GRANULOMATOSES SYSTEMIQUES PDF

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Background: Although peripheral nervous system involvement is common in eosinophilic granulomatosis with polyangiitis EGPA , central nervous system CNS manifestations are poorly described. We also undertook a systematic literature review. Results: We analyzed 26 personal cases and 62 previously reported cases. After a mean follow-up of 36months, 11 patients died including 5 from intracerebral hemorrhages.

Conclusion: EGPA-related CNS manifestations form 4 distinct neurological pictures: ischemic lesions, intracerebral hemorrhages, cranial nerve palsies and loss of visual acuity.

Such manifestation should prompt practitioners to consider EGPA in such conditions. Long-term neurological sequelae were common, and intracerebral hemorrhages had the worst prognostic impact. Keywords: Cerebral hemorrhage; Cranial nerve palsy; Eosinophilic granulomatosis with polyangiitis; Optic neuritis; Stroke. This site needs JavaScript to work properly. Please enable it to take advantage of the complete set of features! Clipboard, Search History, and several other advanced features are temporarily unavailable.

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Abstract Background: Although peripheral nervous system involvement is common in eosinophilic granulomatosis with polyangiitis EGPA , central nervous system CNS manifestations are poorly described.

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Rituximab has been proven superior over conventional immunosuppressant therapy for remission maintenance. Its efficacy on the vasculitis is less clear. This article reviews the current role for targeted immunotherapy in AAV, its validated indications, and avenues for further development. Rituximab in these indications is not less effective than cyclophosphamide and is particularly useful in patients with refractory or relapsing disease, women of childbearing potential, and patients previously treated with cyclophosphamide. Rituximab is more effective than cyclophosphamide for treating relapses. For remission maintenance therapy, which is indispensable, rituximab has been proven superior over conventional immunosuppressive treatment.

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Pituitary dysfunction is a rare manifestation of granulomatosis with polyangiitis GPA Wegener. The main aim of this multicenter retrospective study was to describe the characteristics and outcomes of pituitary manifestations in patients with GPA included in the French Vasculitis Study Group database. Among the GPA patients included in the database, 9 1. The median age at diagnosis of GPA and pituitary involvement was 46 and Pituitary involvement was present at onset of GPA in 1 case and occurred later in 8 patients after a median follow up of Magnetic resonance imaging was abnormal in 7 patients. The most common lesions were an enlargement of the pituitary gland, thickening of the pituitary stalk, and loss of posterior hypersignal on T1-weighed images.

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