Cardiac sympathetic denervation precedes nigrostriatal loss in the E46K mutation of the alpha-synuclein gene (SNCA).

INTRODUCTION: Here we report the case of an asymptomatic carrier of the E46K substitution in alpha-synuclein gene where we have documented that cardiac sympathetic denervation precedes nigrostriatal dopaminergic loss. MATERIAL AND METHODS: She has been followed up regularly with standard neurological examination, UPDRS, neuropsychological formal testing, parkinson disease sleep scale-PDSS, Epworth scale, Hamilton-D scale, SCOPA Aut, orthostatic hypotension test, brief smell identification test, polysomnography, cerebral 123-I-FP-CIT SPECT, and, 123I-MIBG cardiac scintigraphy. RESULTS: She shows no presence of orthostatic hypotension. Olfactory test results demonstrate normal limits. In the PSG the nocturnal sleep shows mild abnormalities although the sleep efficiency and stage proportion remain under normal limits. The 123-I-FP-CIT SPECT is normal; in contrast, the 123I-MIBG cardiac scintigraphy shows a complete lack of isotopic uptake compatible with a severe sympathetic myocardial denervation. CONCLUSION: This example of monogenic autosomal dominant parkinsonism due to an alpha-synuclein mutation favours the hypothesis that peripheral autonomous nervous system involvement occurs earlier than the CNS degeneration.

Phase I and pharmacologic study of irinotecan administered as a 96-hour infusion weekly to adult cancer patients.

PURPOSE: We conducted a phase I and pharmacologic study of a weekly 96-hour infusion of irinotecan to determine the maximum-tolerated dose, define the toxicity profile, and characterize the clinical pharmacology of irinotecan and its metabolites. PATIENTS AND METHODS: In 26 adult patients with solid tumors, the duration and dose rate of infusion were escalated in new patients until toxicity was observed. RESULTS: In 11 patients who were treated with irinotecan at 12.5 mg/m(2)/d for 4 days weekly for 2 of 3 weeks, dose-limiting grade 3 diarrhea occurred in three patients and grade 3 thrombocytopenia occurred in two patients. The recommended phase II dose is 10 mg/m(2)/d for 4 days given weekly for 2 of 3 weeks. At this dose, the steady-state plasma concentration (Css) of total SN-38 (the active metabolite of irinotecan) was 6.42 +/- 1.10 nmol/L, and the Css of total irinotecan was 28.60 +/- 17.78 nmol/L. No patient experienced grade 3 or 4 neutropenia during any cycle. All other toxicities were mild to moderate. The systemic exposure to SN-38 relative to irinotecan was greater than anticipated, with a molar ratio of the area under the concentration curve (AUC) of SN-38 to irinotecan of 0.24 +/- 0.08. One objective response lasting 12 months in duration was observed in a patient with metastatic colon cancer. CONCLUSION: The recommended phase II dose of irinotecan of 10 mg/m(2)/d for 4 days weekly for 2 of 3 weeks was extremely well tolerated. Further efficacy testing of this pharmacologic strategy of administering intermittent low doses of irinotecan is warranted.

Phase II trial of paclitaxel by 96-hour continuous infusion in combination with cisplatin for patients with advanced non-small cell lung cancer.

Our purpose was to determine the antitumor efficacy and safety profile of the combination of paclitaxel administered by 96-h continuous i.v. infusion followed by bolus cisplatin in patients with untreated advanced non-small cell lung cancer (NSCLC). Fifty-eight patients with untreated advanced or recurrent NSCLC were enrolled between October 1995 and December 1998. The median patient age was 60 years (age range, 34-75 years). Twenty-four patients were female. The majority of patients (n = 52) had an Eastern Cooperative Oncology Group performance status of 0/1. Twelve patients had stage IIIB NSCLC, 43 had stage IV disease, and 3 had recurrent disease after prior resection. Seven patients had received cranial irradiation for brain metastases, and 5 patients had received bone irradiation before enrollment. Patients were treated with paclitaxel (120 mg/m2/96 h) by continuous i.v. infusion followed by cisplatin (80 mg/m2) on day 5. Therapy was administered every 3 weeks as tolerated until disease progression or a maximum of six cycles. A total of 264 cycles of therapy were administered. Twenty-nine patients received all six cycles. Forty-six patients had measurable disease, with 20 patients achieving a partial response, and no complete responses were seen (overall response rate, 43%; 95% confidence interval, 29-60%). The median progression-free survival was 5.5 months. At a median potential follow-up of 27.2 months, the median survival for all 58 enrolled patients was 8.5 months, and the actuarial 1-year survival was 37% (95% confidence interval, 25.9-50.5%). This is the most extensive evaluation of prolonged continuous infusional paclitaxel in patients with advanced-stage cancer. In contrast to predictions from in vitro cytotoxicity models, the regimen does not appear to be obviously superior to shorter infusion times in the clinical setting. Additional trials of this regimen in patients with NSCLC are therefore of low priority.

