Abstract
Purpose
To describe a patient who developed concurrent acute posterior multifocal placoid pigment epitheliopathy (APMPPE) and posterior scleritis.
Observations
We describe a middle-aged woman that developed eye pain and photopsia. She was found to have a “T-sign” on ultrasound of the right eye and multiple, nearly confluent, ill-defined subretinal whitish lesions in both eyes. After an extensive laboratory evaluation and neuroimaging, her photopsia, pain with eye movements, and subretinal lesions began to regress on high dose systemic corticosteroids.
Conclusions and Importance
This is the first reported case of bilateral APMPPE and concurrent posterior scleritis. Our case highlights the importance of performing a full review of systems, specifically eliciting neurological changes, and dilated eye examination in all new uveitis cases.
Introduction
Posterior scleritis is defined by painful inflammation of the posterior sclera exacerbated by eye movements and represents only 1 in 10 of all cases of scleritis. While rare, this entity can be associated with underlying systemic diseases (19.4–37.7% of cases). Misdiagnosis, at least initially, is not uncommon with vague symptoms and subtle clinical findings. As such, ultrasonography has become a mainstay in evaluation. , Other anatomic regions of the eye can be affected by posterior scleritis such as an anterior chamber reaction, optic nerve edema, choroidal nodules, and neurosensory retinal detachments.
Unlike posterior scleritis, acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is not frequently associated with underlying systemic conditions. However, like posterior scleritis, it is rare with an estimated incidence of 0.15 cases per 100,000 persons. Patients can develop photopsia, metamorphopsia, and varying levels of vision loss that may be preceded by a viral prodrome. This typically self-limited, bilateral, inflammatory chorioretinopathy is defined by multiple subretinal, yellowish-white, 1/8th – 1/4th optic disc diameter placoid lesions of the posterior pole that block early and stain late on fluorescein angiography. There can be some involvement of the anterior segment (cells and flare) and vitritis at the time of presentation. Similarly, serpiginous choroiditis is a rare, bilateral inflammatory chorioretinopathy, but unlike APMPPE, is typically a single, yellowish, larger lesion that extends centrifugally from the peripapillary region in a “snake-like” manner. Ampiginous choroidopathy is on the spectrum of APMPPE and serpiginous chorioretinitis in which the APMPPE-like placoid lesions tend to fuse creating larger subretinal lesions and has a predictable fundus autofluorescence pattern in which subtle hyperautofluorescence in areas of activity are seen early in the disease course that then become hypoautofluorescent as activity subsides. Ampiginous lesions can then become recurrent compared to the chronic activity of serpiginous chorioretinitis.
As with any acute uveitis flare, multiple anatomical structures within the eye can be affected simultaneously. For example, it is not uncommon to have macular edema with an anterior uveitis or vascular leakage in pars planitis. However, to our knowledge, concurrent APMPPE and posterior scleritis have not been reported to occur contemporaneously. In the following manuscript, we describe the first case and our management of ampiginous choroiditis with concurrent posterior scleritis.
Case report
A 62-year-old-woman with past medical history significant for a prior infection with histoplasmosis requiring a partial lung resection thirty years prior to presentation was referred to our uveitis clinic due to worsening pain with eye movements of several weeks’ duration and photopsia of unclear etiology. At the time of evaluation, her BCVA was 20/30 in her right eye (OD) and 20/20 in her left eye (OS) and her intraocular pressures measured 12 mmHg in both eyes (OU). Her ocular motility examination was unremarkable except that she noted pain in all gazes. Her anterior segment exam was remarkable for tenderness to palpation of OD greater than OS and the lack of cell and flare. Dilated fundus examination revealed no cell or haze within the vitreous, but there were large, irregular patches of whitish, ill-defined, subretinal lesions most prominent around the optic nerve and within the macula ( Fig. 1 ). These were in contrast to the other well-circumscribed, partially pigmented, atrophic choroidal lesions within this same area ( Fig. 1 ). Multimodal imaging further defined the irregular subretinal lesions discovered on clinical examination. On autofluorescence, these lesions were hyper- and hypoautofluorescent, while on fluorescein angiography these lesions appeared to block early and stain late with late leakage around the borders of the lesions ( Fig. 1 ). B-scan ultrasonography identified a “T-sign” OD with fundus thickening and infiltration of Tenon’s space, but no definitive “T-sign” OS ( Fig. 1 ).
A review of systems, past medical history, and focused laboratory evaluation were unremarkable for granulomatosis with polyangiitis, tuberculosis, sarcoidosis, syphilis, rheumatoid arthritis, and basic metabolic or hematologic derangements, known underlying systemic conditions associated with posterior scleritis, or choroiditis except for positive HLA-B27 haplotyping ( Table 1 ). These symptoms and clinical findings (multiple, large creamy subretinal lesions of various ages) we felt were consistent with a posterior scleritis and concurrent APMPPE/ampiginous choroiditis spectrum disease OU.
| Serum | |
|---|---|
| HLA-B27 | Pos |
| HLA-B51 | Neg |
| Quantiferon Gold | Neg |
| ACE/Lysozyme | WNL |
| Rheumatoid factor | WNL |
| ANA | WNL |
| FTA/RPR | Neg |
| Bartonella Serology | Neg |
| ANCA | Neg |
| Brucella | Neg |
| Borellia | Neg |
| anti-dsDNA | Neg |
| Toxoplasmosis Serology | Neg |
| HIV | Neg |
| ESR | WNL |
| CRP | WNL |
| TSH | WNL |
| T4 | WNL |
| CSF | |
| Protein | WNL |
| Glucose | WNL |
| Cell Count | WNL |
| Culture | Unremarkable |
| HSV PCR | Neg |
| VZV PCR | Neg |
| Cryptococcal antigen | Neg |
| Oligoclonal Bands | Neg |
| VDRL | Neg |
| Cytology | Unremarkable |
| Flow cytometry | Unremarkable |
| Imaging | |
| CT Chest | Remote infection, no signs of sarcoidosis |
| MRI Brain | Hyperintense lesions of the brain |
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