Ординатура / Офтальмология / Английские материалы / Practical Manual of Intraocular Inflammation_Dick, Okada, Forrester_2008

cyclosporine should be used as steroid-sparing agents in patients with severe disease who are likely to require prolonged treatment (>6 months) and in patients with relative contraindications to corticosteroid therapy such as those with diabetes mellitus

lPrognosis: Visual outcomes good with initiation of adequate treatment in the acute phase

Sympathetic Ophthalmia

Sympathetic ophthalmia (SO) is a disorder closely related to VKH disease, with similar ocular manifestations that occur in individuals with a history of penetrating ocular trauma or intraocular surgery. A prospective study has shown that SO in the modern era is most likely related to previous vitrectomy, particularly for retinal detachment (61) or scleral buckling, and occurs usually within one year of the surgical procedure. SO has been known to occur after skin trauma such as burns and (Nd: YAG) laser iridotomy, although these are rare. Intraocular inflammation is usually more severe in the eye with the history of trauma or surgery (the exciting eye) than in the fellow eye (the sympathizing eye) and can be much more variable when compared to VKH disease. For example, choroidal detachment with mild anterior segment inflammation may be the initial manifestation in a sympathizing eye. Detection of intraocular inflammation in the exciting eye may be difficult to distinguish from postoperative inflammation. Prodromal symptoms similar to those commonly noted in VKH disease may occur, but they are unusual in SO, as are the late skin and hair manifestations of alopecia, poliosis, and vitiligo. However, both SO and VKH disease share a strong association with the HLA-DR4 haplotype (62), and therefore may have a higher incidence in the same ethnic groups in which VKH disease commonly occurs. Early diagnosis can lead to good visual outcomes; however, since SO is a rare disease with great variability in initial manifestations, it is often not suspected in the early stages.

lPresenting symptoms: Red eye, floaters, decreased vision in exciting eye only, the sympathizing eye only, or both

lMedical history/demographic associations: Previous penetrating ocular trauma or surgery

lOcular signs (Fig. 24): Always bilateral, with no one pattern of manifestations. Anterior: Mild-to-moderate usually granulomatous anterior segment inflammation. Anterior segment inflammation may be the only manifestation of SO. Posterior: Acute stage—mild vitritis, serous retinal detachment, choroidal detachment, multifocal choroidal white lesions, papillitis. Convalescent stage—generalized fundus depigmentation, nummular chorioretinal atrophic scars, geographic chorioretinal

Figure 24 Fundal color photographs demonstrating features of sympathetic ophthalmia. (A) Multiple, large nummular chorioretinal scars are observed in the periphery of the exciting eye of the a patient with sympathetic ophthalmia. (B) Choroidal detachment for 3608 was observed in the sympathizing eye of a different patient. The exciting eye had a history of uneventful scleral buckling for rhegmatogenous retinal detachment.

lPrognosis: Visual outcomes may be good, depending on baseline visual function prior to the onset of SO

Systemic Lupus Erythematosis

Systemic lupus erythematosis (SLE) is a systemic vasculitic disorder characterized by the development of a variety of autoantibodies, most notably various antinuclear antibodies and antiphospholipid antibodies, and circulating immune complexes. The disease is most common in young to middle-aged women who may experience systemic symptoms of fatigue, fever, malaise, and/or arthralgias before ocular symptoms develop. The disease can eventually cause arthritis, glomerulonephritis, pericarditis, pleurisy, various hematological abnormalities

Figure 25 Fundal color photograph and flourescein angiogram of retinal features in SLE. (A) Severe retinal vasculitis involving both veins and arteries. (B) Fluorescein angiography of another patient shows points of dye leakage with pooling in areas of focal retinal detachment.

such as leukopenia and thrombocytopenia, splenomegaly, and numerous central nervous system abnormalities.

