SHM Converge Scientific Abstract ePoster Gallery-Inflammatory Mononeuritis Multiplex: An Unusual Presentation of Anca-Associated Vasculitis (Aav)

Pdf Summary

This case describes a 65-year-old woman with type 1 diabetes complicated by retinopathy and nephropathy, a remote renal transplant, prior breast cancer in remission, sarcoidosis in remission, and hypothyroidism. She presented with three days of rapidly progressive, severe pain, numbness, and weakness affecting all four extremities after a recent episode of acute bronchitis six weeks earlier. She also reported fatigue, diffuse bruising, and myalgias.<br /><br />On exam, her vital signs were normal, but she had marked neurologic deficits consistent with mononeuritis multiplex: absent sensation below both ankles, profound distal weakness in the wrists and feet, and reduced wrist extension. She also had diffuse, non-tender ecchymoses on both upper extremities.<br /><br />Laboratory studies showed elevated inflammatory markers (ESR and CRP) with otherwise unremarkable CBC/CMP except for baseline creatinine elevation. Autoimmune testing was notable for positive anti-myeloperoxidase (MPO) antibodies and a high p-ANCA titer. Urinalysis revealed mild proteinuria and microscopic hematuria. MRI of the brain and spine and CT chest/abdomen/pelvis showed no acute abnormalities or malignancy.<br /><br />A biopsy of the transplanted kidney demonstrated pauci-immune, necrotizing crescentic glomerulonephritis with segmental fibrinoid necrosis, confirming ANCA-associated vasculitis (AAV), most consistent with microscopic polyangiitis. The case highlights an unusual presentation of AAV in which peripheral neuropathy/mononeuritis multiplex was the dominant initial manifestation, without major pulmonary or renal symptoms at presentation.<br /><br />The authors emphasize that clinicians should consider AAV in patients with acute peripheral neuropathy, since earlier diagnosis and treatment improve outcomes. Standard therapy includes pulse-dose steroids followed by a taper plus immunosuppression, with rituximab shown in trials to be more effective than cyclophosphamide for inducing remission.

Asset Subtitle

Vincent M. Del Duca

Meta Tag

Author List Sonia Bharel, Vincent M. Del Duca

Category Clinical Vignettes

Concept ANCA-Associated Vasculitis

Concept Mononeuritis Multiplex

Concept Peripheral Neuropathy

Concept Vasculitic Neuropathy

Concept Granulomatosis with Polyangiitis

Distinguished Non-Finalist

Presenter Organization Thomas Jefferson University

Presenting Author Vincent M. Del Duca

Track Adult

Keywords

ANCA-associated vasculitis

microscopic polyangiitis

mononeuritis multiplex

peripheral neuropathy

MPO-ANCA

p-ANCA

pauci-immune glomerulonephritis

necrotizing crescentic glomerulonephritis

renal transplant

rituximab

ANCA-Associated Vasculitis

Mononeuritis Multiplex

Peripheral Neuropathy

Vasculitic Neuropathy

Granulomatosis with Polyangiitis