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Abstract: PO1759

IgG4-Related Kidney Disease Associated with an Unusual Vasculitic Peripheral Neuropathy

Session Information

Category: Trainee Case Report

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Baig, Muhammad Tariq, The Ohio State University Wexner Medical Center, Columbus, Ohio, United States
  • Satoskar, Anjali A., The Ohio State University Wexner Medical Center, Columbus, Ohio, United States
  • Jiang, Benjamin, The Ohio State University Wexner Medical Center, Columbus, Ohio, United States
  • Freimer, Miriam L., The Ohio State University Wexner Medical Center, Columbus, Ohio, United States
  • Ayoub, Isabelle, The Ohio State University Wexner Medical Center, Columbus, Ohio, United States
Introduction

IgG4-related disease is a systemic autoimmune fibro-inflammatory disorder showing lymphoplasmacytic infiltrates with predominance of IgG4+ plasma cells and variable amounts of storiform fibrosis in the affected tissues. When kidneys are the only organs involved it is called IgG4-related kidney disease (IgG4-RKD). Neurologic involvement is less common and known to manifest as hypophysitis and pachymeningitis. Peripheral neuropathy is rare. Our case illustrates an unusual presentation of IgG4-RKD with vasculitic peripheral neuropathy, which has never been reported before

Case Description

A 55-year-old Southeast Asian woman with allergic rhinitis presented to her PCP with burning and tingling from the knees down and difficulty with gait for about 6 months. Neurological examination was notable for weakness of ankle dorsiflexion and plantarflexion and loss of pinprick and vibration sense distal to ankles. This was attributed to iron deficiency anemia and a compressed nerve. However, her symptoms worsened on iron supplements and gabapentin and were accompanied by weight loss. CT scan of abdomen showed heterogeneous masses of the kidneys with few enlarged retroperitoneal lymph nodes. Kidney biopsy was performed and showed storiform fibrosis and plasma cell rich interstitial inflammation (>30 IgG4+ plasma cells/HPF) suggesting IgG4-RKD. Further work up was significant for serum IgE 1309 IU/ml (1.5-165), IgG4 177 mg/dl (2.4-121), ANA >1:1280, positive MPO-ANCA, RF 38 IU/ml (<14), ESR 77 mm/hr. CRP, complement levels and kidney function were normal. Prior to initiating therapy for IgG4 RKD she was referred for sural nerve biopsy for concern of associated vasculitis. Nerve biopsy showed severe myelinated and unmyelinated fiber loss in all fascicles, a recanalized epineural blood vessel, and dense perineural mononuclear cell infiltrates consistent with vasculitic neuropathy. Additionally, immunostaining showed IgG4 plasma cells up to 10/HPF. Her symptoms resolved with steroids, IVIG and rituximab. Serum IgG4 level improved to 26 mg/dl.

Discussion

IgG4-RD can have varied systemic manifestations. Although neurologic disease is less commonly seen in IgG4-RD, we report for the first time an associated vasculitic neuropathy that should be considered and worked up in symptomatic patients