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Pauci-Immune Crescentic and Sclerosing Glomerulonephritis with Concurrent Organizing Pneumonia in a 10-Year-Old Boy with Granulomatosis with Polyangiitis: A Case Report
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Borhan Moradveisi    , Rama Naghshizadian1 |
| 1- , R.naghshizadian@gmail.com |
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Abstract: (11 Views) |
Background and Aim: Granulomatosis with polyangiitis (GPA) is a rare ANCA-associated vasculitis in children, typically involving the upper/lower respiratory tract and kidneys. Renal involvement often manifests as pauci-immune crescentic glomerulonephritis, which may progress to chronic sclerosing lesions despite treatment. This report presents an unusual pediatric GPA case with simultaneous fibrocellular crescents, focal segmental glomerulosclerosis-like lesions, and organizing pneumonia, emphasizing the importance of histopathological subclassification.
Case Presentation: A 10-year-old boy, a known case of GPA, presented with edema and proteinuria. Laboratory tests revealed normal serum creatinine (0.6 mg/dL), elevated BUN (20 mg/dL), and leukocytosis with left shift. Urinalysis showed proteinuria. Chest CT without contrast suggested organizing pneumonia. Kidney needle biopsy contained 10 glomeruli, two with global sclerosis, two with segmental sclerosis, and two with fibrocellular crescents. Mild mesangial expansion and 10–15% interstitial fibrosis were noted. Immunofluorescence was completely negative (pauci-immune pattern). Electron microscopy revealed 40–50% foot process effacement without immune deposits. A prior left elbow biopsy had shown severe leukocytoclastic vasculitis. Brain MRI revealed bilateral subcortical high T2/FLAIR parietal signals consistent with post-ictal changes; MRA was normal. ANCA serology was drawn but the titer was not retrievable. The patient was treated with pulse methylprednisolone, followed by oral prednisolone and rituximab according to KDIGO 2021 guidelines for ANCA-associated vasculitis with renal involvement.
Conclusion: This case illustrates that even in pediatric GPA with apparently normal renal function, histopathological evaluation can reveal advanced chronic changes such as fibrocellular crescents and segmental sclerosis. Such findings carry prognostic significance and may justify aggressive immunosuppression. The coexistence of organizing pneumonia further underscores the multisystem nature of the disease. Early biopsy and tailored therapy are critical to halt progression toward end-stage renal disease.
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| Keywords: Granulomatosis with Polyangiitis, Glomerulonephritis, Pauci-Immune, Crescentic Glomerulonephritis, Organizing Pneumonia, Child, Biopsy |
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Type of Study: case report |
Subject:
Medicine - Pediatric
Received: 2026/05/4 | Accepted: 2026/05/25
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