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PubMed1993

Fundus pathology in neurosarcoidosis.

We describe the ocular symptomatology in 6 patients with neurosarcoidosis. All patients suffered from uveitis posterior or panuveitis. Ophthalmoscopy revealed multifocal chorioretinic lesions, periphlebitis, severe papilledema or an isolated chorioretinic process. The similarity with birdshot chorioretinopathy is described. Nuclear magnetic resonance imaging, computer assisted tomography and visual field examination can all contribute to the determination of neurological involvement in sarcoidosis.

Adolescent↗
PubMed2004

[Ocular involvement in Behçet's disease: first 5-year-results for visual development after treatment with interferon alfa-2a].

BACKGROUND: Behçet's disease (BD) is a systemic vasculitis of unknown origin. Up to now ocular involvement has a poor visual prognosis irrespective of immunosuppressive treatment. Now recent open studies have shown that interferon alfa-2a (IFN alfa-2a) is very effective in the treatment of ocular BD. The purpose of this study was to evaluate the long-term development of visual acuity (VA) in patients with severe ocular BD who were treated with IFN alfa-2a. METHODS: We included 15 eyes of 9 patients with an active panuveitis and/or retinal vasculitis due to BD refractory to immunosuppressive treatment. VA before initiation of IFN-therapy was compared to VA at the end of the follow-up time. RESULTS: Mean follow-up time was 68.8+/-10.6 months. Mean duration of IFN-treatment was 40.6+/-17.0 months. With the exception of one patient IFN alfa-2a could be discontinued in complete remission of ocular symptoms. During the follow-up time 10 eyes showed an increase of VA of two lines or more. In 5 eyes VA remained stable. There was no decrease of VA in any eye. If a macular edema was present, a quick response to IFN alfa-2a was seen. No eye developed a pale optic disk during follow-up. CONCLUSION: Compared to conventional immunosuppressants, interferon alfa-2a seems to be much more effective to prevent a loss or decrease of VA over a long period of time in patients with severe ocular BD.

Adult↗
PubMed2005

[Adamantiades-Behcet's disease].

Adamantiades-Behcet's disease (ABD) is a chronic recurrent vasculitis whose aetiology is still unclear. The first description goes back to B. Adamantiades und H. Behcet. The disease mainly occurs in Mediterranean, Middle and Far Eastern countries. However, it is not limited to these regions and can be found worldwide, and, as a result of increasing human mobility, is steadily becoming more significant. Clinical findings reveal recurrent oral and/or genital ulcers, arthritis and skin involvement in the form of erythema nodosum and superficial thrombophlebitis. Relapsing ocular involvement is one of the major manifestations in ABD and can be found in 60-80% of patients, resulting in retinal vasculitis, neuropathy or panuveitis. Eye involvement can lead to blindness in the affected eye in 20-50% of cases. Steroids, cyclosporin A und azathioprin are available to treat affected eyes. Studies of new treatment modalities including interferon-alpha or TNF antibodies are under way.

Adrenal Cortex Hormones↗
PubMed2005

[Vision-threatening complications in childhood uveitis].

PURPOSE: We analyzed the epidemiologic data and vision-threatening complications in different forms of childhood uveitis. METHODS: This retrospective study included 187 consecutive patients with onset of uveitis before the age of 16 years classified as anterior (AU), intermediate (IU), posterior (PU), and panuveitis (PanU). We analyzed the epidemiologic data as well as visual acuity, uveitic complications and the conservative and surgical therapy. RESULTS: Associated disease was observed in 85 of 187 patients. The most common complications in AU patients were cataract, posterior synechiae, band keratopathy and CME. IU was accompanied by dense vitreous opacities, cataract and CME. Macular scars were the most frequent cause for visual loss in PU. PanU was complicated by dense vitreous opacities, cataract, retinal detachment, CME and phthisis bulbi. CONCLUSIONS: Childhood uveitis is frequently associated with systemic immune-mediated diseases. The diverse uveitis types have different but typical complications.

Adolescent↗
PubMed2007

[Pseudoangiitis in bilateral ocular ischemia].

