IgG4-Related Neuropathy

Abstract

The newly recognized entity IgG4related disease (IgG4-RD) is characterized by an elevated IgG4 serum concentration and tissue infiltration by IgG4-positive plasma cells. We describe, for the first time, the clinical features and nerve biopsy findings of a patient with IgG4-RD who presented with neuropathy in the extremities.Observations: A 55-year-old man had histopathologically defined IgG4-RD that manifested as sensorymotor neuropathy. The neuropathic features were multiple mononeuropathies with electrophysiological findings suggestive of axonal neuropathy. Marked thickening with abundant collagen fibers and infiltration of IgG4-positive plasma cells were observed in the epineurium of the biopsied sural nerve. A moderate degree of myelinated fiber loss without evidence of segmental demyelination was present, whereas necrotizing vasculitis was not found. Oral prednisolone therapy ameliorated the neuropathic symptoms.Conclusions and Relevance: This case of IgG4-RD presented as sensory-motor neuropathy with pain and sclerosis of the skin in the extremities. The differential diagnosis of neuropathy should include IgG4-RD.

IgG4-Related Neuromyopathy Associated With Recurrent Pleural Effusion

“…Our case is different from previous reports of IgG4-RD peripheral nervous system involvement, which seem to reveal 2 patterns: the first pattern is of an asymmetrical axonal peripheral neuropathy 13,14 suggestive of mononeuritis multiplex with elevated inflammatory markers. Sural nerve biopsy in a classical case 13 revealed infiltration of IgG4+ plasma cells and fibrosis in the epineurium, whereas in another case 14 showed tumefactive lymphoid infiltrates without IgG4+ plasma cells or fibrosis.…”

“…Putative mechanisms of peripheral neuropathy in IgG4-RD include focal ischemia resulting from occlusion of vessels by infiltrating inflammatory cells or from constriction by marked fibrosis. 13 In our case, the prebiopsy treatment with steroids probably masked the pathologic findings and resulted in significant clinical improvement.…”

“…Neurological manifestations described in IgG4-RD include hypopituitarism with IgG4-related hypophysitis, 5,6 pachymeningitis, 7-9 orbital pseudotumor, 10,11 and peripheral nerve involvement (perineural inflammation). [12][13][14] We report a case with recurrent pleural effusion and weight loss followed by progressive numbness and weakness associated with an IgG4 disorder.…”

Neurological Manifestations of IgG4-Related Disease

“…To the best of our knowledge, there is only a single report of neuropathy ascribed to IgG4-RD [101]. The authors described a 55-year-old man with histopathologically confirmed IgG4-RD manifesting as mononeuritis multiplex, with electrophysiological findings suggestive of axonal neuropathy.…”

IgG4-Related Sialoadenitis with a Skin Lesion and Multiple Mononeuropathies Suggesting Coexistent Cryoglobulinemic Vasculitis

“…However, IgG4-RD rarely involves peripheral nerves. There have been a few reported cases involving the peripheral nervous system, which were characterized by nerve-centered distinct soft tissue masses (4,5).…”