Granulomatosis de wegener refractaria: rol terapéutico de rituximab: revisión de tres casos / Refractoy wegener's granulomatosis: therapeutic rol of rituximab

El tratamiento convencional para la granulomatosis de Wegener, que incluye esteroides y ciclofosfamida, no es suficiente para el control de la actividad de la enfermedad en algunos pacientes considerados refractarios y conlleva cierta toxicidad relacionada al mismo. La depleción de células B con rituximab ha demostrado ser efectivo en el tratamiento de varias enfermedades autoinmunes, incluyendo las vasculitis sistémicas asociadas a anticuerpos anticitoplasma de neutrófilos (ANCA). Reportamos tres casos de granulomatosis de Wegener refractarios tratados exitosamente con rituximab.

Conventional therapy for Wegener's granulomatosis, steroid and cyclophosphamide, fails to control disease activity in same refractorypatients and has treatment-related toxicity. B cell depletion therapyusing rituximab has been shown to be effective for certain autoimmunediseases, including antineutrophil cytoplasmic antibody (ANCA) associated vasculitis. We report three refractory cases of Wegener’s granulomatosis successfully treated with rituximab.

A Case of Wegener's Granulomatosis with Retinal Pigment Epithelial Detachment and Subretinal Hemorrhage

PURPOSE: To report a case of very limited Wegener's granulomatosis presenting with inflammation of the anterior segment, orbital pseudotumor, retinal pigment epithelial detachment, and subretinal hemorrhage. METHODS: A 54-year-old man who was treated for pachymeningitis was seen in consultation for painful swelling of the left eye and periorbit, and visual loss of the right eye developed 2 days after admission. He was diagnosed with peripheral keratitis, scleritis, anterior uveitis, and pseudotumor of the left eye, and RPE detachment in the macula of the right eye. He was started on treatment with antibiotics, NSAIDs, and steroids. Twelve days later, he had no response to the treatment and developed otitis media and skin ulcer. A skin biopsy was performed. RESULTS: Histological analysis showed a necrotizing inflammation of the small vessels in the skin lesion. The clinical and radiologic findings and the positive antineutrophil cytoplasmic antibody (ANCA) test were consistent with a diagnosis of Wegener's granulomatosis. Treatment with oral steroids and cyclophosphamide improved the systemic and ocular symptoms dramatically. One year later, he developed choroidal neovascularization and subretinal hemorrhage in the right eye. He was started on oral steroids and cyclophosphamide, with a dramatic improvement in his general health and ocular state.

A Case of Propylthiouracil Induced Anti: Neutrophil Cytoplasmic Antibody (ANCA) Positive Vasculitis / 대한내분비학회지

A 40-year-old woman just had started to take propylthiouracil for Graves disease, However, the treatment had to be interrupted because she developed skin rash, arthritis, chest pain, fever and proteinuria after 2 months. The serologic study revealed antineutrophil cytoplasmic antibody(ANCA) positivity, especially showing perinuclear pattern. The anti-myeloperoxidase titer was high. The hematoxylin & eosin stain of the specimen obtained from kidney was compatible with pauci-immune glomerulonephritis with crescent formation. There were no immune complex deposits under electron microscope. Such findings suggested propylthiouracil-induced vasculitis. Vasculitis is a rare side effect of propylthiouracil. Recently more cases of vasculitis associated with anti-thyroid drug with ANCA positivity are being reported up to about 36 cases worldwide. There are possibilities of underdiagnosis of this side effect, meaning more cautions on the patients under anti-thyroid drug treatment. We present a case with review of related literature.