Filtration and local synthesis of lacrimal proteins in acquired immunodeficiency syndrome.

In AIDS the onset of the ocular dry syndrome, characterized by lacrimal hyposecretion and deterioration of the corneal and conjunctival epithelium, generally accompanies the clinical aggravation of immunodepression. The study of serum and lacrimal proteins contributes to our knowledge of the pathophysiology of this syndrome. The lacrimal clearance of albumin indicates changes in the permeability of the haemato-lacrimal and conjunctival barrier. Lacrimal monomeric IgA and IgG are mainly of plasmatic origin, while polymeric IgA and IgM are synthesized in situ. The concentrations of these analytes thus reflect ocular humoral immune status. They show a strong humoral protein response in patients with cytomegalovirus retinitis. Lacrimal concentrations of lactoferrin and lysozyme were found to be significantly decreased in AIDS patients with ocular dryness, reflecting a decrease in the secretory activity of the lacrimal gland. Moreover, ocular lympho-plasmocyte infiltration was observed in several patients, with an increase in lacrimal beta 2-microglobulin concentrations. These various lacrimal proteins could be good markers of the ocular dry syndrome in AIDS.

[Changes in the fundus in subacute sclerosing panencephalitis. Apropos of 23 cases]. / Altérations du fond d'oeil au cours de la panencéphalite sclérosante subaiguë. A propos de 23 cas.

Subacute sclerosing panencephalitis (SSPE) is a fatal slow encephalitis. It is related to a chronic infection of the central nervous system by a defective measles virus. Among 41 patients with SSPE, 23 had ocular involvement. The ophthalmologic involvement preceded the onset of neurological signs in 4 cases, they were detected at first presentation in 9 cases, and they occurred in the course of the disease in 10 cases. The ocular involvement was bilateral in 19 cases. Various ophthalmologic features were reported: retinitis (16 eyes), macular pigment disturbances (4 eyes), optical neuritis (33 eyes), visual agnosia (1 patient). The macular lesions were typical of the disease, but at the acute stage they were liable to be confused with toxoplasmosis. This complication of measles should disappear with the systematic vaccination of 12 month old children.

[Ocular prognosis in Behçet's disease]. / Pronostic oculaire de la maladie de Behçet.

The ocular prognosis of Behçet's disease was assessed in 31 French native patients with ocular involvement. The mean follow-up time was 6 years. Ophthalmologic manifestations revealed the disease in 61% of patients, and it was the first symptom in 29%. Two groups of patients could be identified: the minor forms (23%) which remained stable with colchicine alone and the severe forms (77%) which required steroid (32%) and/or immunosuppressive (45%) therapy. In the severe forms, 32 eyes were unchanged, 4 eyes showed improvement, and 12 eyes showed deterioration in visual acuity. Positivity of HLA B5, sex and complete form of the disease were not predictive factors of ocular involvement, or of its seriousness. At 6 years, 16% of the eyes (versus 14% at the beginning) had a visual acuity less than 1/10, and 71% (versus 74% at the beginning) had a visual acuity greater than 8/10. A close collaboration between ophthalmologists and internists allows a good visual prognosis in Behçet's disease.

[Syphilitic uveitis and human immunodeficiency virus infection]. / Uvéite syphilitique et infection par le virus de i'immunodéficience humaine.

Ocular syphilis is rare in human immunodeficiency virus infected individuals. We think that syphilis should be considered in evaluating such patients presenting with uveitis. Most often, ocular syphilis includes retinitis associated with anterior or posterior uveitis, sometimes with optic neuritis. Concurrent neurosyphilis is frequent and may be more aggressive; it may progress more rapidly and cause more atypical signs than in patients without human immunodeficiency virus infection. This suggests the need for lumbar puncture in the evaluation of coinfected patients. The standard serological tests for syphilis (in blood and cerebrospinal fluid) may be nonreactive in human immunodeficiency virus seropositive patients. It may be because of the alteration of immunologic response of such patients. All coinfected patients with human immunodeficiency virus and syphilis should be treated with high-dose intravenous penicillin G sodium as recommended for neurosyphilis. We describe two human immunodeficiency virus infected patients with ocular syphilis and neurosyphilis.

