| Cogan's syndrome is a rare clinical entity defined by the association of a nonsyphilitic interstitial keratitis and vestibuloauditory dysfunction, typically Meniere's disease-like; the condition has been reported in association with a variety of cutaneous diseases. We now report a case of pyoderma gangrenosum complicating Cogan's syndrome in a 57-year-old woman, which then healed dramatically, as more interestingly did the associated uveitis with minocycline therapy. ( info) |
2/555. The treatment of refractory uveitis with intravenous immunoglobulin. PURPOSE: To study the treatment of uveitis that has not responded to immunosuppressive medication. Intravenous immunoglobulin (IVIg) effectively treats a variety of autoimmune diseases, but it has not been adequately studied in the treatment of uveitis. methods: The trial included patients who satisfied criteria that included noninfectious uveitis, active inflammatory disease, and a failure to respond adequately to immunosuppressive medication. We treated two patients with IVIg (0.5 gm/day, 3 days/mo initial dosage) as a pilot study and then treated an additional eight patients with a similar dosage as part of a formal but uncontrolled protocol. RESULTS: patients on the protocol have been followed for a median of 11 months and have received a median of 7.5 treatment cycles. Five of 10 patients have had a clinically important and sustained improvement in visual acuity, and two of eight protocol patients have markedly reduced their immunosuppressive medication. CONCLUSIONS: Intravenous immunoglobulin can benefit some patients with uveitis that is otherwise refractory to immunosuppressive therapy. Although our preliminary experience is encouraging, the use of IVIg for uveitis should be limited because of cost, toxicity, the requirement for repeated administration, and the absence of controlled trials that demonstrate efficacy. ( info) |
3/555. Acute eosinophilic interstitial nephritis and uveitis (TINU syndrome) associated with granulomatous hepatitis. A 23-year-old male presented with renal failure, cholestatic liver enzyme elevation and uveitis. Percutaneous renal biopsy revealed marked eosinophilic infiltration of the renal interstitium, which made the diagnosis of TINU syndrome (Tubulo-Interstitial nephritis and uveitis). Percutaneous liver biopsy showed granulomatous hepatitis, which was not described as a part of TINU syndrome. The diagnostic dilemma and the literature are discussed. ( info) |
4/555. Tubulointerstitial nephritis and uveitis in children and adolescents. Four new cases and a review of the literature. We identified 35 cases of tubulointerstitial nephritis and uveitis (TINU), 31 from a medline search (1966-1996) of the English literature and 4 from our hospital records (1988-1996). To meet the case definition, the patient had to be less than 18 years old and have TINU of unknown cause. Common presenting symptoms included fatigue, weight loss, fever, and abdominal pain. The uveitis was usually anterior and could occur at any time with respect to the onset of the renal disease. Common laboratory features included anemia, increased erythrocyte sedimentation rate, and decreased creatinine clearance. Most patients (33 of 35) had renal biopsies that commonly revealed an intense inflammatory interstitial infiltrate, glomerular sparing, and negative immunofluorescence studies. Of the 35 patients, 26 received systemic corticosteroid therapy (5 of 26 for eye disease); 22 had follow-up for at least 1 year; 13 of 35 patients had a recurrence of their uveitis. The outcome in all 35 cases was normal renal function with no documented visual loss. In conclusion, TINU is a unique syndrome with characteristic clinical features, laboratory changes, and renal biopsy results. Treatment is controversial, and the outcome in children, even if untreated, is excellent. ( info) |
5/555. hyperthyroidism: a novel feature of the tubulointerstitial nephritis and uveitis syndrome. Tubulointerstitial nephritis and uveitis syndrome presents with either renal or ocular manifestations, and associated weight loss. We report two adolescents with transient hyperthyroidism early in the course of the syndrome. hyperthyroidism may represent an unrecognized feature of the disorder and potentially contributes to the accompanying weight loss. Thyroid function should be evaluated in patients with tubulointerstitial nephritis and uveitis syndrome and symptomatic therapy provided when necessary. ( info) |
6/555. Tubulointerstitial nephritis and uveitis in association with Epstein-Barr virus infection. The case of a 13.5-year-old girl with acute tubulointerstitial nephritis and uveitis (TINU syndrome) is presented. The etiology of this rare syndrome, which in most cases involves female adolescents and usually regresses spontaneously, is still unknown. An infection-triggered pathological immune reaction has been considered to play a role in the pathogenesis of this disorder. Here we report for the first time the association of TINU syndrome and Epstein-Barr virus infection. ( info) |