BACKGROUND: The term 'frosted branch angiitis' was initially used to describe an idiopathic form of vasculitis in association with panuveitis. It has since also been used to describe the angiographic phenomenon of diffuse leakage along retinal vessels against the background of other ocular and systemic diseases. METHODS: We describe a 57-year-old male patient with acute bilateral reduction of visual acuity, headaches and absence of any pulse at the temporal arteries. Fluorescence angiography showed bilateral diffuse leakage along all the retinal vessels, which resembled frosted branches. Laboratory parameters and histology were not indicative of vasculitis. Imaging showed complete occlusion of both common carotid arteries and a hypoplastic vertebral artery on the left. CONCLUSIONS: Ocular ischemia may imitate primarily inflammatory conditions in its angiographic appearance.

Angiography↗
PubMed1997

[Acute Borrelia infection. Unilateral papillitis as isolated clinical manifestation].

BACKGROUND: Borrelia burgdorferi is the cause of erythema chronicum migrans and Lyme disease. Ticks like Ixodes ricinus are responsible for transmission. Frequently, the tick bite is not noticed by the patient. Eye manifestations, such as keratoconjunctivitis, scleritis, chronic uveitis, vitritis, chorioretinitis, optic nerve disease, orbital myositis and paresis of the eye muscles, often occur after a long period of time and vary greatly. PATIENTS AND METHODS: We present below the case reports of a man 38 years old and a woman of 31, each with manifestation of an ocular Borrelia infection (papillitis and panuveitis, respectively). RESULTS: By antibody-screening with the ELISA technique and Western Blot analysis we were able to prove the serological infection. After specific antibiotic therapy, ocular inflammation improved rapidly, as did visual acuity. The papillitis only healed partially. CONCLUSIONS: In case of therapy-resistant inflammation of the eye we have to exclude general infections because cortisone therapy alone may result in worsening the condition. VECP can be used effectively in the differential diagnosis of papilloedemas. Early diagnosis and therapy of an acute Borrelia infection restrict the extent of the lesions and prevent ocular and general late manifestations. Seronegative values in subjects strongly suspected of having Lyme disease do not necessarily exclude the diagnosis of Lyme disease.

Borrelia burgdorferi Group↗
PubMed2005

Blau syndrome and related genetic disorders causing childhood arthritis.

Blau Syndrome (BS) is an inheritable disorder characterized by granulomatous polyarthritis, panuveitis, and exanthema. It was described by Edward Blau in 1985, the same year in which Douglas Jabs reported a very similar family. Clinically indistinguishable from early onset sarcoidosis (EOS), both are now known to share a mutated form of caspase recruitment domain-15 (CARD 15), a protein involved in activation of nuclear factor kappa B which is in turn an up-regulator of pro-inflammatory cytokine transcription. An association between BS and EOS was suspected for years given the striking similarities of the core triad (arthritis-uveitis-dermatitis) and a common emerging pattern of systemic involvement. Hence, the familial form (BS) and the sporadic form (EOS) are almost certainly the same illness/defect, inherited in the first and acquired in the second as a result in most cases of a de novo mutation. Another form of granulomatous arthritis with uveitis, Crohn's disease, has also been associated with mutations in CARD 15 (albeit at a different domain) and despite similar phenotypes there are obvious differences including gut inflammation and pyoderma gangrenosum in Crohn's disease. This paper will review the clinical characteristics of these three disorders and their association with mutations in the CARD 15 gene.

Adolescent↗
PubMed2004

Cutaneous hypopigmentation following a posterior sub-tenon triamcinolone injection.

PURPOSE: To report a case of cutaneous skin hypopigmentation following a posterior sub-Tenon triamcinolone acetonide injection for uveitis. DESIGN: Observational case report. METHODS: A 28-year-old African American female with Adamantiades-Behcet disease and panuveitis was administered a posterior sub-Tenon injection of triamcinolone acetonide and developed cutaneous hypopigmentation. RESULTS: The patient developed skin hypopigmentation of the upper eyelid adjacent to the site of the sub-Tenon injection. CONCLUSION: Injection of corticosteroids into the sub-Tenon space can cause hypopigmentation of the adjacent tissues, especially in heavily pigmented individuals.

