IgG4-related neuromyopathy associated with recurrent pleural effusion

Waqar Waheed, Joshua Nickerson, Abiy B. Ambaye, Mohamed Ali Babi, Rup Tandan

Research output: Contribution to journalArticlepeer-review

6 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)210-219
Number of pages10
JournalJournal of clinical neuromuscular disease
Issue number4
StatePublished - Jun 6 2015
Externally publishedYes


  • IgG4-related disorder
  • neuromyopathy
  • pleural effusion
  • pleural histopathology
  • serial IgG4 levels

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology


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