Case Reports: Mimics of Polyarteritis Nodosa

Discussion

Rare, non-atherosclerotic, non-inflammatory arteriopathies, vEDS and SAM are potentially life-threatening, as indicated in our cases. Both conditions can present with imaging findings that mimic PAN and can similarly lead to devastating complications including aneurysm formation, dissection and spontaneous vascular rupture.

Despite the potential similarities with regards to presentation, vEDS and SAM are managed differently than PAN. Given immune-mediated underpinnings, PAN typically requires treatment with systemic glucocorticoids and immunosuppres­sants, such as mycophenolate mofetil or metho­trexate. In severe, organ- or life-threatening disease, cyclophosphamide may be used.³⁵ In contrast, management of vEDS and SAM do not involve immunosuppression and are primarily preventive and supportive—including regular vascular surveillance, strict blood pressure control and lifestyle modifications (e.g., avoidance of unnecessary invasive procedures and trauma). In the setting of complications, such as hemorrhage, ischemia or rapid evolution of vascular defects, surgery, endovascular embolization or stenting may be required.^18,28

Ultimately, vEDS and SAM should be considered in the differential for patients presenting with clinical features or imaging suggestive of PAN, especially in the absence of systemic symptoms, because accurate diagnosis can have profound impacts on management.

Shane Murray, BMBS, RhMSUS, obtained his medical degree at the University of Limerick, Ireland. He completed an internal medicine residency at Mount Sinai Morningside & Mount Sinai West Hospitals, New York, and a rheumatology fellowship at Columbia University Irving Medical Center, N.Y. He joined the Department of Rheumatology at the Yale School of Medicine in 2024.

Zhe Ran Duan, MD, PhD, obtained her medical degree at Weill Cornell Medical College, New York. She completed an anatomic pathology residency at Columbia University Irving Medical Center, New York, and is currently completing a surgical pathology fellowship there.

Daniel DeMizio, MD, obtained his medical degree at Albert Einstein College of Medicine in New York. He completed his internal medicine residency at Mount Sinai Hospital in New York and his rheumatology fellowship at Columbia University Irving Medical Center, N.Y. He has served as chief of rheumatology at Richmond University Medical Center, New York, since 2023.

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