lPresenting symptoms: Decreased vision, ocular involvement may be asymptomatic

l Medical history/demographic associations: Adult women

lOcular signs (Fig. 25): When intraocular inflammation is present, it is usually bilateral, albeit frequently asymmetric in severity. Anterior: No anterior segment intraocular inflammation. Posterior: Absence of vitritis; multifocal choroiditis, retinal vasculitis, serous retinal detachment, optic neuropathy. Other: Keratoconjunctivitis sicca, peripheral ulcerative keratitis, scleritis

lSystemic signs: Fever, “butterfly” malar rash, discoid rash, Raynaud’s phenomenon, alopecia, oral ulceration

lWorkup: Elevated erythrocyte sedimentation rate and/or C-reactive protein, and the presence of antinuclear antibodies and antiphospholipid antibodies would support the diagnosis of SLE. Serum creatinine and urinalysis should be examined to screen for renal disease

lTreatment: Successful control of systemic disease using corticosteroids, immunosuppressive drugs, and/or biological agents usually results in control of the intraocular inflammation

l Prognosis: Visual outcomes are relatively good unless optic atrophy ensues

Multiple Sclerosis

Multiple sclerosis (MS) is a demyelinating disease of the central nervous system that usually presents to the ophthalmologist with optic neuritis or internuclear

ophthalmoplegia and other gaze palsies. MS can also cause mild intraocular inflammation, usually in the form of a peripheral retinal periphlebitis. It is unclear whether this feature is associated temporally with the development of optic neuritis. However, periphlebitis is observed in roughly one-quarter of patients with optic neuritis, and patients with optic neuritis in the setting of periphlebitis may have a higher incidence of progression to clinically diagnosed MS (63).

l Presenting symptoms: Floaters, decreased vision

lMedical history/demographic associations: History of neurological abnormalities

lOcular signs: PSII (other than optic neuritis) may be unilateral or bilateral and not dissimilar to many cases with intermediate uveitis. Anterior: Mild anterior segment intraocular inflammation. Posterior: Anterior vitritis, pars planitis, periphlebitis, or venous sheathing in the peripheral retina. Other: Optic neuritis, internuclear ophthalmoplegia, skew deviation, cranial nerve palsies, nystagmus

l Systemic signs: Neurological deficits may be present

lWorkup: Cerebrospinal fluid examination may be positive for oligoclonal bands. Brain MRI may show active plaques in the periventricular regions

lTreatment: Topical and/or periocular injections of corticosteroids are generally adequate to control active intraocular inflammation. Peripheral periphlebitis alone does not require treatment, although more severe posterior segment inflammation may benefit from immunosuppression.

Systemic interferon-b therapy may be used to treat the underlying MS

lPrognosis: Visual outcomes usually good for intraocular inflammation unless optic neuritis occurs

Frosted Branch Angiitis

Frosted branch angiitis is a term used to describe the severe retinal vasculitis, particularly affecting the veins, which can accompany CMV retinitis (64) and many other infections such as tuberculosis and syphilis, as well as presumed autoinflammatory processes such as systemic lupus erythematosus. Regardless of any primary infection, the pathogenesis of frosted branch angiitis is believed to be autoinflammatory in nature.

lOcular signs: Bilateral in roughly three-fourths of patients. Anterior: Mild-to-moderate anterior segment inflammation. Posterior: Mild-to- moderate vitritis, prominent perivascular sheathing, with or without retinal hemorrhages, retinitis consistent with CMV infection, papillitis

104 Chapter 3

Presumed Autoinflammatory Diseases,

Without Systemic Manifestations

The following section describes presumed autoinflammatory ocular disorders that occur in the absence of systemic manifestations. Unlike those with systemic manifestations, these are not necessarily bilateral. There is much overlap and confusion with the clinical delineation of many of these disorders, also referred to collectively as white dot syndromes, complicating research in this field and the ability of clinicians to treat patients. In fact, a definitive diagnosis is probably less important than determining whether sight is under threat. Autoimmunity to retinal antigens has been shown in some of these conditions, however, the relationship with “so-called” autoimmune retinopathy is not clear. Studies in animal models have shown that many of the different clinical syndromes can be mimicked by immunization to a restricted set of retinal or ocular antigens (see chap. 6). For the practicing physician, determination of the threat to sight is the key to good management.

