[Management of orbital inflammation in internal medicine: a retrospective case series of 29 patients]. / Prise en charge de l'inflammation orbitaire en médecine interne : à propos d'une série de 29 patients consécutifs.

PURPOSE: To describe a case series of patients investigated in internal medicine for an inflammation of the orbit and to clarify the clinical and pathological features of patients with idiopathic orbital inflammatory syndrome (IOIS). PATIENTS AND METHODS: Forty patients were consecutively referred by a specialized center where an orbital biopsy was performed in case of accessible lesion. Eleven patients were excluded because of missing data or diagnosis of lymphomas, periorbital xanthogranulomas, or Graves' disease. Patients with systemic disease (SD) or auto-immune disorder (AID) that validated the international criteria, or those having an IOIS in the absence of local or systemic etiology, were included. The clinicopathologic and immunologic characteristics of IOIS patients, their treatment and their evolutionary profiles are reported according to the histological types described by Mombaerts. RESULTS: Of the 29 patients enrolled, eight had a dacryoadenitis revealing a SD/AID, mainly a necrotizing vasculitis, seven patients had a presumed IOIS and 14 an IOIS histologically documented. The presentation of IOIS was dominated by a diffuse involvement of the orbit. Corticosteroids were administered alone or with an immunosuppressant in 57 and 24% of IOIS patients, respectively. The incidence of relapse/resistance was higher than that of remission, particularly in case of presumed IOIS or in its classical form. All four patients with a stage III-IV of Chisholm were relapsing or resistant. CONCLUSION: A dacryoadenitis may reveal some types of SD/AID. Unlike the severe sialadenitis, the form of sclerosing IOIS may not be a factor associated with relapse or resistance.

[Basedow exophthalmos]. / L'exophtalmie basedowienne.

Dysthyroid orbitopathy is a disease that is edematous and inflammatory, generally chronic, sometimes subacute or acute, and is characterized by exophthalmos in the majority of cases, retraction of the lid, restrictive strabismus with diplopia, corneal ulceration, ocular hypertension, and compressive optic neuropathy. Proptosis is the cardinal sign. Exophthalmos is frequently axial and is bilateral in 85%-90% of cases. Displacement of the globe in the vertical and horizontal axes is not rare, particularly when intense enlargement of an extraocular muscle produces a mass effect. Proptosis stems from a conflict for the space in the orbital cavity. The infiltration of the fat, muscles, and lacrimal gland by lymphocytes, plasma cells, and mucopolysaccharides, which are very hydrophilic, all contribute to the orbitopathy. The majority of patients with minimal exophthalmos do not require special treatment since they tend to improve spontaneously. In severe forms, it is important to evaluate the activity for steroid use to eliminate the inflammation in the soft tissues and to make rehabilitative surgery possible under better conditions. When a good response is not obtained, radiotherapy is evaluated. The surgical treatment by bone orbital decompression and sometimes lipectomy is indicated in inactive forms, disfiguring exophthalmos, certain particular cases such as dysthyroid optic neuropathy, ocular hypertension, corneal exposition.

[Ophthalmologic disease in sarcoid-like granulomatosis and true sarcoidosis in immunodeficiency. Four case reports]. / Atteintes ophtalmologiques de la sarcoïdose et des "sarcoid-like reaction" dans les déficits immunitaires. A propos de 4 cas.

Granulomatosis lesions occurring after diagnosis of primary or secondary immunodeficiency are not accidental and have been described in a small number of patients suffering from various diseases: common variable immunodeficiency (CVID), malignancy (lymphoma and solid tumors), and acquired immunodeficiency syndrome (AIDS). Two types of granulomatosis can appear: true sarcoidosis and sarcoid-like reaction. We report four patients, two with CVID and two with malignancy, in whom clinical granulomatosis appeared a few months to a few years after diagnosis of immunodeficiency. They developed noncaseating granulomas of the lung, spleen and liver associated with conjunctival granulomas and bilateral panuveitis. The granulomatous disorder was diagnosed after immunodeficiency on histopathological studies revealing noncaseating granulomas. Causation agents such as infectious organisms and environmental compounds were excluded. The relationship between sarcoid-like reaction, true sarcoidosis and immunodeficiency is discussed. The underlying pathophysiology responsible for the association between granuloma formation and immunodeficiency in the same patient remains obscure. It may be quite difficult to distinguish true sarcoidosis and sarcoid-like reaction. It is possible that these two entities are the clinical extremes of a common pathological process.

[Cataract surgery and age-related maculopathy: benefits in terms of visual acuity and quality of life--a prospective study]. / Chirurgie de la cataracte et dégénérescence maculaire liée à l'âge: bénéfice en termes d'acuité visuelle et de qualité de vie--étude prospective.

