Ординатура / Офтальмология / Учебные материалы / Retinal Vascular Disease Joussen Springer

choroidal infiltrates associated with systemic sarcoidosis may occur in eyes remarkably free of other signs of inflammation. The infiltrates tend to radiate from the region of the optic nerve in a confluent ameboid-like pattern. They generally respond to corticosteroids and may be the first recognized manifestation of systemic sarcoidosis [13].

25 III Choroidal circulatory disturbances have been observed in sarcoidosis in the absence of visual symptoms or retinal lesions [41]. Indocyanine angiography in patients with ocular sarcoidosis may show features of choroidal ischemia [73].

Choroidal granulomas may exist without signs of anterior chamber or vitreous inflammation or intracranial granulomas [8]. They are observed in 5 % of patients with ocular sarcoid. They are typically bilateral, pale yellow, elevated lesions that resolve spontaneously or with oral corticosteroids. They may recur but good vision is usually maintained. Optic disk granuloma may be the presenting sign of ocular sarcoidosis (Fig. 25.5.10) [9]. It may mimic papilledema or papillitis but is usually unilateral and responds well to steroids. Vision may or may not be affected. They respond to systemic steroids. Granuloma of the retina has rarely been reported in sarcoidosis [3].

Retinal pigment epithelial detachment has also been noted in sarcoidosis [6]. Serous retinal detachment at the macula may occur in association with pulmonary angiitis secondary to sarcoidosis [71]. The underlying mechanisms of subretinal exudation are thought to include choroidal vascular perfusion and permeability changes, which result in increased choroidal interstitial fluid with further extension into the subretinal space.

Other less frequent manifestations include posterior scleritis with annular ciliochoroidal detachment causing angle closure glaucoma [19].

Neurological manifestations of sarcoidosis are varied and are identified in 12 % of patients with systemic sarcoidosis [67]. Posterior segment involvement is associated with neurological disease in 27 % of cases. Neurological manifestations may include optic nerve disease, cranial nerve palsies, encephalopathy, and disorders of the hypothalamus and pituitary gland. Chiasmal syndromes and motility disorders may also occur. Facial nerve palsy is the most common manifestation of CNS involvement and is usually self limiting. Optic nerve involvement may be caused by direct sarcoid tissue infiltration or compression by cerebral mass or chronic meningitis or secondary to posterior segment involvement. Usually, the involvement of the optic nerve is an indication for systemic treatment. Diagnosis is often difficult due to the fact that the clinical presentation can mimic other disorders, such as multiple sclerosis. Sarcoidosis is also an important differential diagnosis in uveomeningeal syndromes [7].

25.5.5.3 Ocular Sarcoidosis in Children

Sarcoidosis is relatively rare in children and is an uncommon cause of childhood uveitis. The disease may run in families suggesting possible genetic and environmental etiology. There are two age groups of pediatric sarcoidosis. Children under 4 years of age are characterized by the triad of rash, uveitis, and tenosynovitis. The more frequent is the occurrence of sarcoidosis in older children (8 – 15 years of age),

which is characterized by almost universal lung involvement and manifestations in eyes, skin, liver, and spleen. The diagnosis of sarcoidosis in children with uveitis should be suspected when the uveitis presents with red eyes and there are keratic precipitates of the mutton-fat type, when there is anterior and posterior segment involvement or when the tenosynovitis affects multiple joints. The prognosis for children is more favorable than for adults [33].

25.5.6 Diagnosis of Sarcoidosis

A fully directed clinical history and examination is mandatory. The diagnosis is established when clinical and radiographic findings are supported by histologic evidence of non-caseating epithelioid cell granulomas found on tissue biopsy. However, the initial investigations may be negative and it may not be possible to demonstrate a non-caseating granuloma. The common investigations include serum angiotensin converting enzyme (S-ACE) and chest radiography.

25.5.6.1Serum Angiotensin Converting Enzyme

S-ACE is thought to correspond with macrophage activity. It catalyzes the conversion of angiotensin I to angiotensin II. S-ACE is used as both a diagnostic and prognostic indicator of sarcoidosis. It is elevated in 50 – 80 % of patients with active sarcoidosis but can also be elevated in other disorders. In approximately 20 – 50 % of patients, sarcoidosis can be present without elevated ACE levels [4]. This may be due to the fact that the disease is in its early phase, chronic stage or quiescent (burnt out) stage. The absolute value has no prognostic significance. The combination of raised S-ACE levels with abnormal gallium scanning is a specific and sensitive tool for diagnosing patients suspected of having ocular sarcoidosis who had normal chest radiographs [50].

