Exophthalmos in systemic lupus erythematosus

In systemic lupus erythematosus (SLE) exophthalmos is distinctly rare. This article describes the first patients with SLE in whom exophthalmos was preceded by pseudotumor cerebri. The apparent lack of resolution of the exophthalmos with corticosteroids in our case emphasizes the potential need for therapeutic alternatives such as immunosuppressive agents, orbital irradiation, and/or plasmapheresis.     

Ocular manifestations of systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a chronic, immunologic disorder that may affect multiple organ systems. Keratoconjunctivitis sicca is the most common ocular manifestation, but visual morbidity is usually due to retinal and neuro-ophthalmic manifestations of the disease. Ocular manifestations of lupus are a reflection of systemic disease. The presence of ocular manifestations should alert the clinician to the likely presence of disease activity elsewhere. Therefore, all patients with ocular lupus should be carefully evaluated for systemic involvement to detect potentially treatable and preventable complications of the disease. In addition, the ophthalmologist should include SLE in the differential diagnosis of many retinal vascular and neuro-ophthalmic disorders. The ophthalmologist may play an important role in the care of patients with SLE, since ocular inflammatory lesions may precede potentially serious extraocular disease.     

Subretinal neovascularization in systemic lupus erythematosus

Choroidal involvement in systemic lupus erythematosus (SLE) occurs infrequently. We describe a patient with unusual manifestations of lupus oculopathy: prominent choroidal vasculopathy associated with multiple chorioretinal scars and subretinal neovascularization in the macular area. Choroidal disease was the primary feature of ocular involvement. SLE should be considered in the differential diagnosis in patients with inflammatory choroidal lesions.     

Intracranial hypertension in systemic lupus erythematosus

PURPOSE: Intracranial hypertension (IH) mimicking pseudotumor cerebri (i.e., idiopathic IH) has been reported in individuals with systemic lupus erythematosus (SLE) since the 1960s. Although various mechanisms have been proposed (e.g., venous thrombosis, medication side effect, and immunologic or inflammatory disease) none have been proven to be causal. METHODS: Retrospective chart review of patients with IH and SLE at a single tertiary care institution. RESULTS: In a cohort of IIH patients the percentage of patients in our study with SLE was 1% (4 in 410). Three out of the four patients had serologic evidence for a hypercoagulable state but only one had cerebral venous sinus thrombosis. Two patients had onset or worsening of IH in close temporal relationship to steroid withdrawal. The course, treatment, and prognosis of our patients with SLE and intracranial hypertension did not differ significantly from our clinical experience with IIH or with the published natural history data for IIH. CONCLUSIONS: The association of SLE and IH may occur more than by chance alone. Steroid withdrawal in the treatment of the SLE may be a predisposing or precipitating factor in the development of IH in these patients rather than the inflammatory effects of SLE per se. The hypercoagulable state in some patients with SLE may also produce cerebral venous sinus thrombosis as an additional potential mechanism of IH.     

Persistent choroidopathy in systemic lupus erythematosus

We report a case of a rare entity, choroidopathy in association with systemic lupus erythematosus (SLE), previously described in only twelve patients to our knowledge. It manifested by multiple focal serous elevations of the sensory retina and/or serous detachments of the retinal pigment epithelium bilaterally. No retinal vascular changes were observed. The probable pathogenesis is related to choroidal vascular disease due to systemic hypertension secondary to lupus nephritis, vasculitis, or a combination of these.     

Homonymous hemianopia and systemic lupus erythematosus

The case of a 23-year-old woman with systemic lupus erythematosus and homonymous hemianopia is presented. A calcarine cortical infarct occurred as a result of a postpartal exacerbation of her disease and was demonstrated by CT scanning of the brain. The neuro-ophthalmic manifestations of SLE are reviewed briefly. The authors propose to include collagen vascular disease in the differential diagnosis of homonymous hemianopia in the young age group.     

Retinal microangiopathy in systemic lupus erythematosus.

We performed complete ocular examination, including fluorescein angiography of the fundus, in 50 ambulatory patients with moderately active or inactive systemic lupus erythematosus; none had ocular symptoms. One patient had drusen and two patients had increased intraocular pressures with disk cupping attributable to orally administered corticosteroids. No patient had detectable abnormalities by ophthalmoscopic examination. Despite this apparently normal condition, fluorescein angiography revealed single or multiple microaneurysms in nine patients (13 eyes) and retinal capillary dilatations with leakage of fluorescein in six patients (six eyes). Both abnormalities occurred in two patients. These capillary changes in moderately ill patients with lupus erythematosus are probably the residue of previous acute vasculitis, perhaps due to immune-complex deposition.     