PURPOSE: To assess the benefits of cataract surgery in patients with age-related macular degeneration (AMD) in terms of visual acuity and quality of life. PATIENTS AND METHODS: Forty-two eyes of 30 patients were included in this prospective study between October 2003 and January 2005. The eyes were divided into two groups: the first group (n=12) with geographic atrophy and neovascularization (late-stage AMD) and the second group (n=30) with drusen and retinal pigment epithelium abnormalities (early-stage AMD). Assessment included best corrected visual acuity (BCVA) and quality-of-life measures (VF-14 questionnaire) before surgery and BCVA and the VF-14 score after surgery. RESULTS: In the first group, there was no statistical difference but a trend toward improvement in BCVA (p>0.05). In terms of quality of life, there was a statistical difference between pre- and postoperative VF-14 (p=0.0078). In the second group, there was a statistical difference between pre- and postoperative BVCA (p<0.0001). In terms of quality of life, there was a statistical difference between pre- and postoperative VF-14 (p<0.0001). CONCLUSION: This study shows a benefit in terms of visual acuity and quality of life in the majority of patients with age-related maculopathy after cataract surgery, even in late-stage AMD. Indeed, we noted a marked improvement in visual acuity in cases of early-stage AMD and a slight improvement of visual acuity in late-stage AMD. Quality of life was improved at both stages. In conclusion, cataract surgery is justified in patients with AMD.

[Neuro-ophthalmologic initial presentation of sarcoidosis]. / Manifestations neuro-ophtalmologiques révélatrices d'une neuro-sarcoïdose.

PURPOSE: To describe different forms of neuro-ophthalmologic onset of sarcoidosis: clinical signs, means of diagnosis, treatment, and progression. PATIENTS AND METHODS: Retrospective study of 13 patients with neuro-ophthalmologic initial onset of sarcoidosis diagnosed in three departments between 1997 and 2003. RESULTS: There were ten women and three men, with a mean age of 36 years. Six patients suffered from diplopia. In three cases, the cavernous sinus was involved; the three other patients with diplopia had meningoradiculitis. Nine patients had infiltration of the anterior visual pathway: the optic nerve was involved in five cases, the chiasm in two cases, and two patients had papilledema. Two patients also had both symptoms. The dosage of the angiotensin-converting enzyme level was evaluated in 11 patients and was elevated in six cases. Nine patients underwent a lumbar puncture; the cerebrospinal fluid protein was high in seven cases. Chest radiography and CT were abnormal in nine cases of 11. Ten patients had histological proof of sarcoidosis; the three others had enough evidence to support this diagnosis. All of them were treated with systemic corticosteroids. The diplopia improved for the six patients. Among the seven patients with optic nerve or chiasmal infiltration, one recovered completely, two were partially improved, and four remained stable. CONCLUSIONS: Diplopia and anterior visual pathway abnormalities can be the manifestation of initial onset of sarcoidosis; therefore this diagnosis must be kept in mind when these frequent neuro-ophthalmologic signs are encountered. Complementary exams, mainly biopsy of the involved areas with histological analysis, are needed to confirm this diagnosis. Corticosteroid treatment is generally followed by improvement, but relapses may occur.

[Should patients with age-related macular degeneration have cataract surgery?]. / Faut-il opérer de la cataracte les patients porteurs d'une dégénérescence maculaire liée à l'âge?

PURPOSE: To study the progression of visual acuity (VA) and retinal abnormalities in patients with age-related macular degeneration (AMD) after cataract surgery. METHODS: Forty eyes of 25 patients with AMD who had had cataract surgery were included in the study. They were divided up into two groups according to AMD stage: the first group with drusen and retinal pigmented epithelium abnormalities and the second in the severe stages of age-related macular degeneration with atrophy and neovascularization. RESULTS: In the first group (15 eyes), we found an improvement in VA in 93.3% of the eyes. New manifestations of neovascularization appeared in only one eye (6.7%) 1 year after surgery. In the second group (25 eyes), we found atrophy in 80% of the eyes and neovascularization in 20% of the eyes before surgery. There was an improvement in VA after cataract surgery in 68% of the eyes, and in three eyes (17%) VA worsened. CONCLUSION: In our study, patients with AMD improved VA and quality of life after cataract surgery. The same results of VA improvement after surgery can be found in the literature. However, no conclusions can be made concerning the progression of fundus lesions on a short term, though it seems that cataract surgery may accelerate the progression of AMD lesions 5 years after surgery.

