title: IgG4-Related Neuromyopathy Associated With Recurrent Pleural Effusion.
abstract: OBJECTIVE
  Immunoglobulin G4-related disease (IgG4-RD) is an increasingly recognized idiopathic systemic disorder associated with elevated serum IgG4 level and tissue infiltration by IgG4-positive plasma cells. Multiple neurological manifestations, including peripheral neuropathy, have been described in IgG4-RD. Our objective is to discuss a case report and review of the literature, which would expand the spectrum of IgG4-RD.
  
  
  METHODS
  We describe the clinical features and biopsy findings in a patient with IgG4-RD who presented with features suggestive of neuromyopathy in the setting of recurrent pleural effusion and weight loss.
  
  
  RESULTS
  Electrodiagnostic findings were suggestive of an irritable myopathy and polyradiculoneuropathy with primary demyelination and secondary axonal degeneration. Pleural biopsy and laboratory studies confirmed the diagnosis. Improvement was sustained with steroid therapy.
  
  
  CONCLUSIONS
  We describe the first case, to our knowledge, of IgG4-related neuromyopathy associated with recurrent pleural effusion. Our case expands the clinical spectrum of IgG4-RD. Neurologists should be aware of this treatable disorder and in the appropriate clinical context consider it in the differential diagnosis of neuromyopathy.
authors.affiliation: Departments of *Neurological Sciences, †Radiology, and ‡Pathology, University of Vermont College of Medicine and Fletcher Allen Health Care, Burlington, VT.
authors.forename: Waqar
authors.initials: W
authors.lastname: Waheed
authors.forename: Joshua
authors.initials: J
authors.lastname: Nickerson
authors.forename: Abiy B
authors.initials: AB
authors.lastname: Ambaye
authors.forename: Mohamed-Ali
authors.initials: MA
authors.lastname: Babi
authors.forename: Rup
authors.initials: R
authors.lastname: Tandan
mesh_terms: D000368:Aged; D001327:Autoimmune Diseases / Q000276:immunology / Q000473:pathology*; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D008297:Male; D009468:Neuromuscular Diseases / Q000276:immunology / Q000473:pathology*; D010996:Pleural Effusion / Q000276:immunology / Q000473:pathology*; D012008:Recurrence
chemical_list: D007074:Immunoglobulin G