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An unusual form of kidney injury without glomerulonephritis in microscopic polyangiitis: a case report

Cihan Uysal1, Tugba Yilmaz2, Hafsa Kocyigit3

  • 1Department of Nephrology, Erciyes University Medical School, Dede Efendi Sreet, Köşk District, 38030, Melikgazi, Kayseri, Turkey. drcihanuysal@hotmail.com.

BMC Nephrology
|April 2, 2023
PubMed
Abstract

Insights

Microscopic polyangiitis (MPA), a form of antineutrophil cytoplasmic autoantibody associated vasculitis (AAV), can present atypically with isolated kidney tubulointerstitial disease. This case highlights the importance of considering MPA even without typical glomerular findings for appropriate treatment.

Area of Science:

  • Nephrology
  • Rheumatology
  • Pathology

Background:

  • Microscopic polyangiitis (MPA) is a small-vessel vasculitis and a common cause of pulmonary-renal syndrome.
  • Antineutrophil cytoplasmic autoantibody associated vasculitis (AAV) typically presents with pauci-immune necrotizing and crescentic glomerulonephritis.
  • Tubulointerstitial lesions can occur in AAV but isolated involvement without glomerular damage is uncommon.

Observation:

  • A 70-year-old male presented with acute kidney injury requiring dialysis.
  • Kidney biopsy revealed acute tubulointerstitial nephritis without glomerular injury.
  • Computerized tomography showed interstitial pulmonary fibrosis, and myeloperoxidase-antineutrophil cytoplasmic autoantibody was positive.

Findings:

  • The patient was diagnosed with MPA despite the atypical isolated tubulointerstitial nephritis.
  • Treatment followed the regimen for crescentic glomerulonephritis, including pulse steroids, cyclophosphamide, and plasmapheresis.
  • The patient unfortunately succumbed to a severe SARS-CoV-2 infection during induction therapy.

Implications:

  • Isolated tubulointerstitial inflammation in the kidney can be an atypical presentation of MPA.
  • Recognizing this variant is crucial for guiding appropriate treatment in AAV.
  • This case underscores the diagnostic challenges and potential severity of MPA presentations.
Keywords:
Acute kidney injuryCase reportDialysisMicroscopic polyangiitisTubulointerstitial nephritis

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