Acute posterior multifocal placoid pigment epitheliopathy

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Acute posterior multifocal placoid pigment epitheliopathy
PMID20029144 08 acute posterior multifocal placoid pigment epitheliopathy.png
Placoid lesions seen on fundal examination in APMPPE
Specialty Ophthalmology

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an acquired inflammatory uveitis that belongs to the heterogenous group of white dot syndromes in which light-coloured (yellowish-white) lesions begin to form in the macular area of the retina. Early in the course of the disease, the lesions cause acute and marked vision loss (if it interferes with the optic nerve) that ranges from mild to severe but is usually transient in nature. APMPPE is classified as an inflammatory disorder that is usually bilateral and acute in onset but self-limiting. The lesions leave behind some pigmentation, but visual acuity eventually improves even without any treatment (providing scarring doesn't interfere with the optic nerve).

Contents

It occurs equally between men and women with a male to female ratio of 1.2:1. Mean onset age is 27, but has been seen in people aged 16 to 40. [1] It is known to occur after or concurrently with a systemic infection (but not always), showing that it is related generally to an altered immune system. Recurrent episodes can happen, but are extremely rare. [2] [3]

Signs and symptoms

The onset of ocular symptoms are usually preceded by episode of viral or flu-like symptoms such as fever, cough or sore throat (however this is not always the case). Patients can typically present erythema nodosum, livedo reticularis, bilateral uveitis, and sudden onset of marked visual loss associated with the appearance of multiple lesions in the retina. These lesions may be colored from grey-white to cream-shaded yellow. Other symptoms include scotomata and photopsia. In weeks to a month times the lesions begin to clear and disappear (with prednisone) leaving behind areas of retinal pigment epithelial atrophy and diffuse fine pigmentation (scarring). Rarely choroidal neovascularization occur as a late onset complication. [4]

Cause

The cause of the inflammation remains unknown. It is hypothesized that it may occur as an autoimmune response to a mild infection, or it may be viral in nature, as evidenced by the preceding flu-like illness that generally accompanies it. [2] [4] It is associated with an increased incidence of both HLA-B7 and HLA-DR2.

The underlying etiology of APMPPE continues to cause debate. [5] [6] The term 'Pigment Epitheliopathy' was chosen by Gass [7] to reflect what he thought was the tissue most significantly affected. Van Buskirk et al., [8] and Deutman et al. [9] proposed choriocapillaris ischemia as the more likely primary etiology. Indocyanine green angiography (ICGA), [10] and OCT angiography (OCTA) [11] [12] [13] studies have provided support for choriocapillaris involvement.

However, a novel hypothesis was proposed implicating a direct neurotropic infection as a possible underlying cause given the dynamic changes observed along the neuronal pathway of the retina [14]

Diagnosis

Diagnosis is usually made on clinical appearance alone on fundoscopy and/or retinal imaging. Supplementary tests such as Optical coherence tomography(OCT) and fundus fluorescein angiography/Indocyanine angiography together with OCT-Angiography are commonly performed to help aid diagnosis and monitoring. Fundus photography is useful to document the appearance of APMPPE lesions. [1]

Management

The original description categorized APMPPE as a self-limiting condition with a good prognosis, but the disease can be recurrent and result in significant visual loss. [1] Owing to the self-limiting nature of the disease, treatment is generally not required. In cases where lesions appear to be interfering with the optic nerve, methyl prednisone is prescribed, but in recurrent disease an antimetabolite agent may be indicated. [1]

Prognosis

Vision improves in almost all cases. In rare cases, a patient may suffer permanent visual loss associated with lesions on their optic nerve.

Rarely, coexisting vasculitis may cause neurological complications. These occurrences can start with mild headaches that steadily worsen in pain and onset, and can include attacks of dysesthesia. This type of deterioration happens usually if the lesions involve the fovea. [2] [15]

See also

Related Research Articles

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<span class="mw-page-title-main">Chorioretinitis</span> Medical condition

Chorioretinitis is an inflammation of the choroid and retina of the eye. It is a form of posterior uveitis. Inflammation of these layers can lead to vision-threatening complications. If only the choroid is inflamed, not the retina, the condition is termed choroiditis. The ophthalmologist's goal in treating these potentially blinding conditions is to eliminate the inflammation and minimize the potential risk of therapy to the patient.

