Acute posterior multifocal placoid pigment epitheliopathy

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).

Acute posterior multifocal placoid pigment epitheliopathy
SpecialtyOphthalmology

It occurs more commonly in females and is more likely to affect persons between 20 and 30 years of age, but has been seen in people aged 16 to 40. 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.[1][2]

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, livido reticularus, 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.[3]

Cause

Since The cause of the inflammation remains unknown, with various theories of it occurring as an autoimmune response to a mild infection, or the possibility of it being viral because of the preceding flu-like illness that generally accompanies it.[1][3] It is usually associated with HLA-B7 and HLA-DR2.

The underlying etiology of APMPPE continues to cause debate.[4][5] The term ‘Pigment Epitheliopathy’ was chosen by Gass[6] to reflect what he thought was the tissue most significantly affected. Van Buskirk et al,[7] and Deutman et al[8] proposed choriocapillaris ischemia as the more likely primary etiology. Indocyanine green angiography (ICGA),[9] and OCT angiography (OCTA)[10][11][12] studies have provided support for choriocapillaris involvement.

However, a novel hypothesis has recently been proposed implicating a direct neurotropic infection as a possible underlying cause given the dynamic changes observed along the neuronal pathway of the retina [13]

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.

Treatment

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.

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.[1][14]

See also

References
  1. Comu, S; Verstraeten, T; Rinkoff, JS; Busis, NA (May 1996). "Neurological manifestations of acute posterior multifocal placoid pigment epitheliopathy". Stroke: A Journal of Cerebral Circulation. 27 (5): 996–1001. doi:10.1161/01.str.27.5.996. PMID 8623125.
  2. 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.
  3. De Vries, J.J. (June 2006). "Acute Posterior Multifocal Placoid Pigment Epitheliopathy With Cerebral Vasculitis: A Multisystem Granulomatous Disease - Archives of Ophthalmology". archopht.ama-assn.org/. 124 (6): 910–913. doi:10.1001/archopht.124.6.910. Retrieved 2009-09-15.
  4. Zhang AY, Han IC, Goldberg MF. Renaming of Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) to Acute Multifocal Placoid Choroidopathy (AMP-C). JAMA Ophthalmol. 2017;135(3):185. doi:10.1001/jamaophthalmol.2016.5325
  5. Jampol LM, Goldstein DA, Fawzi AA. Keeping the Name of Acute Posterior Multifocal Placoid Pigment Epitheliopathy. JAMA Ophthalmol. 2017;135(3):186. doi:10.1001/jamaophthalmol.2016.5334
  6. Gass JDM. Acute Posterior Multifocal Placoid Pigment Epitheliopathy. Arch Ophthalmol. 1968;80(2):177-185. doi:10.1001/archopht.1968.00980050179005
  7. Van Buskirk EM, Lessell S, Friedman E. Pigmentary Epitheliopathy and Erythema Nodosum. Arch Ophthalmol. 1971;85(3):369-372. doi:10.1001/archopht.1971.00990050371025
  8. Deutman A, Oosterhuis J, Boen-Tan T, Aan de Kerk A. Acute posterior multifocal placoid pigment epitheliopathy - Pigment epitheliopathy or choriocapillaritis. Br J Ophthalmol. 1972;56(12):863-874. doi:10.1136/bjo.56.12.863
  9. Dhaliwal R, Maguire A, Flower R, Arribas N. Acute posterior multifocal placoid pigment epitheliopathy - An indocyanine green angiographic study. Retina- J Retin Vitr Dis. 1993;13(4):317-325. doi:10.1097/00006982-199313040-00009
  10. Klufas MA, Phasukkijwatana N, Iafe NA, et al. Optical Coherence Tomography Angiography Reveals Choriocapillaris Flow Reduction in Placoid Chorioretinitis. Ophthalmol Retina. 2017;1(1):77-91. doi:10.1016/j.oret.2016.08.008
  11. Salvatore S, Steeples LR, Ross AH, Bailey C, Lee RWJ, Carreño E. Multimodal Imaging in Acute Posterior Multifocal Placoid Pigment Epitheliopathy Demonstrating Obstruction of the Choriocapillaris. Ophthalmic Surg Lasers Imaging Retina. 2016;47(7):677-681. doi:10.3928/23258160-20160707-12
  12. Burke TR, Chu CJ, Salvatore S, et al. Application of OCT-angiography to characterise the evolution of chorioretinal lesions in acute posterior multifocal placoid pigment epitheliopathy. Eye Lond Engl. 2017;31(10):1399-1408. doi:10.1038/eye.2017.180
  13. 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
  14. Wolf, Mitchell D; Alward, Wallace LM; Folk, James C (June 1991). "Long-term Visual Function in Acute Posterior Multifocal Placoid Pigment Epitheliopathy". Archives of Ophthalmology. 109 (6): 800–3. doi:10.1001/archopht.1991.01080060064025. PMID 2043067.
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