[Cytomegalovirus retinitis in AIDS. Treatment with intravitreal injections of ganciclovir]. / Rétinite à cytomégalovirus au cours du SIDA. Traitement par injections intravitréennes de ganciclovir.

Cytomegalovirus (CMV) retinitis is the most frequent ocular infection in AIDS, and it is responsible for blindness. Intravitreal injections of ganciclovir in doses of 400 g have been tried in patients who could not tolerate any systemic treatment. Induction therapy consists of 2 injections per week, followed by maintenance therapy with 1 injection per week. The present study involved 17 patients. Twenty-three induction courses could be evaluated; all had a favourable response with an average of 6 injections per eye. Seventeen maintenance courses were evaluated, with a mean of 5 injections per eye. There was no relapse in 10 eyes (70 percent) at 48 days. There was no evidence of toxicity from repeated intravitreal injections: no cataract, retinal detachment or endophthalmitis was observed with a total of 231 injections. Intravitreal ganciclovir appears to be an effective local treatment of CMV retinitis in AIDS patients when systemic therapy cannot be used.

[Retinitis disclosing subacute sclerosing panencephalitis]. / Rétinite révélatrice de panencéphalite sclérosante subaiguë.

SSPE is a panencephalitis who involves children and who leads to death. It is due to a defective measles virus. Ophthalmologic features are frequent and may be the first manifestation of the disease. The most typical ocular involvement is retinitis which can be misdiagnosed as a toxoplasmosis.

Idiopathic acute interstitial nephritis associated with anterior uveitis in adults.

Concomitant renal and ocular lesions have been described in a few systemic diseases. The association of acute interstitial nephritis (AIN) and anterior uveitis without determined cause was first described in children. Recently, the same clinical association has been reported in adults. We report 3 cases of this association and present a review of the literature. Including our 3 patients, 7 cases of this association have been reported in adults. All patients were females aged 27-74 years. Initial symptoms were either ocular, or pseudoviral (fever, myalgia and fatigue). Histological renal studies revealed acute interstitial nephritis with tubular lesions. Immunofluorescence and electron microscopy were not contributive. Ocular prognosis was always good. In 5 patients, the evolution of renal function was excellent with complete resolution of acute renal failure within a few weeks. Chronic renal failure developed in two of the four patients who did not receive systemic steroid therapy (with evolution towards terminal renal failure in one patient). Three of the patients received 60 mg per day of prednisone and none of them developed chronic renal failure. Despite the small number of patients reported and the possibility of spontaneous regression, these data suggest a beneficial effect of systemic steroid therapy to prevent or reduce interstitial inflammation and subsequent fibrosis.

[Serum and lacrimal proteins in acquired immunodeficiency syndrome]. / Protéines sériques et lacrymales au cours du syndrome d'immunodéficience acquise (SIDA).

The concentrations of seven proteins [albumin (Alb), immunoglobulins (IgG, A, M), lactoferrin (Lf), lysozyme (Lzm) and beta 2-microglobulin (beta 2 m)] were measured in the tears and serum of 10 patients with acquired immune deficiency syndrome (AIDS) and in 13 normal subjects. In the AIDS group, alteration of the blood-lacrimal barrier was revealed by the high Alb clearance. In this group also, the significantly high IgG, IgA, IgM and beta 2 m concentrations indicated a systemic and local immune response, with passive transfer of these proteins from serum to tears. However, Lf and Lzm concentrations were normal, showing that the secretory activity of the lacrimal gland remained unchanged.

[Diffuse retinoblastoma--apropos of a case]. / Rétinoblastome diffus--à propos d'une observation.

