AB1145 Acute posterior multifocal placoid pigment epitheliopathy: clinical pattern and management of 79 patients
Authors: J. Álvarez Troncoso, A. Schlinker Giraud, F. Arnalich Fernández, Á. Robles. Marhuenda
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) is an uncommon inflammatory disease causing acute-onset chorioretinal bilateral disease. It typically affects the posterior pole of both eyes leading to visual blurring or scotomas. Although it is thought to be benign, APMPPE has been associated with central nervous system (CNS) manifestations: cerebral vasculitis, meningoencephalitis and cerebral vascular disease. <h3>Objectives</h3> The aim of this study was to define clinical features, systemic manifestations, treatment and outcomes of a review of 79 patients with APPMPE. <h3>Methods</h3> We retrospectively analysed the epidemiology, potential triggers, prodromes, clinical data, ophthalmological study, extraocular manifestations, treatment and outcomes of 79 patients collected through an extensive review of the literature from the first description of Gass JD up to the present time. <h3>Results</h3> A total of 79 patients were reviewed (47 male). Mean age at diagnosis was 30 years (with a range of 8 to 58 years old). 27 patients (34.2%) presented with a previous triggering being flu-like illness the most frequent.<sup>15</sup> However, the complete serological study was only requested in 28 patients (and the immunological study just in 22) Median time from trigger to overt APPMPE was 9 days. Main clinical symptoms were: decreased visual acuity/blurry vision (100%), headache (51.8%) and photophobia (12.2%). Average decreased visual acuity was 13/20. APPMPE is defined by the presence of multiple white-yellowish plaques in funduscopy, and early hypofluorescent areas with late hyperfluorescence in fluorescein angiography. The fundus was pathological and compatible with APPMPE in all cases (100%), as was fluorescein angiography in those that had been performed (59). CNS involvement appeared in up to 50.6% (40 patients). The CNS manifestations were divided into language disorders (11 patients), motor deficit,<sup>20</sup> sensory deficit<sup>20</sup> and other CNS manifestations.<sup>23</sup> The mean time from visual deficit to neurological manifestations was approximately 2 weeks. Cerebrospinal fluid was studied in 37 patients, with a predominance of lymphocytic pleocytosis (mean of 46 cells/mm3) and elevated proteins (mean of 111 mg/dl). Within the neuroimaging studies carried out (58) up to 69.7% were pathological. 67 patients (84.8%) received treatment with corticosteroids. 14 patients (17.7%) also received other immunosuppressants (mainly azathioprine and cyclophosphamide), especially if CNS involvement. Regarding the evolution, 55 patients (69.6%) presented improvement, 12 (15.2%) relapsed and 6 (7.5%) died due to APPMPE. <h3>Conclusions</h3> APPMPE is a rare inflammatory disease which primarily affects the retina. However, the CNS involvement could be more frequent than what is classically described. Also, it seems that there might be a trigger effect either inflammatory or infectious. Steroids and immunosuppressants should be considered in patients with CNS involvement from the beginning. <h3>Reference</h3> [1] Case D, Seinfeld J, Kumpe D, Folzenlogen Z, Jones W, Simpson J, et al. Acute posterior multifocal placoid pigment epitheliopathy associated with stroke: a case report and review of the literature. J Stroke Cerebrovasc Dis2015;24:e295-e302. <h3>Disclosure of Interest</h3> None declared