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[Acute posterior multifocal placoid pigment epitheliopathy. Study of 16 cases]
A Burés-Jelstrup1, A Adán, R Casaroli-Marano
1Hospital Clinic de Barcelona, Barcelona, España.
Objective:
Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) is a rare disease with a probable inflammatory component which mostly affects young patients. The aim of our study was to analyse the demographic and clinical features of this disease in a group of 16 patients.
Methods:
Sixteen patients with APMPPE were included in this study. We analyzed their demographic data (age, sex) and the most relevant clinical findings: visual acuity and retinal disease outcome, association with other systemic diseases and response to treatment. We also collected data from fluorescence angiography, autofluorescence and optical coherence tomography (OCT) in some of the patients.
Results:
Average age at diagnosis was 26.75 years with no sex predilection. Average final visual acuity (Snellen Scale) in our study was 0.73. Four patients presented with a systemic disease related to the APMPPE. Eleven patients were treated with oral steroids (one patient with steroids and cytotoxic agents) while the remaining 5 patients received no treatment.
Conclusions:
In our patients, the average age at diagnosis was less than 30 years, with no sex predilection, as previously described by many authors. The visual outcome is usually good regardless of the treatment given, although there are cases with a bad visual outcome, especially those with foveal involvement when initially seen.
Insights
Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) typically affects young adults with good visual outcomes, often irrespective of treatment. Early foveal involvement can indicate a poorer prognosis in this rare inflammatory eye condition.
Area of Science:
- Ophthalmology
- Retinal Diseases
- Inflammatory Eye Conditions
Context:
- Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) is a rare inflammatory condition affecting young patients.
- Understanding the demographic and clinical spectrum of APMPPE is crucial for diagnosis and management.
Purpose:
- To analyze the demographic and clinical features of APMPPE in a cohort of 16 patients.
- To evaluate visual acuity outcomes, systemic associations, and treatment responses in APMPPE.
Summary:
- The study included 16 patients diagnosed with APMPPE, with an average age at diagnosis of 26.75 years and no significant sex predilection.
- Average final visual acuity was 0.73 (Snellen Scale). Four patients had associated systemic diseases.
- Eleven patients received oral steroids, one received steroids and cytotoxic agents, and five received no treatment.
Impact:
- The findings confirm APMPPE predominantly affects individuals under 30 without sex bias.
- Visual outcomes are generally favorable, though foveal involvement at presentation may predict worse vision.
- This study contributes to the understanding of APMPPE's clinical course and prognostic factors.
