Cases reported "Exophthalmos"

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1/4. Ocular manifestations of patients with circulating antineutrophil cytoplasmic antibodies.

    Antineutrophil cytoplasmic antibodies are seen in patients with systemic vasculitides, especially Wegener's granulomatosis. Antineutrophil cytoplasmic antibodies are helpful laboratory markers for these disease. We report on the ocular findings of six patients with systemic vasculitis who had antineutrophil cytoplasmic antibodies. Four patients had systemic Wegener's granulomatosis, one had microscopic polyarteritis, and in one a specific histopathologic diagnosis could not be made. Two patients were first evaluated for systemic vasculitis because of their ocular manifestations. Ocular findings included ptosis, bilateral lacrimal gland masses, proptosis, choroidal folds, episcleritis, phlebitis, retinal and vitreous hemorrhage, keratitis sicca, and bilateral central scotomas. It was difficult to make a systemic diagnosis in all cases. If systemic vasculitis is in the differential diagnosis of a patient with suggestive ocular findings, antineutrophil cytoplasmic antibody testing should be considered. A prospective study of antineutrophil cytoplasmic antibody testing should be considered in patients with ocular findings that suggest the possibility of vasculitis.
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ranking = 1
keywords = granulomatosis
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2/4. The ocular manifestations of Wegener's granulomatosis. Fifteen years experience and review of the literature.

    Ocular manifestations of Wegener's granulomatosis may occur secondary to contiguous granulomatous sinusitis or as a result of focal vasculitis. Contiguous granulomatous sinus disease causes nasolacrimal duct obstruction, proptosis and ocular muscle or optic nerve involvement. Focal vasculitis unrelated to contiguous upper respiratory tract disease is manifested by conjunctivitis, episcleritis, scleritis, corneoscleral ulceration, uveitis, and granulomatous vasculitis of the retina and optic nerve. A review of 29 cases of Wegener's granulomatosis and three cases of lymphomatoid granulomatosis studied over the past 15 years at the National Institute of Allergy and Infectious Diseases (NIAID) disclosed single or multiple ocular manifestations of disease in 15 patients (47 per cent). The pattern of ocular disease, its relationship to systemic involvement, diagnostic methods and the response to therapy are discussed.
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ranking = 3.5
keywords = granulomatosis
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3/4. Wegener's granulomatosis: unusual indication for orbital decompression.

    Wegener's granulomatosis (WG) is characterized by granulomatous vasculitis of the upper and lower respiratory tract together with glomerulonephritis. The majority of cases have ocular, sinus and ear symptomatology. A case is presented with severe granulomatous disease of the sinuses with contiguous spread to the orbit producing exophthalmos and decreased vision. An orbital decompression relieved the pressure and preserved vision. The definitive medical therapy for WG is cyclophosphamide. Emergency orbital decompression may be necessary to preserve vision in WG in patients with rapidly decreasing vision secondary to high intraocular pressure produced by the granulomatous process.
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ranking = 2.5
keywords = granulomatosis
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4/4. Elevated anti-neutrophil cytoplasmic antibody titer in a patient with atypical orbital pseudotumor.

    We recently examined a patient with ophthalmic manifestations of Wegener's granulomatosis in whom antineutrophil cytoplasmic antibody (cANCA) titers provided helpful diagnostic information. A 40-year-old man who had suffered from bilateral exophthalmos for 9 months was diagnosed initially as having idiopathic inflammatory pseudotumor. The patient exhibited purulent nasal discharge and microhematuria. A histopathologic study revealed vasculitis. His ANCA titer for cANCA was found to be evaluated, and our patient was subsequently diagnosed as having Wegener's granulomatosis. His ocular symptoms resolved and did not recur after treatment with corticosteroid in combination with cyclophosphamide. We believe that cANCA levels should be investigated in patients with orbital pseudotumor as a possible sign of Wegener's granulomatosis.
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ranking = 1.5
keywords = granulomatosis
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