7/555. Intrafamilial occurrence of tubulointerstitial nephritis with uveitis and Vogt-Koyanagi-Harada syndrome. We describe a child with acute tubular dysfunction, reversible renal failure, and uveitis, whose father has Vogt-Koyanagi-Harada syndrome. This is the first reported familial association of these 2 syndromes with distinctively overlapping clinical characteristics. A pathogenic association is proposed. ( info) |
8/555. sarcoidosis presenting in infancy: a rare occurrence. sarcoidosis is infrequent in children. Clinical features of sarcoidosis occurring in children 8-15 years old include pulmonary, lymphnodes and ocular involvement. The picture is similar to that of adult-sarcoidosis. sarcoidosis in infants, however, is rare and it differs from the adult disease. sarcoidosis occurring in infancy has features clinically similar to that of juvenile rheumatoid arthritis. The case presented here is that of an infant who developed sarcoidosis at the age of 3 months. Her illness had all the features of a multisystem illness except for the hilar or parenchymal lung involvement. Because of the complicated and unusual illness the diagnosis of sarcoidosis was not established till the child was five years old. She received corticosteroids and immunosuppressive treatment in varying doses for a long time. Now at the age of 18, the patient's disease seems to have undergone a complete remission. ( info) |
9/555. Clinical course of HTLV-I-associated uveitis. PURPOSE: To define the long-term clinical course and visual outcome of human T-cell lymphotrophic virus type I (HTLV-I)-associated uveitis (HAU). methods: We reviewed the clinical data on 96 eyes of 70 patients, 26 men and 44 women, with HAU, with specific reference to recurrence of the disease and long-term visual outcome. The mean follow-up period was 83 months (range, 12-276 months). RESULTS: The mean age of onset was 42.8 years (range, 7-78 years of age), with men presenting at a significantly younger age. Forty-seven patients had isolated HAU; in 10 patients, HTLV-I-associated myelopathy occurred before or after the onset of HAU; in 14 patients, hyperthyroidism had preceded HAU. A single episode of mild to moderate acute uveal inflammation with resolution in a few weeks or more occurred in 44 (62.9%) patients, and multiple episodes in 26 (37.1%), with a mean interval of 16 months (range, 1-250 months), which affected the same eye, fellow eye, or both. The majority of patients had favorable visual outcome at the last examination, whereas only a few patients suffered poor vision resulting from steroid cataract and retinochoroidal degeneration. CONCLUSIONS: The clinical course of HAU is virtually benign and its visual outcome is favorable, although its recurrence is common. The uveitis is usually isolated and affects a portion of otherwise unremarkable HTLV-I carriers, but it may sometimes be manifest as a symptom of syndromic diseases such as HTLV-I-associated myelopathy or hyperthyroidism. This study describes for the first time cases of HAU that occurred many years before manifestation of HTLV-I-associated myelopathy. ( info) |
10/555. cytomegalovirus retinitis in the era of highly active antiretroviral therapy. A number of striking changes have occurred recently in the presentation and course of cytomegalovirus (CMV) retinitis in patients with acquired immunodeficiency syndrome (AIDS) who are receiving highly active antiretroviral therapy (HAART). Before the use of HAART, CMV retinitis was the most common intraocular infection in patients with AIDS, occurring in up to 40% of patients, typically when CD4 cell counts have decreased to less than 0.10 x 10(9)/L. By studying CMV retinitis, clinicians can investigate whether the rejuvenated immune system that results from HAART can effectively control opportunistic infections in patients with AIDS. In some patients, retinitis has not progressed when specific anti-CMV therapy was discontinued, but a number of patients have developed substantial intraocular inflammation, which has resulted in decreased visual acuity. Anterior uveitis, cataract, vitritis, cystoid macular edema, epiretinal membrane, and disc edema may occur in patients with CMV retinitis who have experienced HAART-associated elevation in CD4 cell counts. Since immune recovery uveitis does not occur in eyes without CMV retinitis, the ocular inflammation appears to be related to the CMV infection. Anti-CMV maintenance therapy likely can be safely discontinued in some patients with CMV retinitis if CD4 cell counts are stable or increasing and have been higher than 0.10 x 10(9)/L for at least 3 months. Immune recovery in patients receiving HAART has been effective in controlling opportunistic infections, but it may also result in intraocular inflammation, which can have adverse effects on the eye. ( info) |