Features

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an idiopathic bilateral inflammatory condition most often affecting young individuals aged 20 to 40 years with equal sex predilection. The disease presents with painless, rapid-onset vision loss, and about half of patients report antecedent flu-like symptoms. Uncommonly, central nervous system (CNS) vasculitis has been reported with serious complications, including parenchymal and basal ganglia stroke, intracranial hemorrhage, and small-vessel vasculopathy. While APMPPE is a self-limited disease, there are other forms of this disorder that have a more unfavorable course and prognosis. Persistent placoid maculopathy is remarkable for persistent choroidal ischemia that may last weeks or months and is often complicated by choroidal neovascularization (CNV). Relentless placoid chorioretinitis may be associated with multiple recurrent lesions in the posterior pole and periphery. Serpiginous choroidopathy may also have a relentless course with the development of lesions in a helicoid pattern around the disc and toward the fovea. These variants fall on a spectrum that can be difficult to specifically categorize some cases.

The exact mechanism of APMPPE is unknown; however, a primary inflammatory process involving the choriocapillaris or the retinal pigment epithelium (RPE) has been proposed. Recently, optical coherence tomography (OCT) angiography (OCTA) and indocyanine green angiography (ICGA) have identified flow reduction in the choriocapillaris, suggesting this layer is the primary site of disease. The underlying etiology may be an immune-driven reaction triggered by viral illness or vaccination rather than direct infection. APMPPE has also been reported after vaccination against meningococcus C, mumps, influenza, and hepatitis B. The disease has been grouped into the so-called uveocerebral vasculitic syndromes along with Vogt–Koyanagi–Harada (VKH) disease, Susac syndrome, and Eales disease. Occlusive choroidal vasculitis along with systemic vasculitis and neurologic complications may be linked. An association has also been identified with infectious and inflammatory conditions caused by delayed-type hypersensitivity reactions, including sarcoidosis, tuberculosis (TB), ulcerative colitis, and group A Streptococcus infection. APMPPE has also been observed in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis and syphilis. Approximately 40 to 50% of patients show positivity for human leukocyte antigen (HLA)-DR2 and HLA-B7, which suggests a common susceptibility to abnormal immune activation.

69.1.1 Common Symptoms

Ocular

Typically, patients note acute-onset vision loss, metamorphopsia, and scotomas; at least 75% of patients have bilateral disease or experience sequential onset of symptoms affecting the fellow eye within days or weeks.

Systemic (Nonneurologic)

About 50% of patients report an antecedent flu-like illness. Other symptoms include erythema nodosum, thyroiditis, nephritis, and systemic vasculitis.

Neurologic

Headache is common. Patients may experience hearing loss, seizure, stroke symptoms, cranial nerve palsies, meningoencephalitis, and cavernous sinus thrombosis. Neurologic complications are important to exclude because APMPPE-associated CNS vasculitis can have serious consequences, including death in rare circumstances.

69.1.2 Exam Findings

In the acute stage, multifocal, plaque-like, creamy yellow–white lesions at the level of the RPE are present that mask the underlying choroidal features (▶ Fig. 69.1). Lesions are localized to the posterior pole and may extend to the midperiphery. There may be mild to moderate vitreous inflammation, although the anterior chamber is typically quiet. New lesions may arise over days or weeks and existing lesions may enlarge. A temporal dissociation of lesions is characteristic. Retinal findings differ in the acute versus the chronic stage.

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