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Abstract: SA-PO235

A Case of Possible Malignancy-Related Polyangiitis Overlap Syndrome with Henoch-Schönlein Purpura and ANCA-Negative Pauci-Immune Glomerulonephritis as a Manifestation of Neuroendocrine Cancer

Session Information

Category: Onconephrology

  • 1700 Onconephrology


  • Ali, Muhammad, Nuvance Health, Poughkeepsie, New York, United States
  • Ghalib, Natasha, Montefiore Medical Center Jack D Weiler Hospital, New York, New York, United States
  • Noor, Fadila, Nuvance Health, Poughkeepsie, New York, United States
  • Collins, Susan E., Nuvance Health, Poughkeepsie, New York, United States

Henoch Schönlein purpura (HSP) is an immunoglobulin A (IgA) mediated small vessel vasculitis involving the skin, gastrointestinal tract, kidneys, and joints. It is often precipitated by infections but has also been reported with malignancies. Pauci immune crescentic glomerulonephritis (PICGN) is a cause of rapidly progressive glomerulonephritis (RPGN) and is usually ANCA positive with a paucity of immune complexes on renal biopsy. It is associated with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), or renal limited vasculitis. Polyangiitis overlap syndrome with features of more than one vasculitis has also been reported. We report a unique case of HSP and ANCA-negative PICGN in association with metastatic neuroendocrine cancer.

Case Description

A 54-year-old male with no medical history presented with fatigue, colicky abdominal pain, and dark urine for 2 weeks. He denied recent respiratory or gastrointestinal infection. He had new-onset hypertension (BP 163/96 mmHg), a purpuric rash on the buttocks and lower extremities, and acute kidney injury (AKI). Urinalysis showed hematuria, pyuria, and proteinuria. Skin biopsy showed IgA vasculitis consistent with HSP. Prednisone was started but he required hospitalization due to worsening AKI (BUN 36 mg/dL & creatinine 3.11 mg/dL). Workup showed anemia (Hb 12.8 g/dL), normal platelet count and coagulation profile, proteinuria of 1g/day, positive ANA (titer 1:80), negative anti-MPO & PR3 ANCA, anti-GBM, and anti smith antibodies. CT abdomen showed multiple liver masses and biopsy showed metastatic neuroendocrine cancer. Renal biopsy showed acute pauci immune necrotizing crescentic glomerulonephritis. Chemotherapy (carboplatin & etoposide) was initiated with steroids leading to the resolution of AKI.


We report a unique case of HSP and ANCA-negative PICGN in association with metastatic neuroendocrine cancer. The difference in skin and renal biopsies indicates possible malignancy-related polyangiitis overlap syndrome. HSP and PICGN can be manifestations of occult malignancy, especially in adult males. Diagnosis of malignancy is crucial as remission of vasculitis may require both treatment of cancer and immunosuppressants.