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Myeloperoxidase-antineutrophil cytoplasmic antibody-negative microscopic polyangiitis with pulmonary haemorrhage and

Yuichiro Endo1, Haruka Minato, Reiko Taki

  • 1Department of Dermatology, Graduate School of Medicine, Kyoto University, Kyoto, Japan.

Case Reports in Dermatology
|September 21, 2011
PubMed
Abstract

Insights

This case report details a patient with myeloperoxidase-antineutrophil cytoplasmic antibody (ANCA)-negative microscopic polyangiitis (MPA) and IgA nephropathy, presenting with severe pulmonary hemorrhage. The findings highlight the importance of monitoring immunoglobulin deposition in MPA for prognosis.

Area of Science:

  • Nephrology
  • Rheumatology
  • Pathology

Background:

  • Microscopic polyangiitis (MPA) is a systemic vasculitis typically associated with myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA).
  • IgA nephropathy is a common glomerulonephritis characterized by IgA deposition in the glomeruli.

Observation:

  • A 59-year-old man presented with ANCA-negative systemic vasculitis, including purpura, nephritis, and pulmonary hemorrhage.
  • Skin biopsy showed leucocytoclastic vasculitis without IgA deposition.
  • Kidney biopsy revealed mesangial nephritis with IgA deposition.

Findings:

  • The patient was diagnosed with MPA co-occurring with IgA nephropathy.
  • This presentation is unusual as IgA nephropathy is less commonly associated with MPA.
  • The co-existence of MPA and IgA nephropathy in this case is notable.

Implications:

  • MPA usually presents with glomerulonephritis and can involve lung hemorrhage.
  • Immunoglobulin deposition in the kidney in MPA may worsen renal dysfunction and prognosis.
  • Close monitoring is crucial for patients with MPA and co-existing immunoglobulin deposition disorders like IgA nephropathy.
Keywords:
IgA nephropathyMicroscopic polyangiitisPulmonary haemorrhage

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