A case of hypertrophic cranial pachymeningitis presenting with scleritis in a patient with undifferentiated connective tissue disease.
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, Behçet's disease, Sjögren syndrome, and temporal arteritis. Here, we report a case of HCP initially presented with scleritis and headache in a patient with undifferentiated 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.
Kim
Ji-Hyeon
JH
Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Holy Family Hospital, Bucheon, Korea.
Joo
Young-Bin
YB
Kim
Jeana
J
Min
Jun-Ki
JK
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D003937:Diagnosis, Differential; D004388:Dura Mater / Q000473:pathology; D006801:Humans; D006984:Hypertrophy; D008279:Magnetic Resonance Imaging; D008297:Male; D008581:Meningitis / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D008947:Mixed Connective Tissue Disease / Q000150:complications / Q000175:diagnosis*; D012261:Ribonucleoproteins / Q000378:metabolism; D015423:Scleritis / Q000150:complications / Q000175:diagnosis*; D014057:Tomography, X-Ray Computed
D000305:Adrenal Cortex Hormones; D012261:Ribonucleoproteins