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.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.

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.

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.

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.

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.

[Graves' ophthalmopathy]. / Orbitopathie dysthyroïdienne.

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. In its severe expression, it is a disfiguring and potentially sight threatening disease that profoundly influences and impairs the quality of life of affected individuals. Only a minority of patients develop severe expressions of the disease, requiring aggressive treatments. Glucocorticoids, orbital radiotherapy and decompressive orbital surgery represent a mainstay and a well established treatment for severe and active GO.