Infliximab in refractory uveitis due to Behçet's disease.

OBJECTIVE: To report 4 cases of refractory panuveitis due to Behçet's disease treated with a novel therapy: infliximab. METHODS: Retrospective study of 3 women and 1 man of Causasian origin with Behçet's disease complicated with panuveitis. Their uveitis was relapsing from 48 to 96 months and was resistant to the combination of colchicine (n = 4), high-dose prednisone (n = 4), pentoxyphilline (n = 2) and various immunossuppressors and/or immunomodulators given successively: intravenous cyclophosphamide (n = 4), azathioprine (n = 3), interferon alpha (n = 3), cyclosporine A (n = 2), oral cyclophosphamide (n = 1), mycophenolate mofetil (n = 1), methotrexate (n = 1), high-dose immunoglobulin (n = 1). Combination with respectively 1, 3, 4 and 5 immunossuppressors and/or immunomodulators failed before institution of infliximab. After informed consent was obtained, infliximab was administered as a single infusion of 5 mg/kg (maximum dose: 400 mg) at day 1, at week 2, 6 and then every 8 weeks. RESULTS: With a follow-up ranging from 7 to 22 months, infliximab was efficient in all cases. The mean prednisone dose decreased from 45 mg to 13 mg daily. Total recovery of visual acuity was observed in half of the cases. Infliximab was well tolerated without fever, severe sepsis or autoimmune manifestation. CONCLUSION: Infliximab may be efficient in refractory uveitis due to Behçet's disease. The optimal dose, rhythm and duration of infliximab infusions need to be standardized.

[Medical treatment of dysthyroid orbitopathy]. / Traitement médical de l'orbitopathie dysthyroïdienne.

The majority of Graves' patients have mild and nonprogressive ocular involvement that does not require aggressive treatment. Local supportive measures such as wearing tinted lenses, proper eyelid hygiene, and the use of wetting agents or gels are usually sufficient to obtain symptomatic relief until eye disease becomes inactive. Smoking must be stopped and euthyroidism controlled. Management of severe forms (10% of patients) is often difficult and does not provide consistently favorable results. The use of glucocorticoids is a well-established method of treatment, indicated in severe inflammatory forms as well as in complicated forms of optic neuropathy, ocular hypertension, and corneal exposure, and are used orally or intravenously, at high doses and for prolonged periods of time. Prisms are useful in cases of diplopia. Favorable effects are reported in slightly more than 60% of cases. Glucocorticoids can be combined with orbital radiotherapy in patients with severe orbitopathy.

[Retinal arteritis in a case of sarcoidosis. A case report]. / Artérite rétinienne dans un cas de sarcoïdose.

Retinal vasculitis is a common finding and occurs in 10% - 83% of patients with sarcoidosis. In most cases, there are bilateral, peripheral and nonischemic periphlebitis. In contrast, periarterial involvement is rarely observed. We report a case of retinal vasculitis associated with sarcoidosis and its differential diagnosis. Sarcoidosis should not be excluded in case of periarteritis.

[Peripheral retinal neovascularization and sarcoidosis: two case reports]. / Néovascularisation périphérique et sarcoïdose.

Peripheral retinal neovascularization occurs in approximately 10% of cases of sarcoidosis. Its pathogenesis is unknown, but it probably results from retinal ischemia and/or inflammation. In cases of peripheral retinal neovascularization associated with sarcoidosis, sickle cell disease should be considered, even if sarcoidosis is histologically proved: new vessels seen in sickle cell disease and sarcoidosis may have a very similar pattern. We present two cases with histologically proven sarcoidosis who developed peripheral neovascularization: the first one had no associated disease and new vessels were likely to be related to sarcoidosis; in the second case, hemoglobin electrophoresis revealed hemoglobin SC, and provided diagnosis of sickle cell hemoglobinopathy.

[Ophthalmologic manifestations of sarcoidosis]. / Les manifestations ophtalmologiques de la sarcoïdose.

Ophthalmologic manifestations of sarcoidosis are polymorphous and can involve all the ocular structures. Benign conjunctive and lacrimal gland involvement predominates. Uveitis is found in 20% of the patients and is often torpid, becoming chronic. Systematic screening for uveitis is warranted in sarcoidosis patients. neuro-ophthalmologic involvement is exceptional and severe, raising major diagnostic difficulties when isolated.

[Central serous chorioretinopathy and systemic steroid therapy]. / Choriorétinite séreuse centrale et corticothérapie systémique. A propos de 14 cas.

