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Journal Français d'Ophtalmologie
Volume 36, n° 6
pages 526-532 (juin 2013)
Doi : 10.1016/j.jfo.2012.12.004
Received : 7 November 2012 ;  accepted : 5 December 2012
Ocular manifestations of the potentially lethal rheumatologic and vasculitic disorders
Les manifestations oculaires des troubles rhumatologiques et vasculaires potentiellement mortels

Figure 1

Figure 1 : 

Necrotizing scleritis in a patient with rheumatoid arthritis in the absence of active arthritis. This is an ominous signal of occult, underlying, potentially lethal systemic vasculitis.

Figure 2

Figure 2 : 

Peripheral ulcerative keratitis in a patient with rheumatoid arthritis. This too is an ominous sign indicative of potentially lethal occult systemic vasculitis.

Figure 3

Figure 3 : 

Necrotizing scleritis and peripheral ulcerative keratitis in a patient with rheumatoid arthritis. Note the folly of attempts at support from scleral grafting in the absence of systemic control of the underlying disease.

Figure 4

Figure 4 : 

Fundus/retina of a 30-year-old female with systemic lupus erythematosus. Note the extensive retinopathy with vaso-occlusion, infarcts, and intraretinal hemorrhages. All indicative of potentially impending central nervous system lupus crisis.

Figure 5

Figure 5 : 

Fluorescein angiogram of the same patient shown in Fig. 4. Note the late staining in the vasculature, clearly indicative of the widespread retinal vasculitis in this patient.

Figure 6

Figure 6 : 

Peripheral ulcerative keratitis with adjacent scleritis. The eye is painful and tender to the touch. It took many months before the underlying diagnosis of granulomatosis with polyangiitis (Wegener's) was established and effective therapy implemented.

Figure 7

Figure 7 : 

Same patient as shown in Fig. 6. Note the extension of the peripheral ulcerative keratitis.

Figure 8

Figure 8 : 

Same patient as shown in Figs. 6 and 7. The necrotizing lesion on the skin of the cheek has now evolved, still without a clear underlying diagnosis.

Figure 9

Figure 9 : 

Worsening of the ocular lesion in this patient in whom diagnosis was eventually made by histopathology, i.e., granulomatosis with polyangiitis (Wegener's).

Figure 10

Figure 10 : 

Histopathology of biopsied ocular inflammation in previous patient. Note the microangiopathy of lymphocytes clustering around blood vessels.

Figure 11

Figure 11 : 

Same patient, same tissue, different field. Note the array of multinucleated giant cells.

Figure 12

Figure 12 : 

Chest X-ray in the same patient diagnosed with granulomatosis with polyangiitis (Wegener's). The chest X-ray 6 months earlier had been perfectly normal.

Figure 13

Figure 13 : 

Sinus X-ray, same patient. As in Fig. 12, the sinus X-ray 6 months earlier had been perfectly normal. Note now, however, the opacification of the right macullary sinus, typical of a patient with sinus pathology from granulomatosis with polyangiitis (Wegener's).

Figure 14

Figure 14 : 

Same patient as in the previous two figures, 6 months into therapy with systemic cyclophosphamide. Note the complete resolution of the ocular inflammation, and the healing of the peripheral ulcerative keratitis. The patient's visual acuity is 20/20.

Figure 15

Figure 15 : 

Auricular involvement in a patient with relapsing polychondritis. This patient had necrotizing scleritis and peripheral ulcerative keratitis for more than 1 year prior to the evolution of the cartilage pathology in the ear.

Figure 16

Figure 16 : 

An additional patient with relapsing polychondritis. Note, in addition to the scleritis and the saddle nose deformity from cartilage damage to the nose, the fact that the patient has a tracheostomy. This was avoidable, had the doctors involved in her care been aware of the clinical significance of the onset of scleritis in a patient with relapsing polychondritis. More aggressive therapy 6 months earlier would have obviated the need for the tracheostomy.

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