used [39]. Pulsed methylprednisolone and cyclophosphamide have been reported to be effective in treating bilateral exudative retinal detachment secondary to ischemic choroidopathy [27, 40].

Central Nervous System and Neuroophthalmological Involvement

Involvement of the CNS occurs in approximately 39–57% of patients with SLE [19, 41]. However, antiphospholipid syndrome increases the risk of nervous system involvement in up to 78% of patients [41].

Anterior Visual Pathway

While not common, the prevalence of optic nerve disease in a referral rheumatology population can be estimated at approximately 1% of patients with SLE. In general, signs of optic nerve disease include reduced visual acuity, impairment of color vision (dyschromatopsia), diminished light brightness sensitivity, decreased contrast sensitivity, afferent pupillary defect, and visual field defects [19]. The patient may present with acute painless visual loss, an altitudinal or arcuate field defect, and optic disk swelling, the typical picture of anterior ischemic optic neuropathy. The patient may also present with similar symptoms but with a normal-appearing optic disk, i.e., a retrobulbar ischemic optic neuropathy. In some cases, there may be associated orbital pain, and the visual field defect may be a central scotoma; thus, the patient may be believed to have an optic neuritis, a papillitis (Fig. 18.9), or a retrobulbar neuritis depending on whether or not the optic disk is swollen. Both optic neuropathy/neuritis and retinal occlusive disease can result in optic atrophy. The histopathologic appearance of SLE cranial neuropathies is thought to be caused by vaso-occlusive disease in small vessels, with only nerve demyelination in milder forms of ischemic disease and axonal damage and necrosis in more severe cases [42]. Visual prognosis following optic neuropathy is generally poor, although good outcomes have been reported.

Treatment options of SLE optic neuropathy include systemic corticosteroids and immunosuppressive agents such as cyclophosphamide and methotrexate [22, 43]. Some patients may

Fig. 18.9 Swollen optic disk (papillitis) in a systemic lupus erythematosus patient. Note the associated cottonwool spots and retinal hemorrhages (Courtesy of Rafael Muci-Mendoza, M.D.)

need anticoagulation in addition to immunosuppression [27]. Devic’s syndrome (neuromyelitis optica or NMO) often presented with blindness and paraplegia is characterized by severe episodes of optic nerve and spinal cord inflammation. NMO-IgG (anti-aquaporin-4) has been recently described as a sensitive and specific marker for NMO. Testing for the NMO-IgG in cases of isolated or recurrent transverse myelitis attributed to current SLE and APLS may help clarify the diagnosis of a distinct disease process likely to cause recurrent and severe disability, warranting more aggressive immunotherapy [44]. Inflammation and/or thrombosis of medium to small vessels in the central nervous system are thought to underlie the nerve damage in this syndrome. Chiasmal involvement (chiasmopathy or chiasmitis) in SLE may occur in patients with positive antinuclear antibody and elevated anti-double-stranded DNA titers or in absence of any other evidence of systemic activity [45, 46].

Posterior Visual Pathway

Lesions of the posterior visual pathways include pupillary abnormalities such as light near dissociation (reduced pupillary light reflex but preserved

Fig. 18.10 (a) Right and (b) left color pictures of ocular fundus showing optic nerve engorgement due to papilledema in a systemic lupus erythematosus patient. (c) Right

and (d) left optic nerves after intracranial hypertension syndrome resolution

near reflex), Horner’s syndrome, Adie’s pupil, visual disturbance ranging from transient amaurosis fugax to cortical blindness, visual hallucinations unformed (e.g., bright lights, straight lines) or highly formed (e.g., faces), as well as visual field defects and scotomas [19, 47]. These features indicate disease in the posterior cerebral artery circulation [19]. Strokes occur in up to half of the patients and most likely result from cardiac valvular disease, coagulopathy, or antiphospholipid antibody syndrome. Immune complex– mediated cerebral vasculitis is associated with SLE but is uncommon [47].

Oculomotor System

Cranial nerve palsies may result in ophthalmoplegias accompanied by diplopia, ptosis, and/or pupillary abnormalities. The incidence of cranial neuropathy is reported to range from 5% to 42% and is often associated with multiple cranial nerve

involvement [19]. Disorders of conjugate gaze such as internuclear ophthalmoplegia (INO), unior bilateral, and one and a half syndrome (INO with ipsilateral horizontal gaze palsy) are seen [48–50]. INO in SLE is uncommon, affecting <5% of hospitalized SLE patients [48]. Other reported oculomotor complications include nystagmus [51] and Miller Fisher syndrome (ataxia, areflexia, and ophthalmoplegia) [52]. A possible cause of oculomotor palsy in SLE patients is microthrombosis associated with presence of antiphospholipid antibodies [53]. Intracranial hypertension syndrome (characterized by an elevated intracranial pressure, papilledema [Fig. 18.10], and headache with occasional abducens nerve paresis, absence of a spaceoccupying lesion or ventricular enlargement, and normal cerebrospinal fluid chemical and hematological constituents) has been reported [19, 54, 55]. Deschler et al. [54] reported a SLE intracranial hypertension syndrome that did not improve