From fibrosis to diagnosis: a paediatric case of microscopic polyangiitis and review of the literature

Rheumatol Int. 2018 Apr;38(4):683-687. doi: 10.1007/s00296-017-3923-y. Epub 2018 Jan 2.

Abstract

We herein describe a case of a 6-year-old girl diagnosed with microscopic polyangiitis (MPA) after a long diagnostic process demanding the cooperation of paediatric respiratory medicine specialists, rheumatologists, nephrologists and radiologist. First symptoms of MPA were observed after Mycoplasma pneumoniae infection and included persistent crepitations at the basis of both lungs and mild haematuria. CT imaging showed features of lung fibrosis, renal biopsy was indicative of mesangial proliferative glomerulonephritis, in the skin biopsy features of microscopic polyangiitis were described. In the laboratory tests pANCA antibodies specific for myeloperoxidase (MPO) were present in high titer. MPA with initial features of lung fibrosis is an unusual presentation of this rare disease, usually observed in adult population. This unique case illustrates the diagnostic and therapeutic challenges in paediatric vasculitis patients with an unusual initial presentation in the form of interstitial lung disease. In the manuscript we also discuss the possible aetiology of lung fibrosis in microscopic polyangiitis MPA and provide the review of the current literature on the topic of childhood-onset MPA.

Keywords: Childhood vasculitis; Microscopic polyangiitis; p-ANCA vasculitis.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic / blood
  • Biopsy
  • Child
  • Diagnosis, Differential
  • Drug Therapy, Combination
  • Female
  • Glucocorticoids / therapeutic use
  • Humans
  • Idiopathic Pulmonary Fibrosis / blood
  • Idiopathic Pulmonary Fibrosis / diagnosis*
  • Idiopathic Pulmonary Fibrosis / drug therapy
  • Idiopathic Pulmonary Fibrosis / pathology
  • Immunosuppressive Agents / therapeutic use
  • Interdisciplinary Communication
  • Microscopic Polyangiitis / blood
  • Microscopic Polyangiitis / diagnosis*
  • Microscopic Polyangiitis / drug therapy
  • Microscopic Polyangiitis / pathology
  • Patient Care Team
  • Peroxidase / immunology
  • Predictive Value of Tests
  • Tomography, X-Ray Computed
  • Treatment Outcome

Substances

  • Antibodies, Antineutrophil Cytoplasmic
  • Glucocorticoids
  • Immunosuppressive Agents
  • Peroxidase