A Case of Hypertrophic Cranial Pachymeningitis Presenting with Scleritis in a Patient with Undifferentiated Connective Tissue Disease.
10.3346/jkms.2010.25.6.966
- Author:
Ji Hyeon KIM
1
;
Young Bin JOO
;
Jeana KIM
;
Jun Ki MIN
Author Information
1. Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Holy Family Hospital, Bucheon, Korea.
- Publication Type:Case Reports
- Keywords:
Hypertrophic Pachymeningitis;
Undifferenciated Connective Tissue Disease;
Scleritis
- From:Journal of Korean Medical Science
2010;25(6):966-969
- CountryRepublic of Korea
- Language:English
-
Abstract:
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.
