Correlation of Immunological Markers with Disease and Clinical Outcome Measures in Patients with Autoimmune Retinopathy

Abstract

To determine if immunological markers (1) are significantly different between autoimmune retinopathy (AIR) patients and controls and (2) correlate with disease progression in AIR patients. Methods: We enrolled patients with a possible AIR diagnosis, as well as control participants without eye disease, autoimmunity, or cancer. Immunological markers were tested in all participants. In addition, AIR patients had up to three blood draws for testing over their disease course. For AIR patients, clinical measures, including visual acuity (VA) and Goldmann visual field (GVF) area, were recorded at each draw. We used the Mann-Whitney U test to compare the immunological markers between AIR patients and controls. We used multilevel mixed-effect regression to investigate the correlation between markers and clinical parameters over time in AIR patients. Results: Seventeen patients with AIR and 14 controls were included. AIR patients had a higher percent of monocytes (Z = 3.076, P = 0.002). An increase in immunoglobulin G against recoverin was correlated with a VA decrease (β = 0.0044, P < 0.0001). An increase in monocyte proportion was correlated with a decrease in GVF area (β = −7.27, P = 0.0021). Several markers of B-cell depletion were correlated with GVF improvement. Conclusions: Monocytes may play a role in AIR pathophysiology and be a disease activity marker. B-cell depletion markers correlated with clinical parameter improvement, particularly GVF. Translational Relevance: This work elucidates immunologic markers that may improve the accuracy of diagnosis and treatment of AIR.

Ocular Paraneoplastic Syndromes

“…Central or ring scotomas should be detected in visual field examination. In electroretinography (ERG), rod- and cone-mediated responses are not recordable or significantly decreased, firstly affecting the a-wave and subsequently lining rapidly to a “flat ERG” [ 8 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 ].…”

“…There are auto-anti-retinal proteins associated with visual symptoms and structural disturbances of retina in paraneoplastic and nonparaneoplastic autoimmune retinopathies [ 20 ]. Interestingly, there are some similarities in the clinical presentation of retinopathy associated with specific autoantibodies.…”

“…In 2018, Adamus [ 20 ] indicated that there was a clinical phenotype for antirecoverin-associated retinopathy that is different from antienolase-associated changes. Differences include onset, ocular symmetry, and clinical presentation, as well as prognosis of the best corrected visual acuity and electrophysiological changes.…”

“…During the course of the disease, rapid rod and cone loss is seen, often with vision acuity reduced to light perception or to no light perception in some cases. The ffERG is useful since there is a severe decrease in response of rods and cones, in addition to abnormal multifocal ERG [ 7 , 20 , 22 , 83 ]. Antienolase Antienolase antibodies are associated with a subacute or even chronic presentation that is often symmetric.…”

Outer Retinopathy and Microangiopathy in Acute Myelogenous Leukemia

“…5 The abnormalities overlap with another large group of conditions within the spectrum of acute zonal outer occult retinopathy (AZOOR), autoimmune retinopathies (AIRs), and acute fovealitis. [6][7][8][9] In this report, we describe a young woman with acute myelogenous leukemia (AML) who presented with recurrent, bilateral central vision loss, which became severe after consolidative treatment with peripheral blood stem cell transplantation (PBSCT) complicated by cGVHD. The patient showed discrete areas of outer segment and photoreceptor loss that developed slowly over time in each eye, before and after undergoing PBSCT, and which ultimately improved with intravenous rituximab treatment.…”