Adult Onset Asthma and Periocular Xanthogranuloma (AAPOX), a Rare Entity With a Strong Link to IgG4-Related Disease: An Observational Case Report Study.

Adult onset asthma and periocular xanthogranuloma (AAPOX) is a rare non-Langerhans histiocytosis characterized histopathologically by a periocular infiltration of foamy histiocytes and Touton giant cells. Benign hyperplasia with plasma cell infiltration is classically described in eyelids or lymph nodes of AAPOX patients. It is also a characteristic feature of IgG4-related disease (IgG4-RD), a new entity defined by an IgG4-bearing plasma cell infiltration of organs.

Adult-onset asthma associated with periocular xanthogranuloma: new diagnostic and therapeutic approaches in a very rare systemic disease.

Purpose: To report some novel findings concerning the systemic manifestations and treatment of adult-onset asthma and periocular xanthogranuloma, a rare type of non-Langerhans histiocytosis that can lead to important visual dysfunction.

Methods: A retrospective case series of 2 patients was evaluated for orbital and systemic manifestations using fluorodeoxyglucose positron emission tomography/CT and/or orbital MRI. Histological specimens were reviewed in all patients.

Inaugural severe vaso-occlusive retinopathy in systemic lupus erythematosus.

Purpose To report the case of a patient with an inaugural severe bilateral vaso-occlusive retinopathy due to systemic lupus erythematosus. Method Clinical examination, fundus pictures and fluorescein angiography were performed. Results A 26-year-old, healthy, African man presented with a meningo-encephalitic syndrome and a severe bilateral visual impairment.

[Management of orbital inflammation in internal medicine: a retrospective case series of 29 patients].

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.

[Basedow exophthalmos].

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.

Takayasu disease revealed by bilateral loss of vision.

Purpose: Takayasu arteritis is a nonspecific granulomatous inflammatory arteriopathy of unknown cause, most frequently diagnosed in young Asian women. The authors present an atypical initial presentation of Takayasu disease with bilateral loss of vision in a young Haitian man.

Methods: Case report.

[Ophthalmologic disease in sarcoid-like granulomatosis and true sarcoidosis in immunodeficiency. Four case reports].

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.

Successful treatment of idiopathic orbital inflammation with infliximab: an alternative to conventional steroid-sparing agents.

Therapy for orbital pseudotumor, also called idiopathic orbital inflammation, is nonspecific and mainly based on the use of corticosteroids. Two patients with steroid-dependent idiopathic orbital inflammation, one with myositis and the other with dacryoadenitis, and intolerant of standard steroid sparing agents (methotrexate or azathioprine), were treated with infliximab, a monoclonal antitumor necrosis factor alpha antibody. In both patients, orbital manifestations disappeared following treatment.

[Cataract surgery and age-related maculopathy: benefits in terms of visual acuity and quality of life--a prospective study].

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).

[Neuro-ophthalmologic initial presentation of sarcoidosis].

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.

[Graves' ophthalmopathy].

Graves' ophthalmopathy (GO) is a chronic autoimmune process that affects the retrobulbar tissue and has strong etiological links with graves' disease. Pathogenesis is incompletely understood. Symptoms include proptosis, extraocular muscle dysfunction, eyelid swelling and retraction.

[Should patients with age-related macular degeneration have cataract surgery?].

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.

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).

[Medical treatment of dysthyroid orbitopathy].

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.

[Retinal arteritis in a case of sarcoidosis. A case report].

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.

[Peripheral retinal neovascularization and sarcoidosis: two case reports].

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.

[Ophthalmologic manifestations of sarcoidosis].

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.

[Central serous chorioretinopathy and systemic steroid therapy].

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.

[Retinal vascularization in sarcoidosis].

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.

[Eye and 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.

[Cystoid macular edema and cytomegalovirus retinitis in AIDS patients treated with antiretroviral triple therapy].

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.

[Course of cytomegalovirus retinitis in HIV positive patients treated with triple antiretroviral therapy].

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.

[Eye manifestations of sarcoidosis].

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.