Kidney Week

Abstract: PUB154

Severe Myeloperoxidase-ANCA-Associated Crescentic Glomerulonephritis in a Young Woman: A Case of Rapid Progression to ESKD

Session Information

• Publication Only

Category: Glomerular Diseases

• 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

• Rongkiettechakorn, Nuttawut, Golden Jubilee Medical Center, Faculty of Medicine Siriraj Hospital, Mahidol University, Phutthamonthon, Nakhon Pathom, Thailand

Nuttawut Rongkiettechakorn, MD

• Jampong, Malinporn, Golden Jubilee Medical Center, Faculty of Medicine Siriraj Hospital, Mahidol University, Phutthamonthon, Nakhon Pathom, Thailand

Malinporn Jampong, MD

• Russameekulthana, Dechathorn, Golden Jubilee Medical Center, Faculty of Medicine Siriraj Hospital, Mahidol University, Phutthamonthon, Nakhon Pathom, Thailand

Dechathorn Russameekulthana, MD

• Cheunsuchon, Boonyarit, Department of Pathology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand

Boonyarit Cheunsuchon, MD

• Rattanasompattikul, Manoch, Golden Jubilee Medical Center, Faculty of Medicine Siriraj Hospital, Mahidol University, Phutthamonthon, Nakhon Pathom, Thailand

Manoch Rattanasompattikul, MD, FASN

Introduction

ANCA-associated vasculitis (AAV) typically affects middle-aged and older populations. When presenting as rapidly progressive glomerulonephritis (RPGN) in young adults, the diagnosis is often delayed due to low clinical suspicion. We report a rare case of severe Myeloperoxidase (MPO)-ANCA-associated crescentic glomerulonephritis in a 22-year-old woman that resulted in irreversible kidney failure.

Case Description

A 22-year-old Thai woman presented with a 2-week history of gross hematuria, fever, and night sweats, followed by acute dyspnea. On admission, she was hypertensive (150/100 mm.Hg) and tachypneic. Laboratory studies revealed severe acute kidney injury (serum creatinine 16.83 mg/dL), anemia (Hb 7.2 g/dL), and nephritic sediment (proteinuria 4+, dysmorphic RBCs).
Serologic evaluation showed a strongly positive MPO-ANCA (>200 RU/mL) with negative PR3-ANCA, anti-GBM, and viral markers. Kidney biopsy confirmed pauci-immune crescentic glomerulonephritis. Notably, 22 of 28 glomeruli showed crescents (16 fibrous, 4 fibrocellular, 2 cellular), and 14 showed global sclerosis. There was significant chronicity, with 60% interstitial fibrosis and tubular atrophy (IFTA).
The patient received pulse methylprednisolone, plasmapheresis, and cyclophosphamide. Her course was complicated by Staphylococcus aureus pneumonia. Despite aggressive immunosuppression, renal function did not recover, and she remains dependent on maintenance hemodialysis.

Discussion

This case underscores that AAV can present with extreme severity in young patients. The high proportion of fibrous crescents and extensive IFTA at the time of presentation were poor prognostic indicators, suggesting a subacute "smoldering" course prior to the acute presentation.
For clinicians, this case serves as a critical reminder to include AAV in the differential diagnosis of young adults presenting with active urinary sediment and rapidly rising creatinine. Early diagnostic biopsy is paramount; however, as seen here, extensive chronic histologic changes at diagnosis may portend irreversible ESKD regardless of intensive therapy.