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Washington University Experience | MISCELLANEOUS | Idiopathic Hypertrophic Pachymeningitis (IgG4 Disease) | 1A0 Case 1 History
Case 1 History ---- This is a 54 year-old man with past medical history significant for hypertension who presented with a history of several months of sinusitis requiring several courses of antibiotics and tympanostomy. Over the last 2–3 months he developed bilateral eye pain with progressive vision loss culminating in blindness and unintentional weight loss of nearly 100 pounds. A CT scan of the brain showed a right frontoparietal subdural fluid collection, consistent with a hematoma, measuring up to 1 cm in thickness with minimal midline shift. A bilateral temporal artery biopsy and a turbinate biopsy showed no evidence of vasculitis. He underwent drainage of the hematoma without significant improvement of his symptoms. MRI T1 image revealed bilateral subdural collections, with associated diffuse meningeal thickening. There was a 1.5 cm lesion with restricted diffusion in the right parietal lobe and associated contrast enhancement, suspicious for a lymphoma. A subsequent lumbar puncture showed a mildly elevated protein level and a negative cytology. A CT scan of the chest, abdomen and pelvis demonstrated no evidence of lymphadenopathy. A bone marrow biopsy showed normocellular marrow with multilineage hematopoiesis and no lymphoma. Laboratory findings were significant for anemia and mild thrombocytosis. In addition, normal IgG levels with elevated IgG3 (108 mg/dl; nl range = 18.4 – 106 mg/dl) and IgG4 (148 mg/dl; nl range = 2.4 – 121 mg/dl) subclasses were seen. A biopsy of the putative enhancing lesion and the meninges was taken under MRI-guidance.