Bilateral internuclear ophthalmoplegia in systemic lupus erythematosus

Internuclear ophthalmoplegia has been infrequently described in patients with systemic lupus erythematosus. We report a 23-year-old woman with lupus who presented with bilateral internuclear ophthalmoplegia and skew deviation. Additional neurologic findings included dysarthria, hemifacial weakness, hemiparesis, and dysmetria. Computed tomography of the patient's brainstem was unremarkable while magnetic resonance scanning demonstrated two areas of infarction. Magnetic resonance imaging is superior to computed tomography in both neuroradiographic study of the brainstem as well as evaluation of patients with neurologic complications of lupus.     

Ocular myasthenic syndrome and systemic lupus erythematosus

BACKGROUND: Reports on association of systemic lupus erythematosus (SLE) and myasthenia gravis are sparse. CASE REPORT: An 40-year old woman complained of sudden onset of diplopia. A SLE was known for 6 years. Both eyes showed a deficit in upgaze, the right eye showed a deficit in abduction and a slight ptosis. Further ophthalmologic and neurologic status were normal. Magnetic resonance tomographic imaging of the brain and the results of the spinal fluid examination were unremarkable. Sonographic imaging and magnetic resonance tomographic imaging studies showed normal extraocular muscles. The edrophonium test (short acting inhibitor of acetylcholinesterase) was positive (elevation of the ptotic lid after injection of edrophonium). Acetylcholine receptor antibodies were elevated to 70 nmol/l (norm: < 0.25 nmol/l). Thoracic computed tomographic scan showed no evidence of a thymoma/thymus hyperplasia or thymus carcinoma. Systemic steroid treatment resolved the complaints within weeks. CONCLUSIONS: The patient with SLE had an ocular myasthenic syndrome. The etiology of the association of the two autoimmune diseases is not clear. SLE patients showing fatigue signs or pareses should be examined for myasthenia gravis. Patients with isolated ocular myasthenic syndromes should be examined for a generalized immunological disorder.     

伴发视网膜病变的系统性红斑狼疮患者的临床特征

[目的]观察伴发视网膜病变的系统性红斑狼疮(SLE)患者的临床特征.[方法]采用临床对比研究方法.临床确诊为SLE的97例患者纳入本研究.根据眼底检查结果分为视网膜病变组(阳性组)和无视网膜病变组(阴性组).其中,阳性组23例32只眼,阴性组74例148只眼.对比分析两组患者年龄、病程、临床表现、实验室检查结果的异同.[结果]SLE患者中视网膜病变阳性率为23.7％.阳性组中17例22只眼视网膜可见不同程度的棉绒斑、出血、静脉纡曲扩张、动脉痉挛、微动脉瘤、硬性渗出,占阳性例数的73.9％其余6例10只眼以视网膜大血管阻塞为主要表现,占阳性例数的26.1％.阳性组患者皮疹、皮肤血管炎、血红细胞沉降率(ESR)升高、补体C3下降、抗双链DNA(ds-DNA)抗体阳性的发生率较阴性组患者高,相关指标比较,差异均有统计学意义(x2 =9.206,6.987,7.824,8.581,6.599;P＜0.05).阳性组与阴性组患者之间年龄、病程、黏膜溃疡、关节炎、发热、头痛、白细胞减少、血小板减少、蛋白尿≥+++、血尿素氮升高、肌酐升高、抗核抗体(ANA)阳性、抗Sm抗体阳性的发生率相比较,差异无统计学意义(t=0.321,0.063;x2 =0.135,0.046,0.176,0.002,0.036,0.113,0.053,0.032,0.012,0.000,0.004;P＞0.05).[结论]伴发视网膜病变的SLE患者眼底主要表现为静脉纡曲扩张、棉绒斑和视网膜大血管阻塞.皮疹、皮肤血管炎、ESR升高、补体C3下降、抗ds-DNA抗体阳性是伴发视网膜病变的SLE患者的主要临床特征.     

Bilateral deep keratitis caused by systemic lupus erythematosus

We report the optical and ultrasonic biomicroscopy and confocal microscopy findings in bilateral stromal keratitis (keratoendotheliitis), a rare ocular manifestation of systemic lupus erythematosus (SLE). Examination revealed deposits with polyrefringent crystals. Topical corticosteroid produced regression of the corneal edema, but there was an increase in corneal opacity. Ultrasound biomicroscopy images confirmed the deep location of the corneal opacities, and confocal microscopy showed a disruption of the corneal stroma and crystal-like bodies.