Cardiocirculatory manifestations in Parkinson's disease patients without orthostatic hypotension.

The objective of this study was to characterize cardiac sympathetic denervation in Parkinson's disease (PD) patients without neurogenic orthostatic hypotension (NOH), both in terms of hemodynamics and in its relation with vascular denervation. We studied 20 PD patients without NOH. We analyzed the heart rate and blood pressure variability during various physical maneuvers. The following parameters were calculated: expiratory-inspiratory ratio, stroke volume, cardiac output, cardiac index, left ventricular ejection time, left ventricular work index, thoracic fluid content, total peripheral resistance and baroreflex sensitivity (BRS). We also measured direct and spectral derivatives of cardiac (cardiovagal) parasympathetic function. Myocardial I-123 metaiodobenzylguanidine (MIBG) scintigraphy was performed and early and late heart/mediastinum uptake ratios were analyzed. We observed that the late heart/mediastinum uptake ratio was 1.33±0.21. This parameter was correlated with years since diagnosis (correlation coefficient:-0.485; P=0.05), Unified Parkinson's Disease Rating Scale (UPDRS) III score (cc:-0.564; P=0.02) and pressure recovery time in the Valsalva maneuver (cc: 0.61; P<0.001). At rest, it was correlated with BRS (cc:0.75; P=0.003) and low-frequency diastolic blood pressure (LFDBP; cc: 0.58;P=0.017). We found no correlations with any of the cardiography impedance variables. In linear regression models, the variable that best correlated with MIBG results was LFDBP. Our results support that in absence of NOH the degree of denervation of the heart does not produce any effect on its inotropic function. Moreover, BRS and LFDBP can be used as an indirect measure of cardiac sympathetic denervation at rest.

A phase I and pharmacologic study of 9-aminocamptothecin administered as a 120-h infusion weekly to adult cancer patients.

PURPOSE: To define the toxicity profile and the recommended phase II doses of 9-aminocamptothecin (9-AC) administered as a weekly 120-h infusion. METHODS: 9-AC was administered over 120 h weekly to 55 adult cancer patients with solid tumors over doses ranging from 0.41 to 0.77 mg/m2 per day in a phase I and pharmacologic study. 9-AC formulated in dimethylacetamide/polyethylene glycol (DMA) was administered on a 3 of 4-week schedule, and the newer colloidal dispersion (CD) formulation was given on a 2 of 3-week schedule. RESULTS: Overall, 193 courses of therapy were administered over 122 dose levels. On the 3 of 4-week schedule, 9-AC DMA infused at > or = 0.6 mg/m2 per day for 120 h weekly produced dose-limiting neutropenia, thrombocytopenia, and diarrhea, or resulted in 1-2-week treatment delays. Shortening treatments to 2 of 3 weeks resulted in dose-limiting neutropenia and fatigue at infusion rates > 0.72 mg/m2 per day. The ratio of 9-AC lactone to total (carboxylate + lactone) drug plasma concentrations at steady-state was 0.15 +/- 0.07. Clinical toxicities and drug pharmacokinetics were not substantially different between the DMA and CD formulations. One objective response was observed in a patient with bladder cancer and minor responses were observed in patients with lung and colon cancers. Plasma area under the concentration versus time curve for 9-AC lactone modestly correlated with the degree of thrombocytopenia (r=0.51) using a sigmoid Emax pharmacodynamic model. CONCLUSION: The recommended phase II dose for the 9-AC DMA formulation is 0.48 mg/m2 per h over 120 h for 3 of 4 weeks and for the 9-AC CD formulation is 0.6 mg/m2 per day over 120 h for 2 of 3 weeks. Both regimens were well tolerated and feasible to administer.

[The gammagraphic diagnosis of acute pulmonary thromboembolism]. / Diagnóstico gammagráfico del tromboembolismo pulmonar agudo.