25.5.6.2 Chest Radiographs

A staging system is used to classify chest X-rays taken to detect sarcoidosis (Table 25.5.2). These stages do not correlate with disease severity and patients up to stage 3 may be symptomless.

25.5.7 Clinical Course and Prognosis

25.5.7.1 Ocular Disease

Pure ocular sarcoidosis has a relatively benign course, with 72 % maintaining good vision. In a

Table 25.5.2. Staging of X-ray chest in sarcoidosis

[74]astinal and hilar lymph nodes

Panda sign indicates increased uptake in lacrimal and parotid glands

series of 75 patients with ocular sarcoidosis, panuveitis and peripheral multifocal choroiditis were reported to be the main causes of visual loss and accounted for 47 % of patients [40]. Visual morbidity may be due to secondary glaucoma, CME and optic nerve damage. Poor visual prognosis is associated with advancing age, black race, female sex, chronicity of disease, posterior segment involvement and complications of uveitis [66].

25.5.7.2 Systemic Disease

glaucoma therapy may require trabeculectomy ±5- fluorouracil or mitomycin C.

The surgical interventions for the complications of ocular sarcoidosis are performed when the eyes are quiet. The inflammation must be controlled preoperatively and surgery performed under steroid cover in patients with posterior segment disease or history of CME. Intraocular lens (IOL) implantations are well tolerated and good visual results are achieved. The major causes of the decreased visual acuity following cataract surgery are CME, preexisting glaucomatous damage and posterior segment involvement [47]. Rarely, IOL placement after cataract extraction results in inflammatory membrane formation despite anti-inflammatory coverage.

In the absence of capillary closure, retinal neovascularization may regress spontaneously or with systemic anti-inflammatory treatment. Cases with persistent neovascularization benefit from laser treatment when ischemia is present [26]. Laser photocoagulation should be performed when the inflammation is quiescent to prevent the development or exacerbation of cystoid macular edema.

Vitrectomy may be required for cases with persistent vitreous hemorrhage, residual vitreous opacities or retinal detachment.

25.5.9 Treatment of Systemic Sarcoidosis

Corticosteroids are the mainstay of treatment to suppress severe symptoms (e.g., dyspnea, arthralgia, fever) or hepatic insufficiency, cardiac arrhythmias, CNS involvement, hypercalcemia and disfiguring skin infections. Patients with acute disease may need treatment for only a few weeks, but most of those requiring therapy have chronic sarcoidosis and need treatment for years. Maintenance doses of oral prednisolone as low as 5 mg/day may be needed indefinitely to control symptoms and radiological lesions.

About 10 % of patients requiring therapy are unresponsive to tolerable doses of corticosteroid and may need immunosuppressive drugs such as cyclosporin. Although these drugs are often more effective in refractory cases, relapse is frequent after their cessation.

The treatment of chronic neurological symptoms with methotrexate or cyclophosphamide was associated with a better therapeutic response than the treatment with corticosteroids alone. The long-term prognosis of neuro-ophthalmic sarcoid has not been studied in large patient populations, but the data that is available suggests that remission may occur in up to 47 % [12].

25.5.10 Conclusion

Ocular sarcoidosis may present with a wide variety of ocular signs in all parts of the eye and may be associated with either acute or chronic and progressive intraocular inflammation leading to visual deterioration. The diagnosis may be difficult owing to the absence of diagnostic criteria and the variety of presentations. Treatment is aimed at controlling the inflammatory process and it may require the use of topical, periocular or oral steroids plus immunosuppressive agents. In most patients the long term outlook for vision is good.

Case Reports

Case 1

A 70-year-old Caucasian lady presented with blurred vision and floaters associated with recent weight loss and lethargy of 3 months duration. Examination revealed vitreous cells and peripheral multifocal choroiditis. Vision was 6/12 mainly due to associated CME. A chest radiograph revealed bilateral hilar lymphadenopathy and investigations showed a raised S-ACE. Lung function tests showed reduced transfer factor. The patient received oral corticosteroids for both eye and systemic disease that resulted in partial resolution of her symptoms and signs with improvement of vision to 6/9. She is also under the joint care of the chest physicians and is on a maintenance dose of oral steroids.

