Sex: F
Vaccine: MEN
Symptoms: Fluorescence angiogram abnormal, Full blood count normal, HLA marker study positive, HLA-B gene status assay, Headache
Clinical narrative: Acute posterior multifocal placoid pigment epitheliopathy; This is a literature report for the following literature source(s):   A 25 year-old female patient received meningococcal group b rlp2086 (MENINGOCOCCAL GROUP B RLP2086) (Batch/Lot number: unknown) as single dose for immunisation. The patient's relevant medical history and concomitant medications were not reported. Vaccination history included: Hepatitis a vaccine, for immunization; Polio vaccine, for immunization; Tetanus vaccine, for immunization. The following information was reported: RETINAL PIGMENT EPITHELIOPATHY (medically significant, life threatening), outcome "recovered", described as "Acute posterior multifocal placoid pigment epitheliopathy". Therapeutic measures were taken as a result of retinal pigment epitheliopathy. This literature is to report a case of acute posterior multifocal placoid pigment epitheliopathy occurring in temporal association with multiple immunizations in a previously healthy 25-year-old woman. Acute posterior multifocal placoid pigment epitheliopathy was diagnosed based on ophthalmological findings of bilateral placoid subretinal lesions complicated by a serous retinal detachment in the left eye. A previously healthy 25-year-old woman presented with left eye central vision loss in addition to scotoma and a severe headache. Previous to vision loss, she experienced headaches in increasing severity over the course of four days, not relieved with 800 mg of ibuprofen daily. Three weeks prior, she received immunizations. Inoculations included previously received hepatitis A, polio, and tetanus vaccines and her first meningococcal vaccine. There is no history of adverse event to immunizations. Other than a papular rash involving her hands, forearms, feet, and back 3 months earlier, she was in good health. Ophthalmology evaluation included vision, intraocular pressure, slit-lamp, and dilated fundus examination. Examination showed extensive swelling of her macula on the left side with contiguous areas of hyperpigmentation near the superior arcade and hypopigmentation in the posterior pole of the right eye along the superior arcade. The macula optical coherence tomography demonstrated marked subretinal and intraretinal fluid in her left eye. The fluorescein angiography and ICG demonstrated small areas of hypofluorescence in her right eye and placoid lesions with large blocking defect with areas of late leakage and pooling in her left eye. Acute posterior multifocal placoid pigment epitheliopathy was diagnosed based on ophthalmological findings. Neurologic conditions associated with headache were excluded with negative magnetic resonance imaging, computed tomography, lumbar puncture, and X-Ray. Comprehensive viral and bacterial assays, complete blood count, metabolic panels, cardiolipin, and inflammatory markers were negative except for a mildly elevated C-reactive protein (CRP) of 1.26 (less than 0.5) and coxsackie A immunoglobulin g (IgG) of 1:400 (less than 1:100). Through human leukocyte antigen (HLA) typing, the patient was found to possess the HLA-B 40 and HLA-DB1 15 alleles. She was treated with topical prednisolone acetate 1%, which was tapered after three weeks. She was monitored for several months, and visual acuity returned to normal. Conclusion: After investigating other possible causes, immunization was the only identifiable event preceding this patient's APMPPE. Other than temporality, review of published case reports reveals no distinguishing epidemiologic (trigger association, interval, etc.) or demographic (age, gender, etc.) features among the APMPPE cases associated with vaccines. However, this does not rule out vaccines' potential role in triggering APMPPE through an unidentified immune-mediated process in susceptible individuals. Authors have suggested that molecular mimicry, excessive lymphocyte activation, immune complex formation, or cytokine expression may play a role in the pathogenesis of APMPPE. Both MHC (HLA) class I and II cell-surface receptors responsible for the regulation of the immune system in a series of patients with APMPPE. The authors determined that possessing MHC class I HLA-B7 or MHC class II HLA-DR2 antigens conferred a relative risk of acquiring APMPPE of 3.38 and 3.34, respectively. For autoreactive T or B cells to develop, their receptors must be configured such as to capture a foreign protein that shares structural similarities with specific self-proteins. This is known to occur in type I diabetes mellitus (HLA-DR3 and DR4) and ankylosing spondylitis (HLA-B27). In very few of the reported cases of APMPPE have the patients' HLA receptor typing been determined. The author's patient possesses the HLA-DR2 antigen (DRB1 15 allele). The only other vaccine associated case of APMPPE in which HLA typing was performed was also HLA-DR2 positive. HLA antigens associated with infection-associated APMPPE or APMPPE-like presentations include HLA-B7, HLAB27, and HLA-A3 andC7.5,10 TheMHC class I antigens (HLA-A, B, and C) are found on most nucleated cells and are widely distributed in the eye including the vascular (iris, ciliary body, retinal, and choroidal) endothelium. MHC class II antigens (HLA-D) are more restricted and present only on dendritic cells, B-lymphocytes, macrophages, endothelial cells, and activated T-cells. In the eye, they are found on cells in the limbus and uvea (including the choroid). With RPE and/or vascular endothelial cell activation and DR antigen expression, it is possible that either RPE or vascular endothelium serves as antigen-presenting cells and participates in the ocular inflammatory response. The HLA-DR2 receptor may act in concert with infectious or vaccine epitopes to produce the characteristic chorioretinal lesions. As there are manufacturing dissimilarities between the meningococcal vaccine received by this patient and meningococcal vaccine causality cannot be assumed.  No follow-up attempts are possible; information about lot/batch number cannot be obtained. No further information is expected.; Sender's Comments: A causal association between meningococcal group b rlp2086 and retinal pigment epitheliopathy cannot be excluded based on a compatible temporal relation. The impact of this report on the benefit/risk profile of the Pfizer product is evaluated as part of Pfizer procedures for safety evaluation, including the review and analysis of aggregate data for adverse events. Any safety concern identified as part of this review, as well as any appropriate action in response, will be promptly notified to RAs, Ethics Committees, and Investigators, as appropriate.