We aimed to determine the prevalence of acute pulmonary embolism (APE) in our population and to establish the diagnostic reliability of pulmonary scintigraphy (PS), as well as the influence of the clinical context (CC). During a 2-year prospective study, patients were diagnosed by either pulmonary angiography or normal-perfusion PS. A diagnosis of APE was made in 73 (75%) of a total of 97 patients. In the group of 58 patients diagnosed by ventilation/perfusion (V/Q) PS, 33 were classified as "highly probably" having APE; 32 of these in fact had the syndrome (sensitivity 88%, specificity 94%). Combining the "highly probable" patients with the "highly suspected" patients diagnosed by ventilation/perfusion PS, we obtained a sensitivity of 51% and specificity of 100%. Ventilation scintigrams were unobtainable in 28 older patients with greater dyspnea and tachypnea, who were unable to perform the maneuvers. Of the 21 "highly probable" patients as assessed by PS, 19 were diagnosed as having APE (sensitivity 86%, specificity 85%). Combining the "highly probable" patients with the "highly suspected" group, we obtained a sensitivity of 32% and specificity of 100%. Normal perfusion PS excludes clinically relevant APE. "Intermediate" or "slight" probability PS results, even when combined with CC, have no diagnostic validity.

[Predictive value of the pattern-electroretinogram in glaucoma]. / Valor predictivo del pattern-electrorretinograma en el glaucoma.

PURPOSE: To determine the ability of pattern-electroretinogram (PERG) to discriminate individual evolution of glaucomatous visual field losses in glaucoma suspects. METHODS: We studied 151 eyes of 78 GS, classified depending on their risk to develop glaucomatous damage. The initial visual field was normal. Those eyes showing at least 2 reduced amplitudes in the PERG components (P50, N95 and NP) were considered pathologic. The mean follow-up was 30 months (range: 13-50 months); the VF was evaluated at the end of the study. RESULTS: At the beginning of the study the PERG was pathologic in 65 eyes (43%); in 45 of these eyes (69%) the visual field was pathologic at the end of the study. The positive predictive value of the PERG of visual field glaucomatous changes was 69% and the negative predictive value was 80%. The N95 amplitude showed the best predictive value, with 100% and 77% in the high and medium risk groups, respectively. CONCLUSIONS: The PERG study can discriminate the glaucoma suspects that will develop visual field glaucomatous losses.

[Cup-to-disk ratio asymmetry: diagnostic value in glaucoma]. / Asimetría papilar: valor diagnóstico en el glaucoma.

PURPOSE: To assess visual field abnormalities in suspected glaucoma patients showing evident asymmetry in cup-to-disk ratio. MATERIAL AND METHOD: Twenty two hypertensive eyes in 11 subjects evidencing lateral differences in cup-to-disk ratio of more than 2/10 were included in the study. Conventional and short wavelength automated perimetry (SWAP) was performed with a Humphrey field analyser. A retinal nerve fiber layer (RNFL) study was also performed. RESULTS: Differences in IOP were 1.23 mmHg (p<0.05). No differences were evidenced in conventional automated perimetry between both eyes. Average sensitivity (SWAP) showed significantly lower (p<0.05) in those ayes with greater cup-to-disk ratio. RNFL showed a higher rate of defects in eyes with a higher cup-to-disk ratio. CONCLUSION: Lateral differences in cup-to-disk ratio indicate structural glaucomatous damage which is related to IOP levels. Associated functional loss was evidenced with SWAP (Arch Soc Esp Oftalmol 2002; 77: 17-22).

[Short-wavelength automated perimetry (SWAP) in patients with suspected glaucoma (II): correlation with a probabilistic multifactorial model of risk for developing glaucomatous damage]. / Perimetría automatizada de longitud de onda corta (PALOC) en sujetos sospechosos de glaucoma (II): correlación con un modelo multifactorial probabilístico de riesgo de desarrollar daño glaucomatoso.