We report a case of diffuse retinoblastoma in a 6 years old boy. The diagnosis should be suspected in front of the very evocative clinical picture itself. The aspect of the tyndall phenomenon, and of the convex pseudo-hypopyon, with iris pearls and nodules, together with a peripheral, imprecisely limited, retinal mass, non calcified on ultrasonographic examination, are typical. Aqueous paracentesis shows increased levels of Lactic Dehydrogenase (LDH), but most of all the cytologic examination confirms the diagnosis. The only treatment is enucleation. The clinical picture which may simulate uveitis, especially as this particular type of retinoblastoma has a late onset.

[Diffuse infiltrating retinoblastoma]. / Le rétinoblastome infiltrant diffus.

We report 3 new cases of diffuse infiltrating retinoblastoma, and we review the 21 cases of the literature. Clinical features are typical but must not be confused with uveitis. The average age of onset is about 7 years, later than the usual retinoblastoma. Clinical features associate ocular redness, pseudo hypopion, iris nodules, clusters on the pupil and in the anterior chamber, opacities on the posterior face of the cornea. Hypertension appears resistant to medical treatment. The vitreous is hazy but the retina is still visible. The ophthalmoscopic examination reveals exudates covering the peripheric retina, and gray infiltrated retina. Usually there is no focal tumour mass, but totally diffuse tumoral infiltration. Echographic examination does not reveal calcification as in typical retinoblastoma. Neoplastic cells are demonstrated in anterior chamber paracentesis. Cytologic examination of aqueous humor aspirates may be misinterpreted and should be evaluated carefully. Because of tumor cells seeding, lactate dehydrogenase assay is of diagnostic value, prior to considering definitive therapy. None of the tumors were bilateral. The prognosis after enucleation appears good.

[Treatment of sequelae of basedowian exophthalmos]. / Traitement des séquelles de l'ophtalmopathie basedowienne.

Treatment of sequelae of ophthalmopathy of Basedow's disease, essentially exophthalmos, oculomotor disorders and upper eyelid retraction, is of particular importance because of the functional and esthetic problems presented. Improvement in the different symptoms can be obtained by surgical treatment, but patients must have been perfectly stable with respect to both the thyroid and ophthalmologic plan for at least 6 months. It marked exophthalmos exists this is treated initially by orbital enlargement. Oculomotor problems are then resolved and finally treatment is terminated by surgery to eyelids. Very satisfactory functional and esthetic results are obtained.

[Macular manifestations of monoclonal dysgammaglobulinemias. Apropos of 3 cases]. / Manifestations maculaires des dysglobulinémies monoclonales. A propos de trois cas.

Macular oedema is one of the scarcely known manifestations of monoclonal dysglobulinemia. It is most common in Waldenström's macroglobulinemia because the serum's viscosity may reach a very high level, but it occurs also in multiple myeloma (first case described here). This specific oedema is secondary to immunoglobulin deposits in retinal layers and subretinal space. Early treatment is necessary to prevent irreversible visual loss, especially because the patient's expected life span becomes lengthened. Plasmaphereses are very useful in reducing serum's hyperviscosity, and rapidly improve most of the ocular manifestations.

[Incidence of lipid changes in rhegmatogenous retinal detachment, retinal vein occlusion and primary chronic glaucoma]. / Incidence des altérations lipidiques au cours du décollement de rétine rhegmatogène, de l'occlusion veineuse rétinienne, du glaucome chronique primitif.

Several studies suggest that vascular factors may play a role in the pathogenesis of rhegmatogenous retinal detachment, retinal vein occlusion and primary chronic glaucoma. We explored the lipoprotein abnormalities in three groups of 45 patients suffering from each of these diseases compared to three control groups. We did not find significant abnormalities of cholesterol, cholesterol lipoproteins, nor Apolipoprotein B. The only significant abnormality was the presence of a higher rate of hypertriglyceridemia in the retinal vein occlusion and primary chronic glaucoma groups in comparison with their control group. In these two affections, hypertriglyceridemia should be systematically researched and treated.