ASN's Mission

To create a world without kidney diseases, the ASN Alliance for Kidney Health elevates care by educating and informing, driving breakthroughs and innovation, and advocating for policies that create transformative changes in kidney medicine throughout the world.

learn more

Contact ASN

1401 H St, NW, Ste 900, Washington, DC 20005

email@asn-online.org

202-640-4660

The Latest on X

Kidney Week

Abstract: FR-PO206

Heuristic Is a Good Clinical Servant but a Bad Master: A Case of Delayed Diagnosis of Granulomatosis with Polyangiitis

Session Information

Category: Acute Kidney Injury

  • 103 AKI: Mechanisms

Authors

  • Adonteng-Boateng, Percy, University Hospitals, Cleveland, Ohio, United States
  • Kapp, Meghan, University Hospitals, Cleveland, Ohio, United States
  • Negrea, Lavinia A., University Hospitals, Cleveland, Ohio, United States
Introduction

Failure to connect the different and sometimes scattered symptoms of systemic vasculitis may result in delayed diagnosis of severe illnesses like Granulomatosis with Polyangiitis (GPA).

Case Description

51-year-old male treated for CAP/sinusitis at an outside hospital 3 weeks prior and readmitted with diarrhea, hemoptysis, nausea, vomiting, dark stools, joint pains, epistaxis, and purpuric skin rash on extremities. Infectious work up, ANA with reflex, C3, C4, and hepatitis panel were unremarkable, but cANCA was positive at 1:160. Lactoferrin and calprotectin were elevated. CT abdomen was suggestive of colitis. Patient started on IV solumedrol for presumed inflammatory bowel disease (IBD) versus vasculitis, and transferred to our facility for GI evaluation. Serum creatinine rose from 1.0 to 1.8 mg/dl. UA showed hematuria, pyuria but no proteinuria. Colonoscopy with biopsy showed ulcerated mucosa without viral inclusions or features of IBD. Skin biopsy showed leukocytoclastic vasculitis. Working diagnosis was Henoch Schonlein Purpura, patient changed to oral steroids with plans for outpatient dermatology follow up. Kidney function however worsened so nephrology was consulted and a kidney biopsy pursued. This showed pauci-immune necrotizing crescentic GN. Therefore, patient was diagnosed with GPA and IV solumedrol and Rituximab were started. His clinical manifestations improved, but kidney function did not. He underwent PLEX. Creatinine plateaued at 5 mg/dl. At outpatient follow up visit, it decreased from 5.0 to 3.2 mg/dl. Patient reported feeling overall well with no symptoms recurrence.

Discussion

Recognizing the clinical presentations of rare diseases like ANCA associated vasculitis may help clinicians diagnose and promptly initiate of immunosuppressive therapy while awaiting kidney biopsy. This may yield better kidney prognosis.

Jones’ silver stain 200x: Glomerulus demonstrating breaks in the GBM with associated fibrinoid necrosis.