PURPOSE: To evaluate the efficiency and predictability of a multifactorial probability model to assess the risk of developing glaucomatous visual field defects and to determine the correlation between retinal nerve fiber layer (RNFL) photographs and short-wavelength automated perimetry (SWAP) in a population of patients suspected of having glaucoma. METHODS: One hundred and sixty eyes belonging to 83 patients with ocular hypertension [ocular pressure figures over 21 mmHg and normal conventional automated perimetry (AP) (white-white)] were included in the study. The risk of developing glaucomatous defects was assessed by a multifactorial model that includes intraocular pressure. vertical cup-to-disk ratio, age and family background. RNFL photographs and SWAP tests were also performed. RESULTS: SWAPs were pathological in 57 cases (35.6% of the sample); 23% of the perimetries were abnormal in the low-risk group while the moderate and high risk groups showed abnormal perimetries 38% and 56% respectively. The RNFL defects at the different risk levels were consistent with the SWAP results. The multiple regression analysis of the four variables included in the model showed the relationship among the risk factors, evaluated globally, and the presence of glaucomatous damage evaluated by RNFL and SWAP (p<0.001). CONCLUSIONS: The usefulness of this probability model as predictive indicator of subsequent glaucomatous damage is based on the good correlation between the risk scale and the RNFL and SWAP assessments. These techniques evaluate structural (RNFL) and functional (SWAP) changes in early stages of the glaucomatous disease.

[Experimental comparative study of the anti-inflammatory effectiveness of pranoprofen]. / Estudio comparativo experimental de la capacidad antiiflamatoria del pranoprofeno.

PURPOSE: To study the antiinflammatory capacity of topical pranoprofen in comparison with other ophthalmological nonsteroidal antiinflammatory drugs (diclofenac and flurbiprofen) in albino rabbits. METHODS: We have produced an endotoxin-induced uveitis by intravitreal injection of 10 ng of Salmonella typhimurium endotoxin. We have used 48 albino rabbits (4 groups of 12 animals each), first group were control group (intravitreal saline solution), which were treated unilaterally every two hours with 2 drops of: Group II, pranoprofen (1 mg/ml). Group III, diclofenac (0.2 mg/ml). Group IV, flurbiprofen (0.3 mg/ml). We determined clinical signs of inflammation and protein concentration in the aqueous humor. The rabbits were sacrificed 24 hours after the endotoxin administration. RESULTS: In all treated groups we observed a significant reduction (p<0.05) in all studied parameter as compared with control group, except for ciliar hyperemia. Different treatment groups did not show differences. CONCLUSION: The antiinflammatory capacity of pranoprofen is comparable to other NSAIDs derived from propionic acid such as flurbiprofen, obtaining a good control of endotoxin-induced uveitis.

[Antiinflammatory capacity of topical ketorolac in experimental model of ocular inflammation]. / Capacidad antiinflamatoria del Ketorolaco tópico en un modelo experimental de inflamación ocular.

PURPOSE: We studied the antiinflammatory effect of topical Ketorolac-Tromethamine on a model of endotoxin-induced uveitis in albino rabbits. METHODS: Endotoxin-induced uveitis was produced by intravitreal injection in the right eye of 10 ng lipopolysaccharide (LPS) A Salmonella typhimurium endotoxin in 5 microl saline solution. We have used 60 animals (5 groups of 12 animals each). Control group (G-I) was injected with saline (5 microl); endotoxin group (G-II) was injected with 10 ng of endotoxin; groups III, IV and V were injected with the same amount of endotoxin and treated with topical ketorolac-tromethamine every 6, 4 and 2 hours respectively. The animals were sacrificed 24 hours after endotoxin administration. We determined the ocular clinical signs and inflammatory cells and protein concentration in the aqueous humor. RESULTS: In all the groups treated with Ketorolac-Tromethamine we observed a significant reduction (p<0.05) in all parameters studied when compared with those of the endotoxin group (G-II). CONCLUSION: Topical Ketorolac-Tromethamine has demonstrated a significant reduction of endotoxin-induced-uveitis inflammation.

[The Autocad system for planimetric study of the optic disc in glaucoma: technique and reproducibility study]. / Sistema Autocad para el estudio planimétrico de la papila óptica en el glaucoma: técnica y estudio de reproducibilidad.

PURPOSE: To develop a lens planimetry technique for the optic disc using AutoCAD. To determine variability magnitude of the optic disc morphological measurements. METHODS: We employed AutoCAD R.14.0 Autodesk: image acquisition, contour delimitation by multiple lines fitting or ellipse adjustment, image sectorialization and measurements quantification (optic disc and excavation, vertical diameters, optic disc area, excavation area, neuroretinal sector area and Beta atrophy area). Intraimage or operator and interimage o total reproducibility was studied by coefficient of variability (CV) (n=10) in normal and myopic optic discs. RESULTS: This technique allows to obtain optic disc measurement in 5 to 10 minutes time. Total or interimage variability of measurements introduced by one observer presents CV range from 1.18-4.42. Operator or intraimage measurement presents CV range from 0.30-4.21. Optic disc contour delimitation by ellipse adjustment achieved better reproducibility results than multiple lines adjustment in all measurements. CONCLUSIONS: Computer assisted AutoCAD planimetry is an interactive method to analyse the optic disc, feasible to incorporate to clinical practice. Reproducibility results are comparable to other analyzers in quantification optic disc morphology. Ellipse adjustment improves results in optic disc contours delimitation.