Multifocal Evanescent White Dot Syndrome

MEWDS is an uncommon, transient, unilateral, self-limiting disorder of acute onset usually occurring in young adult women and has a benign natural course. Some patients may recall flu-like symptoms preceding the onset of visual disturbance. The fundus changes in MEWDS appear to be limited to the outer retina and the RPE. Many specialists consider MEWDS to be one presentation within a spectrum of disorders, including acute idiopathic blind spot enlargement (AIBSE), acute zonal occult outer retinopathy (AZOOR), acute annular outer retinopathy (AAOR), and acute macular neuroretinopathy (AMN) (65), and these are described in the next section.

lPresenting symptoms: Photophobia, flashing lights, decreased vision, scotoma

lMedical history/demographic associations: Flu-like illness one to two weeks prior. Most common in women aged 20 to 40 years

lOcular signs: Usually unilateral. Anterior: No anterior segment inflammation. Posterior: Acute phase—mild vitreous cells, multiple white spots with rather indistinct borders at the level of the RPE in the posterior pole to mid-periphery (see Fig. 6; also see Fig. 18 of chap. 2), mild papillitis or optic disc edema. All changes resolve over weeks. Mild retinal vascular sheathing may be present. Convalescent phase—fine granular appearance of the macula. CNV is a rare complication. Other: A relative afferent pupillary defect may be present in the acute phase that resolves completely

l Systemic signs: Usually none

lWorkup: Fluorescein angiography will show early punctate hyperfluorescence with late staining of lesions, with or without mild optic disc staining. Indocyanine green angiography will show hypofluorescence of lesions. Goldmann visual field may reveal an enlarged blind spot and electroretinography (ERG) may show generalized reduced amplitudes,

both changes that resolve

l Treatment: Not necessary unless CNV develops l Prognosis: Usually good

MEWDS-Related Outer Retinopathies (AIBSE, AZOOR, AAOR, AMN)

All of the MEWDS-related outer retinopathies are rare, occur somewhat more commonly in young women, and often show evidence of blind spot enlargement in the acute phase of disease. Similar to MEWDS, all are believed to involve dysfunction of the photoreceptors and RPE. As mentioned above, there is considerable overlap of clinical features of these disorders, and the nomenclature and diagnostic criteria for these disorders remain under considerable debate. Thus, although these entities, including MEWDS, have “names” attached to them, they may merely represent variations on a spectrum of the same disease, albeit with possible differences in triggering etiology. Furthermore, progression of visual loss has been reported, with subsequent control on immunosuppression (66). Therefore, assuming that the pathogenesis involves an autoinflammatory process, immunosuppressive therapy should be considered in patients with progressive disease.

lAIBSE is characterized by symptoms of unilateral photopsia and an enlarged blind spot on visual field testing. Funduscopy may show mild disc hyperemia but may also be entirely normal. Unlike MEWDS, it is said that the visual field abnormality in AIBSE may not recover completely. However, a long-term follow-up study has shown that many cases initially diagnosed as AIBSE can be reclassified later as having

Table 5 Birdshot Chorioretinopathy Diagnostic Criteria for Research Purposes by an International Consensus Conference

aDefined by the Standardization of Uveitis Nomenclature (SUN) Working Group (1). bDefined by Nussenblatt et al. (77).

Abbreviation: HLA, human leukocyte antigen. Source: From Ref. 76.

Birdshot Chorioretinopathy

Birdshot chorioretinopathy is an uncommon bilateral disorder occurring in middle-aged Caucasian adults, and is strongly associated with the HLA-A29 haplotype. The disease is characterized by distinctive creamy-white oval lesions deep to the sensory retina oriented in a radiating pattern from the disc into the periphery, with mild papillitis but usually very little anterior segment or vitreous inflammation. Gradual loss of vision over years occurs due to chronic cystoid macular edema (73) with subsequent macular atrophy. Color vision testing and ERG appear to be useful in monitoring disease progression since clinically observable features and visual acuity are not reliable indicators of disease activity (73–75). CNV may occur as a late complication. An international consensus conference has published research criteria for the diagnosis of birdshot chorioretinopathy (Table 5) (76). The aggressive use of systemic immunosuppressive agents and corticosteroids may prolong visual function to some extent (78); however, overall visual prognosis remains poor for this disease.

l Presenting symptoms: Decreased vision, floaters

lMedical history/demographic associations: Middle-aged or older Caucasian individuals