Vasculitic Neuropathy in a Patient with IgG4-related Disease (981)

Objective: We describe a patient with biopsy-proven IgG4-related disease who presented with painful peripheral neuropathy and was found to have vasculitic neuropathy on sural nerve biopsy. Background: IgG4-related disease (IgG4-RD) is an immune-mediated systemic inflammatory condition characterized by tissue infiltration by IgG4-positive plasma cells and elevated serum IgG4 concentrations. Renal, pancreatic, hepatobiliary, pulmonary, aortic, and orbital involvement are often seen. Neurologic involvement is less common and typically involves hypophysitis or pachymeningitis. Peripheral neuropathy has been reported in IgG4-RD though is considered a rarer manifestation. While elevated IgG4 levels have been described in inflammatory neuropathies, vasculitic peripheral neuropathy has not yet been described in IgG4-RD. Design/Methods: N/A Results: A 55-year-old woman presented with a 6 month history of burning and tingling from the knees down and difficulty with gait. She had previously been evaluated for allergic rhinitis, weight loss, and anemia. CT of the abdomen demonstrated heterogeneous enhancement of the kidneys with loss of normal corticomedullary differentiation, and kidney biopsy was consistent with IgG4-RD. Neurological examination was notable for weakness of ankle dorsiflexion and plantarflexion and loss of pinprick and vibration sense distal to the ankles bilaterally. Electrodiagnostic testing showed a significant length-dependent axonal sensorimotor polyneuropathy. The patient underwent sural nerve biopsy, which showed loss of myelinated fibers in several nerve fascicles, a recanalized epineurial blood vessel, and dense perineurial mononuclear cell infiltrates, compatible with vasculitic neuropathy. Conclusions: IgG4-RD is an inflammatory condition that involves multiple organ systems. Neurologic disease is less commonly seen in IgG4-RD and typically involves the central nervous system, with peripheral neuropathy being a rarer manifestation. We report a case of vasculitic peripheral neuropathy in a patient with IgG4-RD, which to our knowledge has not been previously described. IgG4-RD should be considered in the differential diagnosis in patients with peripheral neuropathy and evidence of systemic inflammatory disease. Disclosure: Dr. Jiang has nothing to disclose. Dr. Ayoub has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with ChemoCentryx. Dr. Sahenk has nothing to disclose. Dr. Freimer has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Alexion and Argenx. Dr. Freimer has received research support from Alexion, UCB, Amicus, Alnylum, Ra, Catalyst, and Momenta.