The full range of ophthalmological clinical manifestations in systemic lupus erythematosus.

Purpose: To determine the full range of ophthalmological clinical manifestations in systemic lupus erythematosus (SLE) and to compare the systemic features associated with them. Methods: Files of 13 patients with ocular SLE (n = 20 eyes) diagnosed as per the American College of Rheumatology (ACR) 2012 revised criteria were retrospectively reviewed. Results: The following clinical manifestations were found: keratoconjunctivitis sicca (n = three patients), anterior uveitis associated with an inflammatory pseudo-tumor orbital mass (n = one patient, one eye), episcleritis and periorbital edema (n = one patient, two eyes), posterior scleritis (n = one patient, two eyes), bilateral papillary edema in the context of idiopathic intracranial hypertension (n = one patient, one eye), inflammatory optic neuritis (n = one patient, one eye), and lupus retinopathies with varying degrees of capillary occlusions mainly arteriolar (n = seven patients, 13 eyes) and larger arteries or veins (retinal arteries occlusions and retinal veins occlusions) (n = one patient, two eyes). Some patients presented with combined ophthalmological manifestations.Systemic SLE was discovered by its ophthalmic manifestation in three cases (23%) and was previously known in the other 10 cases (77%). On average, ocular symptoms were seen 8 years after the initial diagnosis of SLE. Other systemic SLE disorders included cutaneous disorders (77%), joint disorders (38%), central nervous system (CNS) disorders (23%), renal disorders (38%), and oral ulcers (23%).Treatment of the ophthalmic system manifestations of lupus included local steroid therapies along with systemic immunosuppression.The most common laboratory ACR criteria were: high levels of antinuclear antibodies (ANA) (100%), positive anti-Sm (64%), anti-dsDNA (27%), low complement levels (27%), and positive antiphospholipid (APL) antibodies (18%). Discussion: SLE activity in the ophthalmic system is characterized by its functional severity and the range of involvement can be categorized by anatomical involvement: presence of anterior uveitis, episcleritis, scleritis, periorbital edema, posterior uveitis with retinal vascular ischemia, or papillary edema. Not currently part of the diagnosis criteria of the SLE ACR given its rarity, the ocular localization of the pathology led to the diagnosis of SLE in three cases; thus, developing a greater understanding of ocular lupus may help in identifying and treating systemic manifestations of lupus earlier.

DIRECTIONAL VARIABILITY OF FUNDUS REFLECTANCE IN ACUTE MACULAR NEURORETINOPATHY: EVIDENCE FOR A CONTRIBUTION OF THE STILES-CRAWFORD EFFECT.

PURPOSE: To document directional reflectivity of fundus lesions in a case of acute macular neuroretinopathy. METHODS: Case report. Clinical and imaging data from a patient with acute macular neuroretinopathy were reviewed. Imaging comprised infrared scanning laser ophthalmoscopy, optical coherence tomography and flood-illumination adaptive optics images acquired through different entry pupils in the cardinal directions (approximately 2° eccentricity). RESULTS: The patient reported acute bilateral paracentral scotoma revealing dark, wedge-shaped macular plaques which by optical coherence tomography were associated with focal loss of the visibility of the cone outer segment tip and inner/outer segment lines. Comparing scanning laser ophthalmoscopy images taken at different entry points in the pupil showed that macular plaques varied from hyporeflectance to isoreflectance. Cone counts by flood-illumination adaptive optics within plaques and optical coherence tomography features of the cone outer segment tip showed also a strong directional variability, peaking at near-normal values. Within each modality, fusion images showed that directional variability covered most of macular plaques. CONCLUSION: The characteristic fundus abnormalities of acute macular neuroretinopathy may show a strong directional variability. Our findings suggest that the Stiles-Crawford effect may be an important factor in signs and symptoms of acute macular neuroretinopathy.

Electroretinogram Findings in Early-Stage Sickle Cell Retinopathy According to Hemoglobin Type.

Purpose: Although extensive clinical research has been performed on structural analysis of sickle cell (SC) retinopathy, functional aspects have been poorly investigated. Our purpose was to report full-field electroretinogram (ffERG) findings in patients with early SC retinopathy according to the following hemoglobin types: HbSS or HbSC (homozygous or heterozygous mutations, respectively). Methods: In this monocentric retrospective observational study, patients affected by nonproliferative SC retinopathy were included from November 2014 to April 2016. Patients were separated into one of the following three groups: HbSS, HbSC, and control. All groups underwent full ophthalmologic examination (fundus examination) and ffERG. For SC patients, additional imaging testing was also performed (fluorescein angiography and spectral domain optical coherence tomography). Results: A total of 24 eyes from 12 patients (6 HbSS and 6 HbSC) and 12 eyes from 6 controls were included. The HbSS group exhibited a dramatic decrease of the b-wave amplitudes for all dark-adapted (DA) ffERG responses when compared with the control group (P = 0.02, P = 0.003, P = 0.005, respectively, after DA 0.01, DA 3.0, and DA 10.0 cd.s.m-2 stimulations) and decreased a-wave amplitudes for light-adapted responses (P = 0.03 after light-adapted 3.0 cd.s.m-2 stimulations). The a-Wave amplitudes were significantly reduced for all dark-adapted and light-adapted responses in HbSC group compared to the control group (P = 0.03, P = 0.01, P = 0.03, respectively, after DA 3.0, DA 10.0, and light-adapted 3.0 cd.s.m-2 stimulations). The HbSS+HbSC groups presented decreased a-wave amplitudes for DA and light-adapted responses and decreased b-wave amplitude after DA 0.01 and 10.0 cd.s.m-2 stimulations when compared to the control group. Conclusions: These results could suggest an early involvement of the inner retinal cells in the disease process in HbSS patients and of the outer retinal cells in HbSC patients. This could provide new insights on the pathophysiology of the retinal affection in HbSS/HbSC SC disease.

DIFFUSE LARGE B-CELL LYMPHOMA-ASSOCIATED-RETINOPATHY CHARACTERIZED BY MINIMAL MORPHOLOGIC CHANGES AND SEVERE FUNCTIONAL IMPAIRMENT.

PURPOSE: To report the case of a patient whose retinal disease was found to be associated with a diffuse large B-cell lymphoma found 30 years after the apparent successful treatment of a classical Hodgkin lymphoma. METHODS: Observational case report. RESULTS: The authors describe the case of a 69-year-old man referred to their Department because of progressive, bilateral vision loss over the last few months. Deterioration in color vision and intense photophobia were also present. His best-corrected visual acuity was 20/400 in the right eye (RE) and 20/800 in the left eye (LE). Slit lamp and fundus examination failed to show any abnormalities. Spectral domain optical coherence tomography (SD-OCT) detected diffuse attenuation of the ellipsoid layers in addition to a focal subfoveal defect in both eyes. Both fluorescein and indocyanine angiographies (FA and ICGA) were normal. Full flash electroretinogram (ERG) revealed bilateral cone rod dysfunction with decreased amplitudes of both a and b waves. CONCLUSION: Because of the late onset of the disease, poor visual acuity compared with a small macular anatomical lesion and a history of Hodgkin lymphoma 30 years ago, a neoplastic etiology was investigated. Poor performance status and chest pain led to a thoracic CT scan, which identified a massive mediastinal tumor. Serum analysis found an abnormal amount of antibody activity within the 40 kD region of Western blot of retina. The diagnosis of diffuse large B-cell lymphoma was established. Systemic examinations found a Stage IV non-Hodgkin lymphoma.