J Korean Med Sci.  2010 Jun;25(6):966-969. 10.3346/jkms.2010.25.6.966.

A Case of Hypertrophic Cranial Pachymeningitis Presenting with Scleritis in a Patient with Undifferentiated Connective Tissue Disease

• Kim JH
• Joo YB
• Kim J
• Min JK

• KMID: 2150882
• DOI: http://doi.org/10.3346/jkms.2010.25.6.966

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.

Keyword

Hypertrophic Pachymeningitis; Undifferenciated Connective Tissue Disease; Scleritis

Figure

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  Fig. 1 Enhanced computed tomography of the chest. (A) In April 2008, irregular soft tissue density measuring about 3.3×1.6 cm was seen in the left upper anterior pericardial region (arrow). (B) In October 2008, 5 months after the initial glucocorticoid therapy, follow up image demonstrated more regression state of the left upper anterior soft tissue density, measuring about 1.7×0.7 cm (arrow).

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  Fig. 2 Magnetic resonance image of the brain. (A) On the 3rd hospital day, axial T1-weighted contrast-enhanced magnetic resonance image shows diffuse thick dural enhancement along the left tentorium and left cerebellar surface. (B). In 4 weeks after the initial glucocorticoid treatment, follow-up magnetic resonance image demonstrated nearly complete regression of dural enhancement. Only very thin dural enhancement remained.

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  Fig. 3 Photomicrographs of sections of dura mater tissue stained with hematotoxylin and eosin,×400. (A) Thickened dura mater revealed extensive fibrosis. (B) Acute and chronic inflammatory infiltrate is consisting chiefly of plasma cells with additional lymphocytes.

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