KMID : 0191120100250060966
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Journal of Korean Medical Science 2010 Volume.25 No. 6 p.966 ~ p.969
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A Case of Hypertrophic Cranial Pachymeningitis Presenting with Scleritis in a Patient with Undifferentiated Connective Tissue Disease
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Kim Ji-Hyeon
Ju Young-Bin Kim Jean-A Min Jun-Ki
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Abstract
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Hypertrophic cranial pachymeningitis (HCP) is an uncommon disorder that causes a localized or diffuse thickening of the dura mater and has been reported to be infrequently associated with systemic autoimmune disorders such as Wegener¡¯s granulomatosis, rheumatoid arthritis, sarcoidosis, Behcet¡¯s disease, Sjogren syndrome, and temporal arteritis. Here, we report a case of HCP initially presented with scleritis and headache in a patient with undifferenciated connective tissue disease (UCTD). HCP was initially suspected on brain magnetic resonance imaging and defined pathologically on meningial biopsy. Immunologic studies showed the presence of anti-RNP antibody. After high dose corticosteroid therapy, the patient¡¯s symptoms and radiologic abnormalities of brain were improved. Our case suggested that HCP should be considered in the differential diagnosis of headache in a patient with UCTD presenting with scleritis.
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KEYWORD
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Hypertrophic Pachymeningitis, Undifferenciated Connective Tissue Disease, Scleritis
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