[Short-wavelength automated perimetry and retinal nerve fiber layer evaluation in glaucoma suspects]. / Correspondencia entre la perimetría automatizada de longitud de onda corta y la capa de fibras nerviosas de la retina en sospechosos de glaucoma.

PURPOSE: To determine the correlation between the structural changes in the Retinal Nerve Fiber Layer (RNFL) and functional losses detected with ShortWavelength Automated Perimetry (SWAP) in a population of patients with suspected glaucoma. METHODS: 49 eyes of 49 ocular hypertensive subjects who met the selection criteria (intraocular pressure greater than 21 mm of Hg and normal standard visual fields) were studied. SWAP was performed with a modified Humphrey Field Analyzer. Visual field indexes (MD, CPSD) were calculated for SWAP. Semiquantitative RNFL scores were given separately to diffuse and localized defects of the RNFL. RESULTS: The MD increased significantly (p<0.001) with higher Diffuse and Total RNFL scores, with good correlation coefficients. A small correlation was found between the CPSD and the RNFR scores (Diffuse, Total and Localized RNFL scores). CONCLUSIONS: Diffuse retinal nerve fiber layer losses are associated with diffuse field loss (MD) while focal structural damage showed no correlation with visual field losses.

[Perimetric probability maps for short wavelength automated perimetry]. / Mapas probabilísticos de puntos diferenciales aplicables a la perimetría automatizada de longitud de onda corta.

PURPOSE: To develop a statistical visual field analysis model (ANESTCAV) suitable for short-wavelength automated perimetry (SWAP), from the probability maps (PM). METHODS: 128 eyes of 128 subjects without ophthalmic pathology were considered. A SWAP test was performed with a modified Humphrey visual field analyzer. To determine the PM (1) we developed a <> (CVNR) corrected for age, (2) we assessed the differences between the CVNR and the visual field of each subject, and (3) from the distribution of the deviations, we determined the confident interval with 4 levels of statistical signification (95, 98, 99 and 99.5%) at each point of the visual field. RESULTS: A regression analysis calculates the <> (VNR) corrected for age at each point of the visual field. The 74 VNR of the visual field constitutes the CVNR corrected for age. Each point has a confident interval for each level of signification which determines the range and probability of value to be normal. CONCLUSIONS: The development of ANESTCAV obtained a precise instrument for perimetric analysis which establishes the ranges of normal results in these exploratory procedures.

[Variability of sensitivity thresholds in short-wavelength automated perimetry (SWAP) in the central vision field]. / Variabilidad de los umbrales de sensibilidad en la perimetría automatizada de longitud de onda corta (PALOC) en las porciones centrales del campo visual.

PURPOSE: To determine the distribution of sensitivity thresholds of short-wavelength automated perimetry (SWAP) in the central visual field, analysing their variability depending on the eccentricity, in normal subjects. METHODS: 128 eyes of 128 normal subjects were analyzed. They underwent a SWAP examination. A lineal regression model was used to establish a <> (SVF). The SVF determines the threshold distribution and its variability. RESULTS: The regression analysis obtained a SVF, age-corrected, which considers the influence of age in the different points of the visual field. The threshold distribution showed higher inter-subject deviations as eccentricity increased (p<0.001). CONCLUSIONS: The determination of variability of the threshold sensitivity deviations in the different points of the central visual field allows future development of perimetric algorythms for SWAP.

[Short-wavelength automated perimetry (SWAP) in subjects with suspected glaucoma (I): assessment of sensitivity thresholds]. / Perimetría automatizada de longitud de onda corta (PALOC) en sujetos sospechosos de glaucoma (I): Evaluación de los umbrales de sensibilidad.