Case 2

A 42-year-old black gentleman with cutaneous sarcoidosis on hydroxychloroquine developed floaters in both eyes for the previous 6 months. Examination revealed a visual acuity of 6/6 bilaterally. Examination showed perivenous sheathing, and small areas of equatorial retinal neovascularization and small choroidal lesions resembling Dalen Fuchs nodules. Fluorescein angiography showed no peripheral vascular closure. Prompt oral steroids led to involution of these vessels and laser photocoagulation was not indicated.

References

1.Akova YA, Foster CS (1994) Cataract surgery in patients with sarcoidosis-associated uveitis. Ophthalmology 101(3):473 – 9

2.American Thoracic Society (1999) Statement on sarcoidosis. Am J Respir Cri Care Med 160(2):736 – 755

3.Augustin AJ, Boker T, Seewald S, et al. (1994) Solitary retinal granuloma as a presenting sign of sarcoidosis. Ger J Ophthalmol 3:71 – 72

7.Brazis PW, Stewart M, Lee AG (2004) The uveo-meningeal syndromes. Neurologist 10(4):171 – 84

8.Campo RV, Aaberg TM (1984) Choroidal granuloma in sarcoidosis. Am J Ophthalmol 97(4):419 – 27

9.Castagna I, Salmeri G, Fama F, et al. (1994) Optic nerve granuloma as first sign of systemic sarcoidosis. Ophthalmologica 208(4):230 – 2

10.Cheung CM, Durrani OM, Stavrou P (2002) Peripapillary choroidal neovascularisation in sarcoidosis. Ocul Immunol Inflamm 10(1):69 – 73

11.Collison JMT, Miller NR, Green WR (1986) Involvement of orbital tissues by sarcoid. Am J Ophthalmol 102:302 – 307

12.Constantino T, Digre K, Zimmerman P (2000) Neuro-oph- thalmic complications of sarcoidosis. Semin Neurol 20(1): 123 – 37

13.Cook BE Jr, Robertson DM (2000) Confluent choroidal infiltrates with sarcoidosis. Retina 29(1):1 – 7

14.Cook JR, Brubaker RF, Savell J (1972) Lacrimal sarcoidosis treated with corticosteroids. Arch Ophthalmol 88(5):513 – 7

15.Crystal RG, Bitterman PB, Rennard SI, et al. (1984) Interstitial lung diseases of unknown cause. Disorders characterized by chronic inflammation of the lower respiratory tract. N Engl J Med 310(4):235 – 44

16.Di Alberti L, Piattelli A, Favia G, et al. (1997) Human herpesvirus 8 variants in sarcoid tissues. Lancet 350:1655 – 1661

17.Dick DJ, Newman PK, Richardson J, et al. (1988) Acute posterior multifocal placoid pigment epitheliopathy and sarcoidosis. Br J Ophthalmol. 72(1):74 – 7

18.Dios E, Saornil MA, Herreras JM (2001) Conjunctival biopsy in the diagnosis of ocular sarcoidosis. Ocul Immunol Inflamm 9(1):59 – 64

19.Dodds EM, Lowder CY, Barnhorst DA (1995) Posterior scleritis with annular ciliochoroidal detachment. Am J Ophthalmol 120(5):677 – 9

20.Duker JS, Brown GC, McNamara JA (1988) Proliferative sarcoid retinopathy. Ophthalmology 95(12):1680 – 6

21.Dursun D, Akova YA, Bilezikci B (2004) Scleritis associated with sarcoidosis. Ocul Immunol Inflamm 12(2):143 – 8

22.Faller M, Purohit A, Kennel N, et al. (1995) Systemic sarcoidosis initially presenting as an orbital tumour. Eur Respir J 8(3):474 – 6

23.Franchescetti A, Babel J (1949) La chorio-retinie en ’taches de bougie’, manifestation de la maladie de Besnier-Boeck. Ophthalmologica 118:701 – 710

24.Furuya K, Yamaguchi E, Itoh A, et al. (1996) Deletion polymorphism in the angiotensin I converting enzyme (ACE) gene as a genetic risk factor for sarcoidosis. Thorax 51(8): 777 – 80

25.Geggel HS, Mensher JH (1989) Cicatricial conjunctivitis in sarcoidosis: recognition and treatment. Ann Ophthalmol 21(3):92 – 4

26.Graham EM, Stanford MR, Shilling JS, et al. (1987) Neovascularisation associated with posterior uveitis. Br J Ophthalmol 71(11):826 – 33

granulomatous inflammation: From sarcoidosis to tuberculosis. Semin Respir Infect 18(1):23 – 32

