Cases reported "Disease Progression"

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1/6. Immunosuppressive therapy in serpiginous choroiditis--case report and brief review of the literature.

    BACKGROUND: At present there is no consensus regarding the efficacy of the immunosuppressive therapy in serpiginous choroiditis. PATIENT AND methods: We describe the case of a patient with a macular serpiginous choroiditis. Despite corticosteroid therapy the choroiditis was progressive and threatened the central vision. Therefore a triple immunosuppressive therapy with prednisone 1 mg/kg body weight/day (mg/kg/d), azathioprine 1.5 mg/kg/d, and low-dose cyclosporine A 4.5 mg/kg/d was introduced. RESULTS: Under this triple immunosuppressive therapy a prompt stabilisation of the chorioretinal process could be observed in fluorescein angiography. After five months of therapy the examination of the visual field shows an impressive recovery. No significant side effects have been noted. CONCLUSIONS: In the literature, until now only six patients with serpiginous choroiditis treated with a triple agent immunosuppressive therapy have been reported. In all cases this regimen seemed to have a positive influence on the disease. Our present observation corroborates the beneficial effect of a triple agent immunosuppressive therapy on the course of serpiginous choroiditis. However, the beneficial effect of such a therapeutic approach needs to be confirmed by further observations.
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2/6. Successful treatment of serpiginous choroiditis with alkylating agents.

    OBJECTIVE: To describe the management and long-term outcomes of patients with serpiginous choroiditis treated with alkylating agents. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Nine patients with active, vision-threatening serpiginous choroiditis who had progressive inflammation while on steroids and/or immunosuppressive agents other than alkylating agents treated at three tertiary care uveitis referral centers. methods: patients received systemic immunosuppression with an alkylating agent, either chlorambucil or cyclophosphamide. prednisone also was given initially and was tapered and discontinued. MAIN OUTCOME MEASURES: visual acuity, clinical disease activity, duration of treatment, duration of drug-free disease remission, and side effects of alkylating agent therapy. RESULTS: No patients had recurrences while on therapy. No further visual loss was encountered after starting the therapy. Six of the patients regained vision. All but two patients achieved prolonged drug-free remissions, ranging in duration between 15 and 96 months (median, 78 months). Side effects included transient bone marrow suppression, nausea, and fatigue. Secondary malignancy was encountered in one patient, whose carcinoma of the urinary bladder was treated successfully. CONCLUSIONS: Adequate immunosuppression with alkylating agents may favorably alter the long-term prognosis of patients with serpiginous choroiditis.
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keywords = choroiditis
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3/6. Cogan's syndrome: a cause of progressive hearing deafness.

    In 1934 Morgan and Baumgartner first described a nonsyphilitic interstitial keratitis (IK) associated with vestibuloauditory dysfunction (Morgan RF, Baumgartner CF, Menier's disease complicated by recurrent interstitial keratitis. Excellent result following cervial ganglionectomy. West J Surg 1934;42:628). Cogan was the first to describe this syndrome as a clinical entity with the report of 5 additional cases in 1945 (Cogan DA: syndrome of nonsyphilitic interstitial keratitis and vestibuloauditory symptoms. Arch Ophthalmol 1945;33:144-9). Since that time, more than 100 cases of Cogan's syndrome (CS) have been in the literature. Cogan's syndrome is a rare clinical disease, which primarily affects young adults; however, published reports range from 2.5 to 60 years for age of onset (Kundell Sp, HD Ochs: Cogan's syndrome in childhood. J Pediatr 1980;97:96-8). This disease primarily affects whites and is believed not to be hereditary. Typical CS is characterized by IK and vestibuloauditory dysfunction. The IK usually occurs with sudden onset and is characterized by photophobia, lacrimation, and eye pain. The vestibuloauditory dysfunction is usually bilateral, presenting with tinnitus, sensorineural hearing loss, and acute episodes of vertigo. Atypical CS presents with significant inflammatory eye disease (ie, scleritis, episcleritis, retinal artery occlusion, choroiditis, retinal hemorrhages, papilloedema, exophthalmos, or tenonitis) with or without IK (Laryngoscope 1960;70:447-9). In this report, we describe a typical case of CS, in which the hearing loss was unresponsive to corticosteroid therapy.
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4/6. Progressive subretinal fibrosis and blindness in patients with multifocal granulomatous chorioretinitis.

