot retinochoroidopathy
  • Diffuse unilateral subacute neuroretinitis
  • Toxoplasmosis