Dilated cardiomyopathy in a patient with antibody-negative Goodpasture's syndrome and pulmonary relapse

Saudi J Kidney Dis Transpl. 2010 Mar;21(2):332-6.

Abstract

The Goodpasture's syndrome, also known as anti-GBM disease, is an uncommon disease, responsible for 20% of all cases of rapidly progressive glomerulonephritis. Anti-GBM antibodies are directed against non-collagenous domain (NC-1) of the alpha-3 chain of type IV collagen. When conventional ELISA assays are used, these antibodies can be detected in almost all the patients. Nevertheless, some reports have described antibody-negative relapsing disease. Some aggravating factors, namely, smoking, pulmonary infection and hypervolemia, may expose embedded antigenic target and may be responsible for the relapse. In addition, these antibody-negative relapses also respond to standard treatment, which comprises of plasma exchange, pulse steroids and cyclophosphamide. Herein, we report a patient who presented at the Selcuk University Meram School of Medicine, Meram, Konya, Turkey, with the pulmonary-renal syndrome. He was also found to have idiopathic dilated cardiomyopathy (DCM). To our knowledge, this is the first report describing co-existence of DCM and anti-GBM disease. There is growing evidence showing strong relation of both DCM and anti-GBM disease with HLA. Although not proven, this might have occurred in our patient. In our opinion, volume overload was facilitated by anuria and DCM and led to an antibody-negative pulmonary relapse. The relapse was treated just as the first episode and the patient improved satisfactorily.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Anti-Glomerular Basement Membrane Disease / complications*
  • Anti-Glomerular Basement Membrane Disease / diagnostic imaging
  • Anti-Glomerular Basement Membrane Disease / immunology
  • Anti-Glomerular Basement Membrane Disease / therapy
  • Autoantibodies / blood*
  • Cardiomyopathy, Dilated / complications*
  • Cardiomyopathy, Dilated / diagnosis
  • Glomerular Basement Membrane / immunology*
  • Humans
  • Immunosuppressive Agents / administration & dosage
  • Lung Diseases / diagnostic imaging
  • Lung Diseases / etiology*
  • Lung Diseases / immunology
  • Lung Diseases / therapy
  • Male
  • Plasma Exchange
  • Pulse Therapy, Drug
  • Recurrence
  • Steroids / administration & dosage
  • Tomography, X-Ray Computed
  • Treatment Outcome

Substances

  • Autoantibodies
  • Immunosuppressive Agents
  • Steroids
  • antiglomerular basement membrane antibody