<span class="mw-page-title-main">Choroid</span> Vascular layer of the eye, containing connective tissue, and lying between the retina and the sclera

The choroid, also known as the choroidea or choroid coat, is a part of the uvea, the vascular layer of the eye. It contains connective tissues, and lies between the retina and the sclera. The human choroid is thickest at the far extreme rear of the eye, while in the outlying areas it narrows to 0.1 mm. The choroid provides oxygen and nourishment to the outer layers of the retina. Along with the ciliary body and iris, the choroid forms the uveal tract.

<span class="mw-page-title-main">Uveitis</span> Inflammation of the uvea of the eye

Uveitis is inflammation of the uvea, the pigmented layer of the eye between the inner retina and the outer fibrous layer composed of the sclera and cornea. The uvea consists of the middle layer of pigmented vascular structures of the eye and includes the iris, ciliary body, and choroid. Uveitis is described anatomically, by the part of the eye affected, as anterior, intermediate or posterior, or panuveitic if all parts are involved. Anterior uveitis (iridocyclitis) is the most common, with the incidence of uveitis overall affecting approximately 1:4500, most commonly those between the ages of 20-60. Symptoms include eye pain, eye redness, floaters and blurred vision, and ophthalmic examination may show dilated ciliary blood vessels and the presence of cells in the anterior chamber. Uveitis may arise spontaneously, have a genetic component, or be associated with an autoimmune disease or infection. While the eye is a relatively protected environment, its immune mechanisms may be overcome resulting in inflammation and tissue destruction associated with T-cell activation.

<span class="mw-page-title-main">Central serous chorioretinopathy</span> Eye disease characterized by leakage of fluid under the retina

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<span class="mw-page-title-main">Cone dystrophy</span> Medical condition

A cone dystrophy is an inherited ocular disorder characterized by the loss of cone cells, the photoreceptors responsible for both central and color vision.

<span class="mw-page-title-main">Drusen</span> Accumulations of extracellular material in the retina

Drusen, from the German word for node or geode, are tiny yellow or white accumulations of extracellular material that build up between Bruch's membrane and the retinal pigment epithelium of the eye. The presence of a few small ("hard") drusen is normal with advancing age, and most people over 40 have some hard drusen. However, the presence of larger and more numerous drusen in the macula is a common early sign of age-related macular degeneration (AMD).

<span class="mw-page-title-main">Optic disc drusen</span> Medical condition

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Fundus photography involves photographing the rear of an eye, also known as the fundus. Specialized fundus cameras consisting of an intricate microscope attached to a flash enabled camera are used in fundus photography. The main structures that can be visualized on a fundus photo are the central and peripheral retina, optic disc and macula. Fundus photography can be performed with colored filters, or with specialized dyes including fluorescein and indocyanine green.

White dot syndromes are inflammatory diseases characterized by the presence of white dots on the fundus, the interior surface of the eye. The majority of individuals affected with white dot syndromes are younger than fifty years of age. Some symptoms include blurred vision and visual field loss. There are many theories for the etiology of white dot syndromes including infectious, viral, genetics and autoimmune.

<span class="mw-page-title-main">Bonnet–Dechaume–Blanc syndrome</span> Medical condition

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<span class="mw-page-title-main">Behçet's disease</span> Inflammatory disorder

Behçet's disease (BD) is a type of inflammatory disorder which affects multiple parts of the body. The most common symptoms include painful sores on the mucous membranes of the mouth and other parts of the body, inflammation of parts of the eye, and arthritis. The sores can last from a few days, up to a week or more. Less commonly there may be inflammation of the brain or spinal cord, blood clots, aneurysms, or blindness. Often, the symptoms come and go.

Multiple evanescent white dot syndrome (MEWDS) is an uncommon inflammatory condition of the retina that typically affects otherwise healthy young females in the second to fourth decades of life.

