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Abstract: TH-PO569

Wunderlich Syndrome with Polyangiitis Overlap: A Diagnostic Challenge

Session Information

Category: Glomerular Diseases

  • 1401 Glomerular Diseases: From Inflammation to Fibrosis

Authors

  • Savvides, Christopher, University Hospitals, Cleveland, Ohio, United States
  • Paradkar, Komal, University Hospitals, Cleveland, Ohio, United States
  • Linz, Marguerite, University Hospitals, Cleveland, Ohio, United States
  • Pradhan, Nishigandha, University Hospitals, Cleveland, Ohio, United States
Introduction

Spontaneous renal hemorrhage, called “Wunderlich syndrome”, is a rare complication of vasculitides, especially polyarteritis nodosa (PAN). We present a patient with spontaneous renal hemorrhage with polyangiitis overlap syndrome.

Case Description

A white male in his 70s presented with leg pain, weakness, and oliguria. He noted worsening bilateral hearing loss and recurrent sinusitis over 6 months and involuntary 30 lb. weight loss over 3 months. Serum BUN/creatinine were 168 mg/dl/11.2 mg/dl and hemodialysis was started. Initial CT scan of abdomen was negative. He developed new onset left sided abdominal pain after 3 days. Repeat CT scan of abdomen showed bilateral renal hematomas (Fig. A) and multiple left renal aneurysms concerning for PAN (Fig. B) on CT angiogram. pANCA titer was 1:320 (<1:20) with anti-MPO titer of 134 AU/ml (0-19). He met classification criteria for both PAN and Microscopic Polyangiitis (MPA) (Table 1). Kidney biopsy was deferred due to high bleeding risk. Hearing improved after pulse methyl prednisone followed by oral prednisone, cyclophosphamide, and plasma exchange but he remains dialysis dependent six months after discharge.

Discussion

We describe a challenging case with features of both PAN and MPA with bilateral spontaneous renal hemorrhages which precluded tissue diagnosis. This clinical presentation is most consistent with "polyangiitis overlap syndrome" which has not been previously described in association with Wunderlich syndrome. Renal hemorrhage should be considered in patients with ANCA associated vasculitides or polyangiitis overlap syndromes with acute abdominal pain.

1990 ACR Criteria for Classification as PAN (least 3 of 10 criteria)2022 ACR/EULAR Criteria for Classification as MPA, (total score ≥5)
Unexplained weight loss > 4kgpANCA or anti-MPO antibodies positive (+6)
Myalgias (excluding shoulder or hip girdles), muscle weakness
BUN >40 mg/dL or serum creatinine >1.5 mg/dL
Characteristic arteriographic abnormalities not from noninflammatory disease processes

Table 1

Fig. A

Fig. B