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Abstract: PO1947

Severe Vasculitis Masquerading as Guillain-Barre Syndrome

Session Information

Category: Pathology and Lab Medicine

  • 1600 Pathology and Lab Medicine

Authors

  • Abifaraj, Farah, Vanderbilt University Medical Center, Nashville, Tennessee, United States
  • Lewis, Julia, Vanderbilt University Medical Center, Nashville, Tennessee, United States
  • Fogo, Agnes B., Vanderbilt University Medical Center, Nashville, Tennessee, United States
Introduction

Polyarteritis nodosa (PAN) is a rare ANCA-negative, necrotizing arteritis affecting small-medium arteries. Due to rarity of the disease, studies are limited. We present a case of PAN with missed diagnosis at presentation and complicated treatment course.

Case Description

75-year-old man with hypertension diagnosed 10 months ago presented to outside hospital with bilateral lower extremity ascending numbness and weakness, and arthralgia. Lumbar puncture was inconclusive. He was treated for Guillain-Barre syndrome with IVIG with some improvement.
Two months later, he presented to our hospital with localized petechial rash, oliguria, lower extremity edema, right foot drop, marked weakness, and acute kidney injury (creatinine (Cr) 4.09mg/dL). He had hematuria, subnephrotic range proteinuria, high inflammatory markers, and low complements. Renal biopsy revealed necrotizing medium vessel arteritis, consistent with PAN. He was started on steroids and oral cyclophosphamide (CYC) with rapid, dramatic improvement in neurologic symptoms.
Unfortunately, a month later, he developed acute hypoxia with worsening multifocal opacities despite adequate diuresis and infectious work-up, including negative bronchoalveolar lavage. Pneumonitis from CYC toxicity was diagnosed. Hypoxia resolved with steroids and replacing CYC with mycophenolate mofetil (MMF). Cr improved to 2.2mg/dL with continued improvement in neuromuscular symptoms.

Discussion

This is a rare presentation of vasculitis causing severe AKI, debilitating peripheral neuropathy and weakness. Neurologic and renal manifestations improved rapidly with treatment. Development of CYC-induced pneumonitis leading to transition to MMF for induction is a rare complication, resulting in rare, but effective treatment. Lastly, development of new onset hypertension in the 8th decade of life warrants work-up for secondary etiologies.

Transmural fibrinoid necrosis of medium-sized renal artery with surrounding inflammatory cells