IgG4-related Disease Manifesting as Pachymeningitis and Aortitis

Abstract

To the Editor: A 57-year-old Iraqi woman presented with a 3-year history of right temporal headache, weight loss, and fatigue. She denied fevers, chills, night sweats, visual changes, jaw claudication, joint pain, or focal neurologic symptoms. Her medications included aspirin and captopril. She had received 1 month of oral prednisone (dose unclear) 4 months prior to presentation. Her vital signs were unremarkable, including symmetric blood pressures in all 4 limbs. She had scalp tenderness without temporal artery nodularity and had bruits of the right carotid, right brachial, and bilateral femoral arteries. The examination was otherwise unremarkable. She was anemic (hematocrit 27.2%) with erythrocyte sedimentation rate (ESR) 115 mm/h and C-reactive protein (CRP) 3.3 mg/dl. Cerebrospinal fluid (CSF) was clear and colorless and showed a lymphocytic pleiocytosis (24 white blood cells with 93% lymphocytes) with mildly elevated protein (71 mg/dl) and normal glucose (51 mg/dl). Negative infectious studies of CSF included bacterial, fungal, and mycobacterial cultures, as well as venereal disease research laboratory test, PCR for herpes simplex virus, and cryptococcal antigen. Magnetic resonance imaging of the brain showed diffuse pachymeningeal thickening and enhancement (Figure 1A) without venous sinus thrombosis by computed tomography (CT) venogram. CT of the chest, abdomen, and pelvis revealed thoracic … Address correspondence to Prof. Schwab; E-mail: schwabp{at}ohsu.edu