Article Text

Download PDFPDF

Granulomatosis with polyangiitis in a patient with biopsy-proven IgG4-related pulmonary disease and coincident small cell lung cancer
  1. Khurram Abbass1 and
  2. Hollis Krug2
  1. 1 Department of Medicine, Regional Medical Center, San Jose, California, USA
  2. 2 Rheumatology (111R), Minneapolis VA HCS, Minneapolis, Minnesota, USA
  1. Correspondence to Dr Hollis Krug, hollis.krug{at}


Granulomatosis with polyangiitis (GPA) was diagnosed in a patient with a 16-month history of IgG4-related lung disease that spontaneously became asymptomatic. Cytoplasmic antineutrophil cytoplasmic antibody (ANCA) was positive at the time of diagnosis of IgG4-related disease (IgG4-RD), but there was no vasculitis or kidney disease. Sixteen months later he developed rapidly progressive glomerulonephritis that responded to cyclophosphamide treatment. While undergoing treatment for GPA, he was found to have a lung mass identified as small cell lung cancer. This mass was present at the time of the IgG4-RD diagnosis. GPA can be confused with IgG4-RD histologically and they rarely coexist. ANCA antibodies are primarily IgG4 subclass. IgG4-RD has been associated with cancer and may improve prognosis. We speculate that this patient may have had small cell lung cancer that incited an IgG4 predominant immune response with coexistent ANCA antibodies that eventually resulted in GPA. Immunosuppressive treatment of GPA likely accelerated the progression of the lung cancer.

  • lung cancer (oncology)
  • malignant disease and immunosuppression
  • pathology
  • vasculitis
  • immunology

This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:

View Full Text

Statistics from


  • Contributors HK recognised the importance of this case, cared for the patient, consulted with consultants during and after the patient’s illness, acquired much of the necessary information and finalised the publication. KA collected the patients clinical information and drafted the original article.

  • Funding This work was supported by the United States Department of Veterans Affairs.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Patient consent for publication Next of kin consent obtained.

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.