49.Pietinalho A, Ohmichi M, Löfroos AB, et al. (2000) The prognosis of pulmonary sarcoidosis in Finland and Hokkaido, Japan. A comparative five year study of biopsy-prov- en cases. Sarcoidosis Vasc Diffuse Lung Dis 17:158 – 166

50.Power WJ, Neves RA, Rodriguez A, et al. (1995) The value of combined serum angiotensin-converting enzyme and gallium scan in diagnosing ocular sarcoidosis. Ophthalmology 102(12):2007 – 11

51.Rothova A (2000) Ocular involvement in sarcoidosis. Br J Ophthalmol 84:110 – 116

52.Rothova A, Alberts C, Glasius E, et al. (1989) Risk factors for ocular sarcoidosis. Doc Ophthalmol 72:287 – 296

53.Rothova A, Lardenoye C (1998) Arterial macroaneurysms in peripheral multifocal chorioretinitis associated with sarcoidosis. Ophthalmology 105(8):1393 – 7

54.Rybicki BA, Maliarik MJ, Major M, et al. (1997) Genetics of sarcoidosis. Clin Chest Med 18:707 – 717

55.Rybicki BA, Maliarik MJ, Poisson LM, et al. (1997) Racial differences in sarcoidosis incidence: a 5-year study in a health maintenance organization. Am J Epidemiol 145(3): 234 – 41

56.Sekiya M, Ohwada A, Miura K, et al. (2003) Serum vascular endothelial growth factor as a possible prognostic indicator in sarcoidosis. Lung 181(5):259 – 65

57.Sharma OP (1972) Cutaneous sarcoidosis: clinical features and management. Chest 61(4):320 – 5

58.Sharma OP (1996) Vitamin D, calcium, and sarcoidosis. Chest 109:535 – 539

59.Sharma OP (1997) Cardiac and neurologic dysfunction in sarcoidosis. Clin Chest Med 18(4):813 – 25

60.Sherman JL, Stern BJ (1990) Sarcoidosis of the CNS: comparison of unenhanced and enhanced MR images. AJNR Am J Neuroradiol 11(5):915 – 23

61.Shetty AK, Zganjar BE, Ellis GS, Jr, et al. (1999) Low-dose methotrexate in the treatment of severe juvenile rheumatoid arthritis and sarcoid iritis. J Pediatr Ophthalmol Strabismus 36(3):125 – 8

62.Siltzbach LE, Geraint James D, Neville E, et al. (1974) Course and prognosis of sarcoidosis around the world. Am J Med 57:847 – 852

63.Somoskovi A, Zissel G, Seitzer U (1999) Polymorphisms at position –308 in the promoter region of the TNF-alpha and in the first intron of the TNF-beta genes and spontaneous and lipopolysaccharide-induced TNF-alpha release in sarcoidosis. Cytokine 11(11):882 – 7

64.Sonoda KH, Enaida H, Ueno A, et al. (2003) Pars plana vitrectomy assisted by triamcinolone acetonide for refractory uveitis: a case series study. Br J Ophthalmol 87(8):1010 – 4

65.Stanbury RM, Graham EM, Murray PI (1995) Sarcoidosis. Int Ophthalmol Clin 35(3):123 – 37

66.Stavrou P, Linton S, Young DW, et al. (1997) Clinical diagnosis of ocular sarcoidosis. Eye 11(3):365 – 70

67.Stern BJ, Krumholz A, Johns C, et al. (1985) Sarcoidosis and its neurological manifestations. Arch Neurol 42(9):909 – 17

68.Tahmoush AJ, Amir MS, Connor WW, Farry JK, Didato S, Ulhoa-Cintra A, Vasas JM, Schwartzman RJ, Israel HL, Patrick H (2002) CSF-ACE activity in probable CNS neurosarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 19(3):191 – 7

69.Takada K, Ina Y, Noda M, et al. (1993) The clinical course and prognosis of patients with severe, moderate or mild sarcoidosis. J Clin Epidemiol 46(4):359 – 66

70.Verleden GM, du Bois RM, Bouros D, et al. (2001) Genetic

predisposition and pathogenetic mechanisms of interstitial lung diseases of unknown origin. Eur Respir J 18:Suppl 32:17s–29s

71.Watts PO, Mantry S, Austin M (2000) Serous retinal detachment at the macula in sarcoidosis. Am J Ophthalmol 129(2):262 – 4

