Acute posterior multifocal placoid pigment epitheliopathy

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Acute posterior multifocal placoid pigment epitheliopathy
Placoid lesions seen on fundal examination in APMPPE
SpecialtyOphthalmology

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).

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

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[edit]

References[edit]

  1. ^ a b c d 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. ^ a b c 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. 79 (4). BMJ group: 384–389. doi:10.1136/bjo.79.4.384. PMC 505108. PMID 7742290.
  4. ^ a b 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.

External links[edit]

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