Anti-hLAMP2-antibodies and dual positivity for anti-GBM and MPO-ANCA in a patient with relapsing pulmonary-renal syndrome

BMC Nephrol. 2011 Jun 8;12:26. doi: 10.1186/1471-2369-12-26.

Abstract

Background: Pulmonary-renal syndrome associated with anti-glomerular basement membrane (GBM) antibodies, also known as Goodpasture's syndrome, is a rare but acute and life-threatening condition. One third of patients presenting as anti-GBM antibody positive pulmonary-renal syndrome or rapidly progressive glomerulonephritis are also tested positive for anti-neutrophil cytoplasmic antibodies (ANCA). Whilst anti-GBM disease is considered a non-relapsing condition, the long-term course of double-positive patients is less predictable.

Case presentation: We report a patient with such dual positivity, who presented with pulmonary hemorrhage, crescentic glomerulonephritis and membranous nephropathy. Plasmapheresis in combination with immunosuppressive therapy led to a rapid remission but the disease relapsed after two years. The serum of the patient was tested positive for antibodies to human lysosomal membrane protein 2 (hLAMP2), a novel autoantigen in patients with active small-vessel vasculitis (SVV). The anti-hLAMP2 antibody levels correlated positively with clinical disease activity in this patient.

Conclusion: We hypothesize that this antibody may indicate a clinical course similar to ANCA-associated vasculitis in double-positive patients. However, this needs to be confirmed on comprehensive patient cohorts.

Publication types

  • Case Reports

MeSH terms

  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / immunology
  • Antibodies, Anti-Idiotypic / blood*
  • Antibodies, Antineutrophil Cytoplasmic / blood*
  • Autoantibodies / blood*
  • Female
  • Glomerulonephritis / blood
  • Glomerulonephritis / immunology*
  • Glomerulonephritis / therapy
  • Hemorrhage / blood
  • Hemorrhage / immunology*
  • Hemorrhage / therapy
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Lung Diseases / blood
  • Lung Diseases / immunology*
  • Lung Diseases / therapy
  • Lysosomal-Associated Membrane Protein 2
  • Lysosome-Associated Membrane Glycoproteins / immunology*
  • Middle Aged
  • Plasmapheresis
  • Recurrence
  • Treatment Outcome

Substances

  • Antibodies, Anti-Idiotypic
  • Antibodies, Antineutrophil Cytoplasmic
  • Autoantibodies
  • Immunosuppressive Agents
  • LAMP2 protein, human
  • Lysosomal-Associated Membrane Protein 2
  • Lysosome-Associated Membrane Glycoproteins
  • antiglomerular basement membrane antibody

Supplementary concepts

  • Rapidly progressive glomerulonephritis with pulmonary hemorrhage