    PURPOSE: To describe the clinical and histopathologic findings in four eyes of three patients who became blind because of multifocal choroiditis and massive subretinal fibrosis. methods: Clinicopathologic correlative study. RESULTS: During a period of several years, three healthy elderly patients developed severe visual loss only partly explained by multifocal chorioretinitis and massive subretinal fibrosis. Histopathologic examination of four eyes from these patients disclosed widespread destruction of the outer retina and retinal pigment epithelium, massive areas of subretinal fibrous tissue proliferation, granulomatous inflammation centered around degenerated and fragmented Bruch's membrane, and chronic uveitis. No infectious organisms were identified by special stains or electron microscopy in one eye. CONCLUSIONS: Clinical and histopathologic findings in these three patients were consistent with an autoimmune disease process directed at the retina, retinal pigment epithelium, inner choroid, or all three. Cellular injury in this location can result in massive subretinal fibrosis. Subretinal fibroplasia, however, is probably a nonspecific reparative response to injury. The pathogenesis of this blinding disorder in elderly patients may be similar to the less severe disease usually occurring in younger patients with multifocal choroiditis, panuveitis, and punctate inner choroiditis.
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keywords = choroiditis
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5/6. fluorescein angiography in the progressive outer retinal necrosis syndrome.

    PURPOSE: Progressive outer retinal necrosis syndrome is a devastating retinopathy seen primarily in patients with acquired immune deficiency syndrome. To provide additional details of the pathogenesis of this disease, the authors describe the evolution of clinical and fluorescein angiographic changes during the course of progressive outer retinal necrosis syndrome. methods: The authors performed serial clinical examinations, fundus photography, and fluorescein angiography in a patient with acquired immune deficiency syndrome with progressive outer retinal necrosis syndrome. Clinical and fluorescein angiographic findings were correlated to provide detailed sequential analysis of the pathologic changes occurring during the course of this disorder. RESULTS: The angiographic changes seen during the various stages of the disease consisted of zonal microvascular alterations, retinal pigment epithelium (RPE) destruction, and choroidal leakage. Retinal damage was correlated closely with regions of choroidal leakage and was clinically evident as outer retinal whitening. disease reactivation occurred as a prominent brush-fire border of intense leakage involving the retina, RPE, and choroid. Extensive damage to the retinal vasculature and RPE was noted in the wake of clinical infection. CONCLUSIONS: The angiographic findings in our patient demonstrate that the progressive outer retinal necrosis syndrome is a retinochoroiditis that involves the full thickness of retina as well as the RPE and choroid. The inflammatory changes seen throughout the course of this disease correlate with the histopathologic patterns reported to date.
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keywords = choroiditis
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6/6. Progressive subretinal fibrosis in patients with rheumatoid arthritis and renal dysfunction.

    We present two patients (56-year-old and 71-year-old women) who developed subretinal fibrosis in parallel with exacerbation of rheumatoid arthritis and deteriorated renal function. The first patient developed subretinal fibrosis in the right eye, coupled with multifocal choroiditis and serous retinal detachment in both eyes, in the course of rapidly progressive glomerulonephritis complicated with rheumatoid arthritis. Antineutrophil cytoplasmic antibody was positive in a perinuclear pattern (pANCA) and the increased dose of prednisolone to 40 mg/day resulted in subsidence of the subretinal inflammation. The second patient developed subretinal fibrosis in the left eye with mottled retinal pigment epithelium of both eyes, in the course of rheumatoid arthritis and stable chronic renal failure. Although the manifestations of subretinal fibrosis and concurrent renal dysfunction were different between these two patients, subretinal fibrosis might be noted as a complication of rheumatoid arthritis and renal dysfunction.
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keywords = choroiditis
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