Adult↗
PubMed2005

Atypical presentation of multiple evanescent white dot syndrome involving granular lesions of varying size.

PURPOSE: To report an atypical presentation of multiple evanescent white dot syndrome (MEWDS). DESIGN: Observational case report. METHODS: Review of the clinical, laboratory, photographic, and angiographic records of a patient with MEWDS. RESULTS: A 29-year-old woman presented with 20/400 vision in the right eye associated with multiple, granular lesions of varying sizes (100-1000 microm) in the outer retina, a prominent panuveitis, diffuse choroidal thickening and enlarged blindspot. The multiple lesions and visual disturbances resolved within 4 weeks with corticosteroid treatment, and the patient was diagnosed with MEWDS. CONCLUSIONS: Clinicians should be aware of atypical manifestations of MEWDS. The patient's unusual fundoscopic changes and prominent intraocular inflammatory reaction are findings that have not been reported in patients with MEWDS.

Adult↗
PubMed2006

[Interest of an internist's consultation in uveitis. Comparative study in 66 cases].

BACKGROUND: After exclusion of a masquerade syndrome, uveitis may be associated with infection, systemic diseases, specific ocular diseases, or may be drug-induced. In order to improve diagnostic strategy in uveitis, we performed a comparative study to assess the importance of internist and ophtalmologist's collaboration and we proposed a well-adapted diagnostic procedure. MATERIAL AND METHODS: A comparative study was performed in a tertiary centre. The population was divided in two groups. The first one was retrospective and consisted of patients treated at the Ophthalmology department of Croix Rousse Hospital from 1991 to 2002 without internist's collaboration. The second one was prospective and consisted of patients referred in the same centre in 2003 and 2004 with intervention of an internist. Patients of less than 18 years of age with pre-existent diagnosis, specific ocular diseases, toxoplasmosis infection, or with human immunodeficiency virus infection were excluded. RESULTS: Sixty-six patients were included. Anterior uveitis was the most frequent form (25 cases), followed by panuveitis (20 cases), posterior uveitis (14) and intermediate uveitis (7). The most frequent cause of uveitis was systemic disease (19 cases) followed by infection (7 cases) and neurological entities (4 cases). The rate of diagnosis was 30.3% without internist's intervention and 60.6% when patients were referred to an internist (P=0,01). The internist intervention was contributive in 75% of diagnoses. CONCLUSIONS: The internist intervention significantly enhances the rate of etiological diagnoses in uveitis. These results should further strengthen the internist/ophthalmologist collaboration for patients with uveitis.

Adult↗
PubMed2000

Immunomodulatory therapy for chronic tubulointerstitial nephritis-associated uveitis.

PURPOSE: To describe the clinical course and treatment with immunomodulatory agents in patients with tubulointerstitial nephritis and uveitis syndrome. METHODS: Retrospective analysis of the charts of six patients with tubulointerstitial nephritis and uveitis syndrome. RESULTS: The mean (+/-SD) age was 24.3 (+/-16.5) years, range 13 to 49 years. Four patients were children, and two were adults. Three were men, and three were women. Five of the six patients had anterior uveitis, and one had panuveitis. All patients had several relapses despite treatment with topical, regional, and oral steroids and methotrexate in one case. The introduction or modification of immunosuppressants (methotrexate, azathioprine, or cyclosporin A) achieved control of the uveitis and prevented relapses over a mean (+/-SD) follow-up period of 19.66 (+/-10.01) months, range 6 to 34 months. No treatment-related side effects were observed. CONCLUSIONS: Tubulointerstitial nephritis and uveitis syndrome is a distinct disease entity in which the nephritis typically resolves, but the uveitis often becomes chronic and is treatment resistant. Immunomodulatory agents can achieve control of the inflammation and prevent relapses.

Adolescent↗
PubMed1994

A case of uveitis associated with idiopathic retroperitoneal fibrosis.

