426 - Severe Functional Decline from a Rare Vasculitis: A Case Report

Thursday, February 22, 2024

5:00 PM – 6:30 PM EDT

Presenting Author(s)

Louis Nikolis, MD (he/him/his)

Resident Physician
University of Colorado Department of Physical Medicine and Rehabilitation
Denver, Colorado, United States

Co-Author(s)

AN

Andrea Nikolis, MD

Resident Physician
University of Colorado Department of Physical Medicine and Rehabilitation
Burr Ridge, Illinois, United States
SL

Susan E. Ladley, MD

Associate Professor
Rocky Mountain Regional Veterans Affairs Medical Center
Aurora, Colorado, United States

Case Diagnosis: Mononeuritis Multiplex secondary to Eosinophilic Granulomatosis with Polyangiitis (EGPA)

Case Description:
A 70-year-old male with history of chronic obstructive pulmonary disease presented with cough, shortness of breath, generalized fatigue, along with several months of proximal lower extremity pain and weakness. He was admitted for treatment of multifocal pneumonia. After completing antibiotics, his respiratory symptoms improved, but his weakness progressed to all extremities. Functionally, he began requiring maximal assistance for bed mobility and transfers.

Laboratory findings demonstrated elevated leukocytes, eosinophils, CRP, CK, and IgE, along with MPO and pANCA positivity. Magnetic resonance imaging demonstrated facet hypertrophy and bilateral foraminal stenosis from L3-S1. Muscle biopsy indicated severe granulomatous vasculitis. Electromyography indicated multiple asymmetric, superimposed mononeuropathies (right radial, right ulnar, left median, and left peroneal).

The results of these tests are most consistent with a diagnosis of mononeuritis multiplex (MNM) secondary to EGPA. The patient was treated with glucocorticoids followed by rituximab and was admitted to the acute rehabilitation service for ongoing care.

Discussions: EGPA is a rare systemic vasculitis with a prevalence of 10.7 to 13 per million. Symptoms of EGPA can manifest in the pulmonary, cardiac, gastrointestinal, skin, and nervous systems. Although rare, EGPA has been documented to cause MNM. MNM is an uncommon form of peripheral neuropathy, usually presenting with motor and sensory symptoms in an asymmetric pattern involving two or more peripheral nerves. Treatment options for EGPA with MNM includes glucocorticoids and immunosuppressive therapies.

Conclusions: MNM is a rare condition that can have variable presentations and be difficult to diagnosis. To ensure timely diagnosis and treatment, a physiatrist should be consulted early to ensure competent electrodiagnostic evaluation, assist in identifying a cause, and guide functional goals for rehabilitation. Further, this case should encourage physiatrists to consider MNM as a diagnosis and recognize that there can be rare explanations.