A 42-year-old man was referred due to a 1-month history of redness, ocular pain, and blurred vision in both eyes. His past medical history included acute otitis media complicated with bacterial pneumonia 1 year previous. He later presented with renal failure and arterial hypertension. He also had had diabetes for the past 10 years.
Ophthalmic examination revealed a visual acuity of 20/50 OD and 20/80 OS. There was an immovable, tender, inflamed scleral nodule on the temporal side of the sclera of the right eye associated with diffuse superior scleral inflammation and thinning of the cornea at IX meridian (Fig. ). In the left eye, there were two nodules in the temporal and superior sclera associated with diffuse scleral inflammation (Fig. ). The anterior chamber was deep with 0.5+ cells (Standardization of Uveitis Nomenclature Working Group [20
]) in the OD and no cells in OS. The rest of the examination was normal. The diagnosis of bilateral nodular scleritis and sclerokeratitis in OD was made; because of the clinical and systemic findings, Wegener's granulomatosis was considered, and high-dose steroid treatment was begun after infectious causes were ruled out.
Fig. 1 Right eye. a Slit-lamp photograph with low magnification showing an inflamed, elevated, immobile, scleral nodule in the temporal part of the sclera. b Slit-lamp photograph with ×16 magnification showing diffuse superior scleral inflammation. (more ...)
Fig. 2 Left eye. a Slit-lamp photograph with low magnification showing an inflamed, elevated, immobile, scleral nodule in the temporal part of the sclera. b Slit-lamp photograph with ×16 magnification showing a superior nodular scleritis. c Slit-lamp (more ...)
Laboratory tests included a complete blood count, an erythrocyte sedimentation rate, antinuclear antibodies, liver enzymes, c-ANCA and p-ANCA, CRP, urinalysis, chest x-ray, thoracic CT, VDRL, a fluorescent treponemal antibody absorption test, and a purified protein derivative skin test for tuberculosis. Serum urea nitrogen was 94 mg/dl and serum creatinine was 2.7 mg/dl; antinuclear antibodies were found to be 1:20 and were considered negative. The rest of the laboratory assessment was negative.
Kidney biopsy was performed because both antineutrophil cytoplasmic antibodies (c- and p-ANCA) and antibodies to proteinase-3 and myeloperoxidase were negative, and thoracic CT abnormalities were not found. Pathologic findings consisted of an adequate kidney biopsy (28 glomeruli) with 53
% global glomerulosclerosis, moderate tubular atrophy, and moderate interstitial fibrosis. The rest of the glomeruli showed diffuse proliferative mesangial hypercellularity with an increase of the mesangial matrix. The biopsy did not show endocapillary hypercellularity, crescents, segmental sclerosis, glomerulitis, vasculitis, or necrosis. Glomerular basal membrane thickness was normal. Immunofluorescence demonstrated granular deposits of IgA in a mesangial pattern with strong intensity (4 out of 4 points). Glomerular staining for IgG, IgM, lambda with less intensity (2 out of 4 points), and kappa predominance (3 out of 4 points) were also found. Others markers such as C1q, C3c, C4c, fibrin, and albumin were negative (Fig. ).
a H&E, ×10. Interstitial fibrosis and tubular atrophy. b H&E, ×40. Mesangial hyperplasia. c IF-IgA, ×5. Positive glomeruli and negative interstice. d IF-IgA, ×40. Mesangial and granular pattern
After one monthly pulse of IV cyclophosphamide therapy, the scleral nodules resolved completely, and there was development of mild scleral thinning. At 2 years of follow-up with azathioprine, there are no recurrences, and mild renal failure is stable.