72.Winterbauer RH, Lammert J, Selland M, et al. (1993) Bronchoalveolar lavage cell populations in the diagnosis of sarcoidosis. Chest 104(2):352 – 61

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73.Wolfensberger TJ, Herbort CP (1999) Indocyanine green angiographic features in ocular sarcoidosis. Ophthalmology 106 (2):285 – 9

74.Yoshikawa K, Kotake S, Ichiishi A, et al. (1995) Posterior sub-Tenon injections of repository corticosteroids in uveitis patients with cystoid macular edema. Jpn J Ophthalmol 39(1):71 – 6

III 25

Core Messages

Systemic necrotizing vasculitides are rare, affecting fewer than 1 in 10,000

Wegener granulomatosis, polyarteritis nodosa (PAN), and Churg-Strauss syndrome (CSS) are histologically distinct, but overlap clinically Pathogenesis is linked to anti-neutrophil cytoplasmic antibodies plus environmental and genetic factors

Ocular manifestations are more common in Wegener granulomatosis and may be localized; retinal vasculitis is rare in all three vasculitides Multidisciplinary management is the rule Treatment is usually initiated with corticosteroids and cyclophosphamide

Mortality lessens with treatment but is higher than in the general population

25.6.1Polyarteritis Nodosa and Microscopic Polyangiitis

25.6.1.1 Synonyms and Related Conditions

Periarteritis nodosa

Microscopic polyarteritis

Small vessel vasculitis

Kawasaki disease

Classic polyarteritis nodosa (PAN) can be further subdivided into that which is hepatitis B related (10 %). Other viruses, such as HIV, CMV, or hepatitis C, have also been associated with PAN. Prevalence is about 4 cases per million.

25.6.1.2 Histopathology

Focal necrotizing arteritis

Mixed cellular infiltrate in the vessel wall Microaneurysm formation

Intraorgan hemorrhage from ruptured microaneurysms

Classic PAN typically involves small and mediumsized muscular arteries with a focal necrotizing arterial inflammation and microaneurysm formation at arterial branch points; the relevance of the classic form of the disease to retinal vascular disease per se is therefore uncertain, although it is the diagnosis typically associated with non-granulomatous vasculitis affecting the eye. Microscopic polyangiitis (MPA), in contrast, denotes vasculitis in arterioles,

venules, and capillaries, and is potentially more relevant to eye disease [22]. Older literature did not separate PAN and MPA and may be confusing.

25.6.1.3 Systemic Course of PAN

Neuropathy (mononeuritis multiplex) Nephropathy

Cutaneous ulcers

Gastrointestinal thrombosis and infarction Musculoskeletal pain

Coronary arteritis CNS involvement

PAN has a systemic onset with fever, malaise, and weight loss, followed by organ specific manifestations listed above in approximate order of frequency. Most patients are middle aged. PAN affects renal function by a vascular nephropathy. MPA uniquely causes glomerulonephritis and pulmonary disease by virtue of its involvement of smaller vessels [26]. Factors associated with a poor prognosis are increased serum creatinine, proteinuria, cardiomyopathy, CNS involvement, and gastrointestinal involvement. Presence of any of these factors is probably an indication for treatment with cyclophosphamide as well as corticosteroids [15]. With the use of cyclophosphamide, 5 year survival is about 80 %.

Kawasaki disease (acute febrile mucocutaneous lymph node syndrome) in children is associated with coronary artery vasculitis and is indistinguishable from infantile PAN histologically; clinically, muco-

cutaneous involvement is necessary to diagnose Kawasaki disease.

25.6.1.3.1 Diagnosis

General laboratory tests including complete blood count and C-reactive protein

Chemistry and urinanalysis to detect glomerulonephritis (MPA)

Hepatitis B surface antigen only in PAN (order cryoglobulins if HepB SAg+) P-ANCA/C-ANCA: anti-myeloperoxidase and anti-serine proteinase 3 (MPA)

Chest X-ray to detect pulmonary infiltrate (MPA) Nerve or muscle biopsy of clinically affected tissue Abdominal angiography to detect microaneurysms in kidney, liver, and mesenteric artery

25.6.1.4 Ocular Manifestations of PAN

Microangiopathy with cotton-wool patches Serous retinal detachment

Retinal vasculitis Artery occlusion

Ischemic optic neuropathy

There are no large series of ocular manifestations of PAN or MPA. Case reports indicate that retinal vasculitis, microangiopathy (cotton-wool spots), and retinal detachment can occur. Despite the rarity of involvement, screening eye examinations in patients with PAN or MPA have been recommended.

