I. Badelon

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. After follow-up for at least 20 months, the 2 patients still receiving infliximab remained event free. Infliximab enabled steroid tapering to less than 5 mg per day, suggesting that infliximab could constitute an alternative to conventional steroid sparing agents.

Atteintes ophtalmologiques de la sarcoïdose et des « sarcoid-like reaction » dans les déficits immunitaires. À propos de 4 cas

Ophthalmologic disease in sarcoid-like granulomatosis and true sarcoidosis in immunodeficiency. Four case reports C. Rohart, I. Badelon, F. Fajnkuchen, S. Nghiem-Buffet, G. Chaine 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.

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. Oral prednisone was initiated at 1 mg/kg daily and gradually tapered to a minimum effective dose. Efficacy was assessed on the basis of an objective observation of decreased swelling. Results: One patient displayed original uptake foci involving intrathoracic lymphadenopathies, the trajectory of the paraumbilical vein and perirectal fat. Low-dose prednisone was able to induce a durable response in the authors’ patients. Conclusions: Fluorodeoxyglucose positron emission tomography/CT may be useful for the diagnostic workup and follow-up assessment of patients with adult-onset asthma and periocular xanthogranuloma. Oral corticosteroids can be used successfully as first-line treatment in such patients.

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

AbstractAdult 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.To determine if AAPOX syndrome shares clinical, biological, and histopathological characteristics with IgG4-RD, we used the comprehensive clinical diagnostic criteria for IgG4-RD in a retrospective case series of three consecutive patients with histologically-proven AAPOX. Patients who were diagnosed with AAPOX at a French academic referral center for orbital inflammation between November 1996 and March 2013 were enrolled. Biopsies from ocular adnexa or other organs were systematically reexamined. For each patient, clinical and serological data, radiologic findings, and treatment were retrospectively analyzed.Two AAPOX patients fulfilled all of the diagnostic criteria for a definite IgG4-RD. One patient who lacked the serological criteria fulfilled the criteria of a probable IgG4-RD.These 3 cases of AAPOX patients fulfilled the IgG4-RD comprehensive clinical diagnostic criteria. To our knowledge, this is the first observational case report study to clearly show a strong relationship between IgG4-RD and AAPOX syndrome.

Chirurgie de la cataracte et dégénérescence maculaire liée à l’âge : bénéfice en termes d’acuité visuelle et de qualité de vie – étude prospective

Cataract surgery and age-related maculopathy: benefits in terms of visualacuity and quality of life – a prospective study C. Rohart, F. Fajnkuchen, S. Nghiem-Buffet, O. Abitbol, I. Badelon, G. Chaine 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 p 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.

IgG4-related diffuse perineural disease

A 55-year-old woman had right exophthalmia. Eleven years previously, she had orbital irradiation for refractory nonspecific orbital inflammation. PET/CT revealed FDG uptake in the right orbit and paravertebral masses (figure 1, A and C). MRI showed an enlargement of the right optic nerve and orbital muscles, and a diffuse infiltration involving lumbodorsal and sacral nerve roots (figure 1, B and D). The orbital biopsy demonstrated immunoglobulin G4 (IgG4)+ plasma cell infiltrate and a storiform fibrosis (figure 2), identical to the histopathologic features of the nerve root biopsy, and suggestive of IgG4-related diffuse perineural disease.1,2 No treatment was started in the absence of neurologic symptoms. One year later, the patient had no further symptoms.

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. The fundus examination revealed multiple retinal vascular occlusions, and a fluorescein angiography showed retinal and choroidal ischemia bilaterally. In addition, based on the neurologic disorders, a pleuritis, a renal disorder and a hematologic disorder, systemic lupus erythematosus, was diagnosed. Conclusions Severe vaso-occlusive retinopathy in a 26-year-old man resulting in a significant visual loss as the initial manifestation of systemic lupus erythematosus. Central nervous system involvement during lupus might be more frequent in patients when serious retinal changes occur.

Faut-il opérer de la cataracte les patients porteurs d'une dégénérescence maculaire liée à l'âge ?

But de l’etude Suivi de l’evolution de l’acuite visuelle (AV) et des lesions du fond d’oeil apres chirurgie de la cataracte chez les patients atteints de degenerescence maculaire liee a l’âge (DMLA). Materiel et methode Quarante yeux de 25 patients atteints DMLA ont ete operes de la cataracte. Les yeux ont ete repartis en deux groupes en fonction du stade evolutif de DMLA : le groupe 1 presente des formes non compliquees : drusen et alterations de l’epithelium pigmentaire ; le groupe 2 presente des formes evoluees avec une atrophie et/ou des neovaisseaux. Resultats Dans le groupe 1, 15 yeux ont ete operes avec une amelioration de l’acuite visuelle dans 93,3 % des cas. Un an apres l’intervention, on retrouvait des lesions neovasculaires dans 1 œil (6,7 %). Dans le groupe 2, 25 yeux ont ete operes. Les lesions initiales etaient atrophiques dans 20 yeux (80 %) et neovasculaires dans 5 yeux (20 %). Soixante-huit pour cent des yeux ont presente une amelioration de leur acuite visuelle. Conclusion d’apres notre etude et la litterature, les patients ayant une DMLA beneficient de la chirurgie de la cataracte en termes d’acuite visuelle et de qualite de vie. Il est difficile de conclure concernant les risques d’evolution des lesions du fond d’œil a court terme, bien qu’il semble que la chirurgie ait un role deletere a long terme (5 ans) [1,2].

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. RESULTS The presenting feature was bilateral loss of vision, a result of malignant hypertensive retinopathy and choroidopathy. Systemic evaluation disclosed inflammatory signs and renal artery obstruction which caused the malignant hypertension. CONCLUSION This case, involving an unusual presentation of Takayasu disease, illustrates how a malignant hypertensive retinopathy and choroidopathy led to the ultimate diagnosis of Takayasu disease.