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Santi Maria Recupero - One of the best experts on this subject based on the ideXlab platform.
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Ocular Inflammation: Can It Be a Sign of Activity of Weber-Christian Disease? A Case Report and Review of Literature
Ocular immunology and inflammation, 2014Co-Authors: Sara Verrilli, Angela Ciarnella, Bruno Laganà, Silvia Calafiore, Fabio Guglielmelli, Luisanna Basile, Maria Giulia Mutolo, Santi Maria RecuperoAbstract:AbstractPurpose: To present a case of Weber-Christian Disease with symptomatic ocular involvment. Weber-Christian Disease is a relapsing febrile nodular nonsuppurative panniculitis. It is characterized by malaise and fever accompanied by subcutaneous inflammatory nodules on the trunk and extremities. It can affect several organs, but ocular signs have been infrequently described in literature. Methods: A 20-year-old woman with Weber-Christian Disease presented with severe bilateral ocular inflammation. A complete ophthalmologic examination was performed. Results: Visual acuity was 20/100 in both eyes and slit-lamp examination showed bilateral iridocyclitis. Bilateral cortico-nuclear cataract did not allow funduscopy and she underwent cataract extraction. Retinal vasculitis was detected. Conclusions: Patients with Weber-Christian Disease can develop severe ocular inflammation. A complete ophthalmolgic examination should be done in these patients, especially when ocular involvement is the main sign of the d...
Don O. Kikkawa - One of the best experts on this subject based on the ideXlab platform.
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Proptosis as the Manifesting Sign of Weber-Christian Disease
American journal of ophthalmology, 1997Co-Authors: James N. Cook, Don O. KikkawaAbstract:Purpose To identify proptosis as a manifesting sign of Weber-Christian Disease (recurrent febrile nodular panniculitis). Method Case report. A 61-year-old man had signs of right proptosis and orbital inflammation that resolved with oral corticosteroid therapy. Orbital inflammation later recurred with associated cutaneous nodules, myalgia, nausea, and malaise. Results Rheumatologic evaluation and subcutaneous nodule biopsy led to the diagnosis of Weber-Christian Disease. The patient required systemic immunosuppressive agents to control the Disease. Conclusion This case shows the rare finding of proptosis as the manifesting sign of Weber-Christian Disease.
S.g. Radenska-lopovok - One of the best experts on this subject based on the ideXlab platform.
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THU0493 Approaches to Treatment of Weber-Christian Disease
Annals of the Rheumatic Diseases, 2013Co-Authors: B.s. Belov, O.n. Egorova, S.g. Radenska-lopovokAbstract:Background Weber-Christian Disease (WCD) is a rare understudied Disease, which manifests itself in recurrent subcutaneous fatnecrosis often involving damage of the internals. Treatment of the Disease is not standardized, and as a matter of fact it is provided empirically. Objectives to assess the efficiency of therapy in case of various forms of WCD Methods We studied 31 patients (29 women, 2 men) with WBD, age 30-74, duration of Disease 8.1±2.4 years. Besides general clinical examination we determined the level of α1-antitrypsin, amylase, lipase, ferritin, creatine phosphokinase, made chest CT scan and carried out the pathomorphology study of skin biopsy specimen taken from the area of a node. The following forms of the Disease were identified: nodular form (NF) (14 patients), plaque form (PF) (12 patients) and infiltrative form (IF) (5 patients). In all cases the lobular panniculitis was verified by means of histopathological examination. All patients took glucocorticoids (GC), average daily dose of 12.8 ± 1.6 mg, including 5.7± 1.4 mg (for NF), 16.6± 1.2 mg (for PF), 17.6 ± 1.7 mg (for IF). In case of NF we additionally prescribed Hydroxychloroquine, the dose of 600 mg per day (8 patients), and Azathioprine, the dose of 100 mg per day (4 patients); in case of PF – Cyclophosphamide (Cph), the dose of 200 mg per week (10 patients) or Azathioprine (150 mg per day, 2 patients). Patients with IF took Cph (200 mg per week). In case there was no effect after 3 months, we prescribed Mycophenolate Mofetil (1.5-2 g per day). On average, the observation period was 6 months. Results The treatment made it possible to achieve a significant positive dynamics in patients with NF and PF: the body temperature got back to normal and there was regression of indurations (26 patients), joint pain intensity was reduced (14 patients), laboratory data on inflammatory intensity revealed decrease (18 patients). Average daily dose of GC was reduced to 4.5±0.7 mg and 11.4±1.1 mg, respectively. In general, a significant improvement was evident in all patients with NF and in 75 % of patients with PF. In case of IF an average daily dose was increased to 25.4±1.7 mg per day. The treatment effect was satisfactory for 2 patients of this group, although it was unsatisfactory for 3 patients. Thus, the need to consider prescribing genetically engineered biological therapy was urged. Treatment tolerance was found good, no adverse events were registered. Conclusions To work out dosages and treatment schedules for patients with WCD further studies are required. Disclosure of Interest None Declared
Sara Verrilli - One of the best experts on this subject based on the ideXlab platform.