PURPOSE: To evaluate the threshold values of short-wavelength-sensitive (SWS, or S-cone pathways) mechanisms, throughout the 30 degrees central visual field, in a glaucoma suspect population, and to compare the results with those obtained in the Retinal Nerve Fiber Layer study. METHODS: We studied the central visual field from 288 eyes of 211 subjects classified as ocular hypertensives (160 eyes) or age-matched normal controls (128 eyes). A modified Humphrey Field Analyser was used to isolate and measure the sensitivity of the SWS mechanisms. Monochromatic red-free photographs (RNFL) were taken with a wideangle fundus camera at the same period. RESULTS: Comparing normal and OHT eyes, we found significant differences in sensitivity for SWS cone pathways in the superior and inferior nasal quadrants, paracentral and peripheral region, and in the superior hemifield (p<0.05). Based on the perimetric criteria described, the SWAP was pathological in 57 cases. which represents 35.6% of the total sample. Using RNFL as early glaucoma diagnostic criterion, SWAP sensitivity and specificity values were 62% and a 89% respectively. CONCLUSIONS: SWAP is a useful test for the early detection of visual field losses. It is more sensitive than, standard automated perimetry and gives a high correlation with RNFL assessment, which has proved capable of detecting signs of glaucomatous damage several years before the onset of the typical visual field defects.

[Glaucomatous damage pattern in swap in the early stages of glaucoma]. / Patrón de daño campimétrico en la perimetría automatizada de longitud de onda corta (PALOC) en las fases iniciales del glaucoma.

PURPOSE: To determine with Short-Wavelength Automated Perimetry (SWAP) the patterns of early glaucomatous damage and to compare frequency of diffuse defects to localized defects. METHODS: One hundred and sixty eyes from 83 ocular hypertensive subjects who met the selection criteria (intraocular pressure greater than 21 mm Hg and normal standard visual fields) were studied. SWAP was performed with a modified Humphrey Field Analyser. Total (TD) and pattern deviation (PD) probability maps were calculated to evaluate the pattern of visual field loss. RESULTS: The frequency of defects was higher in TD than in PD for all levels of defects (p<0.001). There was also a relative topographical distribution of glaucomatous defects. CONCLUSIONS: We documented the existence of predominantly diffuse visual field defects for all depth levels at early glaucoma stages.

[Histopathological evaluation of vitamin E derivatives effects on the healing process of a model of filtration surgery]. / Evaluación histopatológica de la acción de derivados de la vitamina E en el proceso cicatricial de un modelo de cirugía filtrante.

PURPOSE: To describe the effect of alpha-tocopherol derivatives (acetate and acid succinate) on the histopathological characteristics of the surgical fistulae in an experimental model of filtering surgery. METHODS: 30 pigmented rabbits were randomly divided into 3 study groups. Twenty-four hours before surgery the animals were injected subconjunctivally with 0.5 ml of solution: group I (n=10) 0.75% ethanol in balanced saline solution (BSS), group II (n=10) 100 microgrs alpha-tocopherol acetate (AT) in 0.75% ethanol in BSS, group III (n=10) 100 microgrs alphatocopherol acid succinate (ST) in 0.75% ethanol in BSS. Histological findings were evaluated 30 days after surgery. RESULTS: The groups treated with alpha-tocopherol derivatives showed a higher percentage of fistular persistence and smaller wound healing areas. There was a subconjunctival cellular infiltrate of mononuclear and eosinophilic cells. CONCLUSIONS: Histological findings support the clinical use of alpha tocopherol derivatives, AT and ST. to inhibit the wound healing processes associated to filtering surgery failure.

[Retinal vasculopathy in systemic lupus erythematosus: a case of lupus vasculitis and a case of non-vasculitis venous occlusion]. / Vasculopatía retiniana en el lupus eritematoso sistémico: caso de vasculitis lúpica y caso de oclusión venosa no vasculítica.

CLINIC CASE: Two patients with systemic lupus erythematosus presented with vision loss, and were diagnosed with retinal vasculopathy. Patient 1 had occlusive vasculitis with macular oedema and retinal ischaemia in the right eye. Corticosteroid therapy was increased and intravenous rituximab added. Intravitreal therapy and panretinal photocoagulation were performed. Patient 2 presented with a left central retinal vein occlusion without vasculitis but was on anticoagulation therapy due to having an antiphospholipid syndrome. Both patients maintained a stable visual acuity. DISCUSSION: Occlusive lupus retinal vasculitis has severe visual and systemic consequences (central nervous system vasculitis). It is crucial to differentiate it from standard vascular occlusion syndromes.