<span class="mw-page-title-main">Serpiginous choroiditis</span> Medical condition

Serpiginous choroiditis, also known as geographic helicoid peripapillary choroidopathy (GHPC), is a rare, chronic, progressive, and recurrent bilateral inflammatory disease involving the retinal pigment epithelium (RPE), the choriocapillaries, and the choroid. It affects adult men and women equally in the second to seventh decades of life.

<span class="mw-page-title-main">Vogt–Koyanagi–Harada disease</span> Medical condition

Vogt–Koyanagi–Harada disease (VKH) is a multisystem disease of presumed autoimmune cause that affects melanin-pigmented tissues. The most significant manifestation is bilateral, diffuse uveitis, which affects the eyes. VKH may variably also involve the inner ear, with effects on hearing, the skin, and the meninges of the central nervous system.

Geographic atrophy (GA), also known as atrophic age-related macular degeneration (AMD) or advanced dry AMD, is an advanced form of age-related macular degeneration that can result in the progressive and irreversible loss of retinal tissue (photoreceptors, retinal pigment epithelium, choriocapillaris) which can lead to a loss of visual function over time. It is estimated that GA affects over 5 million people worldwide and approximately 1 million patients in the US, which is similar to the prevalence of neovascular (wet) AMD, the other advanced form of the disease.

Optical coherence tomography angiography (OCTA) is a non-invasive imaging technique based on optical coherence tomography (OCT) developed to visualize vascular networks in the human retina, choroid, skin and various animal models. OCTA may make use of speckle variance optical coherence tomography.

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Retina→Optic nerve→Optic chiasma →Optic tract→Lateral geniculate body→Optic radiation→Primary visual cortex

<span class="mw-page-title-main">Indocyanine green angiography</span> Diagnostic procedure

Indocyanine green angiography (ICGA) is a diagnostic procedure used to examine choroidal blood flow and associated pathology. Indocyanine green (ICG) is a water soluble cyanine dye which shows fluorescence in near-infrared (790–805 nm) range, with peak spectral absorption of 800-810 nm in blood. The near infrared light used in ICGA penetrates ocular pigments such as melanin and xanthophyll, as well as exudates and thin layers of sub-retinal vessels. Age-related macular degeneration is the third main cause of blindness worldwide, and it is the leading cause of blindness in industrialized countries. Indocyanine green angiography is widely used to study choroidal neovascularization in patients with exudative age-related macular degeneration. In nonexudative AMD, ICGA is used in classification of drusen and associated subretinal deposits.

<span class="mw-page-title-main">Uveitic glaucoma</span> Glaucoma caused by uveitis or its treatments

Uveitic glaucoma is most commonly a progression stage of noninfectious anterior uveitis or iritis.