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Ocular Inflammation: Can It Be a Sign of Activity of Weber-Christian Disease? A Case Report and Review of Literature
Ocular immunology and inflammation, 2014Co-Authors: Sara Verrilli, Angela Ciarnella, Bruno Laganà, Silvia Calafiore, Fabio Guglielmelli, Luisanna Basile, Maria Giulia Mutolo, Santi Maria RecuperoAbstract:AbstractPurpose: To present a case of Weber-Christian Disease with symptomatic ocular involvment. Weber-Christian Disease is a relapsing febrile nodular nonsuppurative panniculitis. It is characterized by malaise and fever accompanied by subcutaneous inflammatory nodules on the trunk and extremities. It can affect several organs, but ocular signs have been infrequently described in literature. Methods: A 20-year-old woman with Weber-Christian Disease presented with severe bilateral ocular inflammation. A complete ophthalmologic examination was performed. Results: Visual acuity was 20/100 in both eyes and slit-lamp examination showed bilateral iridocyclitis. Bilateral cortico-nuclear cataract did not allow funduscopy and she underwent cataract extraction. Retinal vasculitis was detected. Conclusions: Patients with Weber-Christian Disease can develop severe ocular inflammation. A complete ophthalmolgic examination should be done in these patients, especially when ocular involvement is the main sign of the d...
Petros P. Sfikakis - One of the best experts on this subject based on the ideXlab platform.
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Orbital lobular panniculitis in Weber-Christian Disease: sustained response to anti-TNF treatment and review of the literature.
Survey of ophthalmology, 2010Co-Authors: Ioannis Mavrikakis, Thomas Georgiadis, Kalliopi Fragiadaki, Petros P. SfikakisAbstract:Abstract Weber-Christian Disease is a febrile, relapsing, non-suppurative panniculitis of unknown etiology. Lobular panniculitis is the essential feature in biopsy specimens and evolves through three recognizable stages. We report a case of Weber-Christian Disease with bilateral orbital involvement, at different stages, affecting the orbital fat along with enophthalmos in one orbit, and the upper preaponeurotic fat pad in the other. Weber-Christian Disease was refractory to treatment with conventional immunosuppressive regimens; however, early inflammatory—but not chronic fibrotic—orbital lesions responded dramatically to anti-tumor necrosis factor (TNF) therapy. A literature review revealed five additional cases of orbital Weber-Christian Disease, none treated with anti-TNF antibodies. Of these, four presented initially with proptosis, representing early stages of inflammation, and two subsequently developed enophthalmos, representing late, inactive stage of the Disease. Although orbital Weber-Christian Disease is rare, ophthalmologists need to be aware of this entity. Depending on the stage of inflammation, Weber-Christian Disease should be included in the differential diagnosis of both proptosis and enophthalmos. Anti-TNF antibodies can successfully treat patients at the early inflammatory stage.