BACKGROUND: The manifestations of the ocular toxicity of systemic corticosteroids include posterior subcapsular cataracts and glaucoma. We describe 14 cases of serous detachment of the macula due to central serous chorioretinopathy in patients given long-term steroid therapy, which may be another potential ocular side effect of corticosteroid. CASES REPORT: The 14 (9 men and 5 women) patients were aged from 39 to 55 year old. Their systemic diseases were allergic thrombopenic purpura, optic neuritis, kidney or heart transplant, Churg and Strauss vasculitis, facial palsy, rheumatoid arthritis, systemic lupus and a kidney tumor. None of the patients had hypertension. RESULTS: Serous detachment occurred between 6 days and 10 years after the start of steroid treatment. The higher the doses, the earlier the onset of ocular disease. All patients were symptomatic, with rapid onset of blurred vision. Serous detachment was bilateral in two cases. The fluorescein angiographic finding was in most cases a single small focal hyperfluorescent leak from the retinal pigment epithelium which appeared early in the angiogram and increased in size and intensity. No diffuse degradation of the retinal pigment epithelium was seen on the fluorescein angiogram. Five patients underwent laser photocoagulation of the leaking area followed by resorption of subretinal fluid. In other patients, the symptoms disappeared as the doses of steroid were reduced. CONCLUSION: The pathogenesis of central serous chorioretinopathy remains unclear and is controversial. Corticosteroids are known to worsen the prognosis of idiopathic central serous chorioretinopathy, and serous detachment has been reported after renal transplantation. In most of these cases, chorioretinopathy was combined with diffuse leakage from the choriocapillaris. We discuss the relationship between steroid therapy and focal leakage as seen in idiopathic central serous chorioretinopathy. In conclusion, we describe 14 cases of central serous retinopathy whose clinical and fluorescein angiography were fairly typical, without obvious diffuse degradation of the retinal pigment epithelium. All these patients had been given long-term steroid therapy for various diseases.

[Retinal vascularization in sarcoidosis]. / Vascularite rétinienne au cours de la sarcoïdose.

OBJECTIVES: To describe the characteristics of retinal vasculitis related to sarcoidosis, and to study the radio-clinical findings of sarcoidosis in case of retinal vasculitis. METHODS: We performed a retrospective study on 33 cases of retinal vasculitis associated with sarcoidosis. Our patients had a complete ophthalmological examination and a systematic fluorescein angiography was performed. Recorded data included clinical, biological and radiological evaluation of sarcoidosis. RESULTS: Retinal vasculitis were and remained asymptomatic in almost a third of the patients (n = 10). In 11 patients (33%) a decreased visual acuity of more than 3 lines was observed. Periphlebitis was observed in all cases, and 75.7% were of non ischemic forms. Thirty patients out of 33 (90.9%) were treated with systemic steroids in whom 17 were treated for ophthalmological reasons. The presence of anterior or posterior uveitis, more than one attack of vasculitis, and significant hyalitis were statistically associated with visual acuity decrease. Ninety percent of patients regained their initial visual acuity, only three patients at the end of the medical follow-up remained with a visual acuity below or equal to 2/10. Thirty one patients (91%) presented with mediastinal and pulmonary signs concordant with sarcoidosis on chest x-rays. Extrapulmonary localisations were common in case of retinal vasculitis (91% of patients). CONCLUSION: Retinal vasculitis associated with sarcoidosis are often asymptomatic. In most cases, there are bilateral, non ischaemic periphlebitis. They seem to have a relatively better prognosis than in other etiologies of vasculitis and they are part of an evolutive multisystemic sarcoidosis. Most of the time treatment with systemic steroids was indicated for ophthalmological or extra ophthalmological reasons.

[Cystoid macular edema and cytomegalovirus retinitis in AIDS patients treated with antiretroviral triple therapy]. / Oedème maculaire cystoïde et rétinite à cytomégalovirus chez des patients HIV traités par trithérapie antirétrovirale.

AIM OF THE STUDY: This study evaluated cystoid macular edema (CME) occurring during inactive cytomegalovirus (CMV) retinitis, in AIDS patients treated with highly active antiretroviral therapy (HAART) and without anti CMV treatment for 10 patients. PATIENTS AND METHODS: 12 patients were followed over 24 months. Assessment was carried out on visual acuity, biomicroscopy, fundus photographs every month, angiography for each patient, CD4 cell count and plasmatic viral load. RESULTS: 7 patients presented CME in association with chronic uveitis, large CMV retinitis scars and posterior pole involvement. CONCLUSION: CME is a new complication of CMV retinitis occurring in patients treated with HAART.

[Eye and spondylarthropathies]. / Oeil et spondylarthropathies.

