A Case of Efferent and Afferent Disease

IgG4-related disease (RD) is a diagnosis of exclusion that manifests as inflammation and fibrosis in any organ, including the meninges and orbits. IgG4-RD has manifested as cranial nerve palsies and vision loss in these settings[1]. These symptoms are reversible with treatment.

Poster 24 A Case of Efferent and Afferent Disease Andrew Sumnicht1 1 Loma Linda University, Loma Linda, California, USA Introduction: IgG4-related disease (RD) is a diagnosis of exclusion that manifests as inflammation and fibrosis in any organ, including the meninges and orbits. IgG4-RD has manifested as cranial nerve palsies and vision loss in these settings[1]. These symptoms are reversible with treatment. Description of Case(s): A 49-year-old female with chronic pachymeningitis presented with four weeks of progressive vision loss in the left eye (OS) from 20/20 to 20/400. In the past year she had two cranial nerve palsies in the right eye, each resolving spontaneously. She demonstrated borderline thickening of the retinal nerve fiber layers, a relative afferent pupillary defect, and a severely constricted visual field OS. Vision worsened to no light perception (NLP) OS over three days. Imaging demonstrated a new mass compressing the left optic nerve and diffuse pachymeningeal enhancement. Lumbar puncture showed elevated protein, IgG, IgG index (3.03), and IgG synthesis. Extensive workup revealed normal serum IgG and IgG4 fraction and ruled out known causes of meningitis. Dural biopsy revealed mild fibrosis without plasma cells or IgG4 immunoreactive cells. Her vision improved to 1/200 OS after two weeks of oral prednisone, which the patient self-discontinued due to side effect intolerance. She declined rituximab. Her vision spontaneously improved to 20/60 OS after two more months. Conclusions, including unique features of the case(s): The presumptive diagnosis was IgG4-hypertrophic pachymeningitis (HP). There are at least four other reported cases of biopsyproven IgG4-HP with ophthalmic symptoms. Elevated IgG and IgG4 indices are reported to be reliable markers of IgG4-HP compared with infectious, rheumatologic, and other autoimmune causes of HP[2]. The diagnostic value of serum elevation of IgG and IgG4 has been questioned since IgG4 is only elevated in as many as 51% of cases and IgG is elevated in even fewer[1]. In this case the patient regained significant vision in a previously NLP eye and had a perplexing clinical course and workup. References: Wallace ZS et al, IgG4-related disease: baseline clinical and laboratory features in 125 patients with biopsy-proven disease, Arthritis Rheumatol, 67(9), 2466-2475, 2015 Della-Torre E et al, Diagnostic value of IgG4 indices in IgG4-related hypertrophic pachymeningitis, J Neuroimmunol, 266(1-2): 82-86, 2014 Keywords: Neuro-ophth & systyemic disease ( eg. MS, MG, thyroid), Skull Base, Neuroimaging, Tumors, Miscellaneous Financial Disclosures: The authors had no disclosures. Grant Support: None. 2018 Annual Meeting | 72