References

  1. 1 2 3 4 Testi I, Vermeirsch S, Pavesio C (November 2021). "Acute posterior multifocal placoid pigment epitheliopathy (APMPPE)". J Ophthalmic Inflamm Infect. 11 (1): 31. doi: 10.1186/s12348-021-00263-1 . PMC   8443720 . PMID   34524577.
  2. 1 2 3 Comu S, Verstraeten T, Rinkoff JS, Busis NA (May 1996). "Neurological manifestations of acute posterior multifocal placoid pigment epitheliopathy". Stroke. 27 (5): 996–1001. doi:10.1161/01.str.27.5.996. PMID   8623125.
  3. ↑ Jones, Nicholas P (1995). "Acute posterior multifocal placoid pigment epitheliopathy". British Journal of Ophthalmology. BMJ group. 79 (4): 384–389. doi:10.1136/bjo.79.4.384. PMC   505108 . PMID   7742290.
  4. 1 2 De Vries, J.J. (June 2006). "Acute Posterior Multifocal Placoid Pigment Epitheliopathy with Cerebral Vasculitis: A Multisystem Granulomatous Disease". Archives of Ophthalmology. 124 (6): 910–913. doi: 10.1001/archopht.124.6.910 . PMID   16769850 . Retrieved 2009-09-15.
  5. ↑ Zhang AY, Han IC, Goldberg MF (March 2017). "Renaming of Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) to Acute Multifocal Placoid Choroidopathy (AMP-C)". JAMA Ophthalmol. 135 (3): 185. doi:10.1001/jamaophthalmol.2016.5325. PMID   28097335. S2CID   30890363.
  6. ↑ Jampol LM, Goldstein DA, Fawzi AA (March 2017). "Keeping the Name of Acute Posterior Multifocal Placoid Pigment Epitheliopathy". JAMA Ophthalmol. 135 (3): 186. doi:10.1001/jamaophthalmol.2016.5334. PMID   28099970. S2CID   33896225.
  7. ↑ Gass JD (August 1968). "Acute posterior multifocal placoid pigment epitheliopathy". Arch Ophthalmol. 80 (2): 177–85. doi:10.1001/archopht.1968.00980050179005. PMID   5661882.
  8. ↑ Van Buskirk EM, Lessell S, Friedman E (March 1971). "Pigmentary epitheliopathy and erythema nodosum". Arch Ophthalmol. 85 (3): 369–72. doi:10.1001/archopht.1971.00990050371025. PMID   5100807.
  9. ↑ Deutman AF, Oosterhuis JA, Boen-Tan TN, Aan de Kerk AL (December 1972). "Acute posterior multifocal placoid pigment epitheliopathy. Pigment epitheliopathy of choriocapillaritis?". Br J Ophthalmol. 56 (12): 863–74. doi:10.1136/bjo.56.12.863. PMC   1214795 . PMID   4651978.
  10. ↑ Dhaliwal RS, Maguire AM, Flower RW, Arribas NP (1993). "Acute posterior multifocal placoid pigment epitheliopathy. An indocyanine green angiographic study". Retina. 13 (4): 317–25. doi:10.1097/00006982-199313040-00009. PMID   8115733.
  11. ↑ Klufas MA, Phasukkijwatana N, Iafe NA, Prasad PS, Agarwal A, Gupta V, Ansari W, Pichi F, Srivastava S, Freund KB, Sadda SR, Sarraf D (2017). "Optical Coherence Tomography Angiography Reveals Choriocapillaris Flow Reduction in Placoid Chorioretinitis". Ophthalmol Retina. 1 (1): 77–91. doi:10.1016/j.oret.2016.08.008. PMID   31047399.
  12. ↑ Salvatore S, Steeples LR, Ross AH, Bailey C, Lee RW, Carreño E (July 2016). "Multimodal Imaging in Acute Posterior Multifocal Placoid Pigment Epitheliopathy Demonstrating Obstruction of the Choriocapillaris". Ophthalmic Surg Lasers Imaging Retina. 47 (7): 677–81. doi:10.3928/23258160-20160707-12. PMID   27434902. S2CID   9952347.
  13. ↑ Burke TR, Chu CJ, Salvatore S, Bailey C, Dick AD, Lee RJ, Ross AH, Carreño E (October 2017). "Application of OCT-angiography to characterise the evolution of chorioretinal lesions in acute posterior multifocal placoid pigment epitheliopathy". Eye (Lond). 31 (10): 1399–1408. doi:10.1038/eye.2017.180. PMC   5639187 . PMID   28983094.
  14. ↑ 1. Steptoe PJ, Pearce I, Beare NAV, Sreekantam S, Mohammed BR, Barry R, Steeples LR, Denniston AK. Proposing a Neurotropic Etiology for Acute Posterior Multifocal Placoid Pigment Epitheliopathy and Relentless Placoid Choroidopathy. Investigative Ophthalmology & Visual Science (2021) 62:3447–3447. doi:10.3389/fopht.2021.802962 https://www.frontiersin.org/articles/10.3389/fopht.2021.802962/full
  15. ↑ Wolf MD, Alward WL, Folk JC (June 1991). "Long-term visual function in acute posterior multifocal placoid pigment epitheliopathy". Arch Ophthalmol. 109 (6): 800–3. doi:10.1001/archopht.1991.01080060064025. PMID   2043067.
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