A Paradox Unveiled: CMV and ANCA Vasculitis Coexisting Prior to Immunosuppression with Unique Neuroimaging Findings
Jamie Harris1, Hope Hua1, Kinjal Thakor1, Jason Margolesky1
1University of Miami School of Medicine
Objective:

To describe a rare case of simultaneous CMV infection and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), presenting with mononeuritis multiplex and ventral nerve root enhancement, prior to the initiation of immunosuppressive therapy.

Background:
AAV is a group of disorders that affects small-sized arteries, primarily in the respiratory tract and kidneys. An association between AAV and CMV infection is attributed to immunomodulatory therapies. In this case, CMV infection occurs concomitantly with ANCA vasculitis prior to immunosuppression, suggesting the presence of additional intrinsic factors that increase the risk of infection, such as altered peripheral T-cell compartments influenced by environmental or inflammatory factors, including viral infection
Design/Methods:

Case study

Results:

A 72-year-old woman with hypertension presented with one month of rapidly progressive weakness and inability to ambulate. On exam, she had significant distal weakness of her extremities with left hand “benediction sign” median neuropathy, bilateral foot drop and length dependent sensory loss. EMG/NCS was consistent with mononeuritis multiplex. MRI T-spine showed ventral nerve root enhancement. CSF analysis was normal. Additionally, she had purpuric lesions in her palms and soles, proteinuria, and pulmonary nodules. Her hospital course was complicated by an acute GI bleed. Endoscopy showed esophageal and gastric ulcers which were CMV positive and treated with ganciclovir. Rheumatologic workup yielded elevated serum RF (215 IU/ml), ANA (1:80), and Proteinase-3 antibody (35.5 IU/mL) consistent with AAV. She was treated with IV methyprednisolone and rituximab followed by oral steroid taper.  After treatment, patient had improvement of weakness and could ambulate with assistance.

Conclusions:
This case provides reason to investigate alternative hypotheses for ANCA-related associations with CMV infection. For example, viral infection triggering autoimmune disorders through immunomodulation or innate immune dysregulation causing increased susceptibility to viral infections.
10.1212/WNL.0000000000210923
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