Ocular involvement has been estimated to occur in 10 – 20 % of patients; some manifestations may be related solely to accompanying hypertension. Serous

retinal detachment occurred in association with scleritis in one case [24] and was attributed to choroidal vasculitis in two siblings [35]. Fibrinoid necrosis of choroidal vessels led to serous retinal detachment in one fatal infantile case [14]. Histologically confirmed involvement of the short ciliary arteries produced monocular blackouts of vision

25 III attributed to choroidal vascular insufficiency in one case; ocular ischemic syndrome was not present [31]. Central retinal artery occlusion, contralateral ischemic optic neuropathy, and multiple wedge-shaped choroidal occlusions occurred in a 70-year-old woman in the setting of symptomatic mononeuritis multiplex; sural nerve and muscle biopsy confirmed PAN [19]. More subtle manifestations of vascular involvement in retina, nerve and choroid were recorded in three case reports published in 2001 [33]. Rosen summarized angiographic findings in PAN in 1968 [32].

Despite the common association of macroaneurysms located at the retinal arteriolar branch points in idiopathic retinal arteriolar macroaneurysms and neuroretinitis (IRVAN) [6], there seems to be no association between IRVAN and PAN. There is a case report of bilateral retinal ischemia and unilateral ophthalmic artery microaneurysm in an infant who died of Kawasaki disease [12].

25.6.2 Churg-Strauss Syndrome (CSS)

25.6.2.1 Synonyms and Related Conditions

Allergic granulomatous angiitis

Granulomatous small-vessel vasculitis

Eosinophilic vasculitis

25.6.2.2 Histopathology

Small necrotizing granulomas contain eosinophils. Necrotizing vasculitis is also typical. Glomerulonephritis, when it occurs, is pauci-immune. The role of the eosinophil in pathogenesis is accepted, but the cause of eosinophil activation is not known and may be T cell mediated [18].

25.6.2.3 Systemic Course of Disease

Asthma is the most common finding in CSS and may persist after clinical remission; cases diagnosed following administration of leukotriene receptor antagonists for asthma may be related to steroid withdrawal [23]. Polyneuropathy and constitutional symptoms of fever, malaise, and weight loss are also common [16]. Skin manifestations (palpable purpura, urticaria, nodules) are more frequent in CSS than PAN/MPA or Wegener granulomatosis. Morbidity

related to joints, sinus, GI, or pulmonary involvement is often seen. Ocular involvement is rare.

25.6.2.3.1 Diagnosis

ACR criteria [29]

Four of six

–Asthma, often adult onset

–Eosinophilia > 10 % in peripheral blood

–Sinusitis

–Pulmonary infiltrates

–Neuropathy

–Biopsy with vasculitis and eosinophils

Like MPA and Wegener granulomatosis, CSS is associated with anti-neutrophil cytoplasmic antibodies. Like MPA, CSS is more likely to display p-ANCA (anti-myeloperoxidase or MPO) than c-ANCA.

25.6.2.4 Ocular Manifestations

Orbital inflammatory disease led to the diagnosis of Churg-Strauss syndrome in two patients reported in 2001 [37]. Fifteen previously reported cases were cited that met the ACR criteria for diagnosis and had ocular involvement. The authors hypothesized that orbital inflammatory disease and ischemic vasculitis were distinct manifestation of CSS, with orbital inflammatory disease carrying a relatively good visual prognosis.

Of the nine reported cases of ischemic vascular involvement reported in the literature, the five tested for ANCA were all positive. Manifestations included three anterior ischemic optic neuropathies, one ischemic optic neuropathy, one branch retinal artery occlusion, one central retinal artery occlusion, one central retinal vein occlusion, and one case of retinal vasculitis. Three of the cases had amaurosis fugax [1, 2, 4, 9, 21, 40].

25.6.3 Wegener Granulomatosis

25.6.3.1 Synonyms and Related Conditions

Wegener disease

Systemic necrotizing angiitis

Necrotizing glomerulonephritis

Wegener granulomatosis typically involves a triad of systemic necrotizing vasculitis (87 %), necrotizing granulomatous involvement of the respiratory tract (69 %), and necrotizing glomerulonephritis (48 %) [27]. Localized forms of disease exist, for example, isolated disease of the eye or orbit. Prevalence is about 8 cases per million.