We treated a patient with nongranulomatous panuveitis associated with idiopathic retroperitoneal fibrosis who had symptoms of abdominal pain, severe fever, leg edema, and blurred vision. A high C-reactive protein level, a high erythrocyte sedimentation rate, anemia, and abnormalities of the immune system were seen. Retrograde pyelography disclosed persistent ureteral obstruction. A computed tomographic scan and magnetic resonance imaging showed a dense plaque of fibrous tissue around the kidney and hydronephrosis. Biopsy disclosed fibrosis infiltrated with lymphocytes, plasma cells, histiocytes, and eosinophils. A moderate degree of cellular inflammation in the anterior chamber and cystoid macular edema were present at ocular examination. We believe idiopathic retroperitoneal fibrosis to be a new cause of uveitis of suspected autoimmune origin.

Blood Sedimentation↗
PubMed2005

[Vogt-Koyanagi-Harada syndrome].

INTRODUCTION: Vogt-Koyanagi-Harada syndrome is a rare, inflammatory disease with manifestations affecting the ocular, central nervous, audito-vestibular, and integumentary systems. Vogt-Koyanagi-Harada syndrome is more frequent in Asia but is also described in Europe. We report three new non asiatic cases of this syndrome. OBSERVATIONS: The three patients had bilateral panuveitis and hypoacusia. Two of them had peripheral facial palsy, two of them had vestibular syndrome and one of them lymphocytic meningitis. In each case we found characteristic HLA II typing and in one case we discovered the simultaneous presence of three auto-antibodies: anti-retina (anti-Arrestin type), anti-choroid and anti-cochlea. These patients were treated by corticosteroids but required an additional treatment by cyclophosphamide (0.8g/m2). The clinical course was favorable with visual sequelae for two and auditory one for one. DISCUSSION: These biological and therapeutic elements and a review of the recent literature are in favor of an autoimmune origin of this syndrome.

Adult↗
PubMed2002

Massive mycobacterial choroiditis during highly active antiretroviral therapy: another immune-recovery uveitis?

PURPOSE: To describe the ocular presentation of disseminated mycobacterial disease occurring during immune-recovery in a patient with acquired immune deficiency syndrome (AIDS). STUDY DESIGN: Case report and literature review. PARTICIPANTS: A 41-year-old AIDS patient with a prior diagnosis of cytomegalovirus retinitis. METHODS: The patient developed progressive, bilateral multifocal choroiditis with panuveitis 2 months after beginning and responding to highly active antiretroviral therapy. His left eye became blind and painful and was enucleated. Pathologic examination revealed massive choroiditis with well-formed, discrete granulomas and multiple intracellular and extracellular acid-fast organisms within the choroidal granulomas. Culture and polymerase chain reaction of vitreous specimens revealed Mycobacterium avium complex (MAC). RESULTS: Empiric, and later sensitivity-guided, local and systemic antibiotic therapy was used to treat the remaining right eye, but it continued to deteriorate. Despite medical therapy, three vitrectomies and repeated intravitreal injections of amikacin, a total retinal detachment ensued. One week after the third vitrectomy, the patient died from mesenteric artery thrombosis in the setting of disseminated mycobacterial disease. CONCLUSIONS: This is the first report of ocular inflammation as the presenting finding in the recently recognized syndrome of immune-recovery MAC disease. Pathogenesis of this entity is related to an enhanced immune response to a prior, subclinical, disseminated infection. The formation of discrete granulomas, normally absent in MAC infections in AIDS, reflects this mechanism.

AIDS-Related Opportunistic Infections↗
PubMed1996

Ocular malaria. A clinical and histopathologic study.

PURPOSE: Clinical and histopathologic findings of ocular lesions in malaria rarely have been described. This study reports lesions in three patients with malaria, with a histopathologic study of eyes obtained at autopsy of one of these patients. METHODS: Various ocular lesions were documented in three patients with malarial infection, with histopathologic study of one patient. In all three patients, the diagnosis of malaria was confirmed by the demonstration of malarial parasites in peripheral smears and by fluorescent microscopy of acridine orange-stained buffy coat preparations of venous blood. RESULTS: Multiple superficial blotchy retinal hemorrhages over the posterior pole were seen in the first patient, whereas acute bilateral panuveitis and secondary glaucoma were seen in the second, which on resolution showed multiple blotchy superficial retinal hemorrhages with perivasculitis. The third patient had cerebral malaria and was found to have subconjunctival and retinal hemorrhages. This patient died of pulmonary thromboembolism, and the eyes were obtained at autopsy. On gross examination, there were multiple retinal hemorrhages in the posterior pole. Histopathologic study showed cytoadherence of parasitized erythrocytes as well as schizonts and gametocytes of Plasmodium vivax within the retinal and choroidal blood vessels. CONCLUSION: The authors' findings suggest that patients with unexplained blotchy retinal hemorrhages should be investigated for malarial infection, especially if they reside or have travelled in endemic areas.