Eye involvement can occur in the course of ankylosing spondylitis and other spondylarthropathies (reactive arthritis, enteropathic and psoriatic rheumatism). The most frequent presentation is an acute anterior, unilateral, relapsing, sometimes in the contralateral eye uveitis. Local treatment that associates corticosteroids and cycloplegia usually leads to a complete healing, without sequelae. Involvement of other ocular structures is rare in spondylarthropathies.

[Course of cytomegalovirus retinitis in HIV positive patients treated with triple antiretroviral therapy]. / Evolution de la rétinite à cytomégalovirus chez des patients HIV sous trithérapie antirétrovirale.

PURPOSE: The recent developments of protease inhibitors raise big hopes for the fight against AIDS. Combined in a triple therapy with 2 nucleoside analogs, those molecules strongly inhibit human immunodeficiency virus (HIV) replication. Their biological and clinical efficacy for increasing CD4 cell count and survival time has been proved. We have followed evolution of Cytomegalovirus (CMV) retinitis in 12 patients treated by triple antiretroviral combination therapy since April 1996. PATIENTS AND METHODS: Twelve AIDS patients with CMV retinitis background and a treatment by triple therapy were followed in this prospective study. At the onset of combination therapy, retinitis was cicatricial in 10 patients and active in 2 patients. Mean CD4 cell count was 23 +/- 16 CD4/microL [4-50]. Follow-up included ophthalmoscopic examination every 2 weeks and fundus photographs every month. CD4 cell count was noted at each exam. RESULTS: The mean time follow-up after onset of combination therapy was 10.3 +/- 2.4 months [5-14]. Retinitis relapsed in 6 patients (50%) within the 3 first months, and with a CD4 cells count higher than 75/microL for 3 patients. Inflammation response was noted in 3 patients. There was no recurrence after the third month, except in 1 case at the 9th month. CD4 cell count was 122 +/- 41 CD4/microL [30-350] after 6 months. As retinal lesions were completely still for some patients, maintenance therapy was reduced, then even stopped. At the end of follow-up, maintenance therapy was stopped for 8 patients. For these patients, retinitis remained stable without maintenance therapy for a mean time of 4,5 months (10 months in 1 case). Recurrence occurred for only 1 patient after 9 months of triple therapy and 2 months without maintenance therapy. For the 11 remaining patients, retinitis remained stable for a mean total time of 9.5 +/- 2.5 months [4.5-12.5]. CONCLUSION: Those first results show that clinical and therapeutic history of CMV retinitis may be changing. There seems to be a restoration of immune defenses that allowed suppression of maintenance therapy for 8 out of 12 patients. Our results need to be confirmed by a longer follow-up and multicentric studies.

[Eye manifestations of sarcoidosis]. / Manifestations ophtalmologiques de la sarcoïdose.

Ophthalmologic manifestations of sarcoidosis are multiform, and all parts of the structure of the eyeball can be affected. Involvement of the conjunctiva and of the lacrimal glands is the most frequent and is benign. Uveitis is observed in 20% of patients. It is generally torpid, evolving in chronic fashion. The possibility of uveitis requires routine investigation in all patients with sarcoidosis. Neuro-ophthalmologic involvement is rare but severe. When isolated, it presents difficult problems of diagnosis.

[Oculomotor paralysis in disseminated lupus erythematosis]. / Paralysies oculomotrices dans le lupus érythémateux disséminé.

The inferior oblique isolated damage in the systemic lupus erythematosus is being described. The ocular palsies are few and exceptionally isolated. Few studies have been published on the damage topography which can relate to micro-infarcts, or focal vasculitis. The involvement is either central or peripheral. The diagnosis by NMR is more reliable than by TDM.

[A case of MEWDS. "The multiple evanescent white-dot syndrome"]. / A propos d'un cas de MEWDS. "Syndrome des taches blanches rétiniennes fugaces".

A young white man developed acute bilateral visual loss with no previous general illness. Ophthalmoscopic examination showed multiple small yellow-white lesions scattered throughout the posterior poles and mild periphery fundus. There was also fine granularity of two foveal areas and one optic disc margin was blurred. Fluorescein angiography showed early hyperfluorescence of the lesions and late staining of the retinal pigment epithelium. Electrophysiologic abnormalities were transient, asymmetric, more marked in photopic than in scotopic. The origin could be in retinal bipolar cells. These lesions regressed, with return of normal visual function within several weeks. These clinical findings are different from others acute inflammatory diseases primarily involving retinal pigment epithelium and photoreceptors. This aspect is usually described as "multiple evanescent white dot syndrome". The etiology of this syndrome remains unknown with no evidence of systemic disease. A history of flulike illness is rare.