An overlap of granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis

Saudi J Kidney Dis Transpl. 2017 May-Jun;28(3):639-644. doi: 10.4103/1319-2442.206450.

Abstract

We present a case report of overlap of granulomatosis with polyangiitis (GPA; formerly known as Wegener's granulomatosis) and eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome). We report a 45-year-old female who presented with rapidly progressive renal failure associated with fever, polyarthralgia, and respiratory symptoms with cytoplasmic antineutrophilic cytoplasmic antibody (ANCA) and proteinase (PR-3) antigen positivity. Computerized tomography scan of the chest showed diffuse alveolar hemorrhage with renal biopsy revealing pauci-immune necrotizing crescentic glomerulonephritis with intense eosinophilic infiltration suggestive of eosinophilic GPA (EGPA). Our patient had ANCA-associated vasculitis (AAV) with features suggestive of both GPA and EGPA. She was treated with methylprednisolone and cyclophosphamide and attained remission after 2 weeks of therapy. This is a rare report of a patient with AAV having features of both EGPA and GPA.

Publication types

  • Case Reports

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic / blood
  • Biomarkers / blood
  • Biopsy
  • Churg-Strauss Syndrome / complications*
  • Churg-Strauss Syndrome / diagnosis
  • Churg-Strauss Syndrome / drug therapy
  • Churg-Strauss Syndrome / immunology
  • Cyclophosphamide / therapeutic use
  • Disease Progression
  • Drug Therapy, Combination
  • Female
  • Glucocorticoids / therapeutic use
  • Granulomatosis with Polyangiitis / complications*
  • Granulomatosis with Polyangiitis / diagnosis
  • Granulomatosis with Polyangiitis / drug therapy
  • Granulomatosis with Polyangiitis / immunology
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Methylprednisolone / therapeutic use
  • Middle Aged
  • Myeloblastin / immunology
  • Renal Insufficiency / etiology
  • Tomography, X-Ray Computed
  • Treatment Outcome

Substances

  • Antibodies, Antineutrophil Cytoplasmic
  • Biomarkers
  • Glucocorticoids
  • Immunosuppressive Agents
  • Cyclophosphamide
  • Myeloblastin
  • Methylprednisolone