Acridine Orange↗
PubMed1996

Enlarged blind spots in chorioretinal inflammatory disorders.

PURPOSE: This study was undertaken to better characterize patients with multifocal choroiditis and panuveitis (MCP), punctate inner choroidopathy (PIC), multiple evanescent white dot syndrome (MEWDS), and diffuse subretinal fibrosis syndrome. The specific aim was to determine whether these disorders were different entities or part of a spectrum of diseases with similar features. METHODS: Seventy-nine patients were included in this study. Most of the patients have been followed up prospectively since July 1980 with some found retrospectively. RESULTS: Forty-one patients had MCP, 16 had PIC, 6 had diffuse subretinal fibrosis syndrome, and 16 had MEWDS. Patients with MCP had visual loss and visual field defects caused directly by visible lesions or recurrent inflammation around old lesions. In particular, clustering of lesions around the optic nerve and nasal periphery was seen in patients with MCP and appeared to be related to visual field loss. Patients with PIC also had enlarged blind spot and other field defects explained by fundus lesions. Patients with PIC and MCP did not have recurrent lesions on extended follow-up. Patients with diffuse subretinal fibrosis syndrome represented a subset of patients characterized with lesions in the posterior pole, sever scarring, and visual loss. Patients with MEWDS had the least inflammation with symmetrically distributed lesions. Minimal permanent chorioretinal scarring was seen in patients with MEWDS. Visual field defects improved in most patients with MEWDS and PIC, whereas most patients with MCP and diffuse subretinal fibrosis syndrome did not improve. CONCLUSIONS: Although enlarged blind spots are a feature of all four disorders, other clinical, angiographic, and electroretinographic evidence suggest that these are different entities.

Adolescent↗
PubMed2005

[Cystic fibrosis and uveitis in childhood and multiple sclerosis at adulthood].

We report the case of a patient with cystic fibrosis who suffered severe bilateral uveitis, with hypopyon, retinal vasculitis, and polyarthritis when he was 6 years old. No etiology could be found. Multiple sclerosis began when he was 22 years old. This panuveitis was clinically very different from the uveitis usually associated with multiple sclerosis. Polyarthritis and skin vasculitis have been reported in the course of cystic fibrosis, but no uveitis nor retinal vasculitis have been described. This raises the question of the role of multiple sclerosis and cystic fibrosis in the pathogenesis of this case of uveitis.

Adult↗
PubMed1998

Iris retraction syndrome associated with nonrhegmatogenous retinal detachment.

PURPOSE: To present the first documentation of iris retraction syndrome in eyes with nonrhegmatogenous retinal detachment. PATIENTS AND METHODS: One patient with age-related macular degeneration and another with panuveitis developed exudative retinal detachment with iris retraction configuration. Ultrasound biomicroscopy was performed to investigate the anatomic relationship of structures in the anterior segment of the eye. RESULTS: Ultrasound biomicroscopy demonstrated a severe backward bowing of the peripheral iris with irido-ciliary body and irido-zonular contact as well as broad iris lens touch. The iris retraction syndrome resolved after pupil dilation and disruption of the pupillary adhesions in both cases. The retinal detachment resolved several months later, without surgery. CONCLUSION: Iris retraction syndrome appears not to be exclusive to rhegmatogenous retinal detachment but can present in eyes with exudative - nonrhegmatogenous retinal detachment. Thus, when the configuration of the iris shows bowing in patients with retinal detachment, iris retraction syndrome should be considered and prompt pupil dilation should be carried out.

Aged↗