ABSTRACT
Peripheral neuropathy is a common manifestation of systemic vasculitis. The etiology of vasculitic peripheral neuropathy is generally classified into two groups: systemic and nonsystemic. In systemic vasculitic neuropathy (SVN), neuropathy is a consequence of a systemic disease, most commonly involving medium and small vessels throughout the body. There are three main clinical presentations: multifocal neuropathy, distal symmetric polyneuropathy, and overlapping multifocal neuropathy. Specifically, distal symmetric polyneuropathy affects multiple somatic nerves diffusely in a symmetric and length-dependent pattern (also known as the classic stocking-glove pattern). This case represents an atypical presentation of SVN, presenting with widespread symmetric polyneuropathy.A 73-year-old woman presented with distal acute on chronic bilateral upper and lower extremity weakness, sensory changes, and widespread pain. Symptoms started about three months prior and gradually worsened with progressive difficulty with ambulation and required assistive devices. Elevated ESR is at 70 mm/hour, CRP at 25.66 mg/dL, elevated c-ANCA titers at 1 : 320 and PR3 at 5.0 AI, and elevated creatine kinase (CK) at 500-600 U/L. A muscle biopsy of the left vastus showed neurogenic atrophy without myositis. Initial improvement was with oral prednisone, but was stopped on discharge. Many purpuric and petechial lesions were developed on distal legs/feet and right fourth digit distal gangrene. EMG showed distal, symmetric, and axonal polyneuropathy affecting the upper and lower extremities and acute denervation in more distal muscles. The patient received pulse dose steroids and two doses of rituximab induction therapy and was discharged with an oral steroid taper. The patient's symptoms started as distal symmetric neuropathy at the onset and progressively worsened over the course of 3 months. Neuropathy, both on the exam and on EMG, seemed to have developed more rapidly than expected, regardless of its distribution. The EMG showed severe peripheral nerve damage and denervation, which is unusual for ANCA-associated systemic vasculitis.
ABSTRACT
OBJECTIVE: The objective of this article is to investigate clinical presentations and outcomes of systemic lupus erythematosus (SLE) patients with infection admitted to the intensive care unit (ICU). METHODS: SLE patients with infection, SLE patients with noninfectious causes, and non-SLE patients with infection were identified from the Cooper University Hospital Project IMPACT database between 2002 and 2010. We examined demographic data, APACHE II scores, physiologic data, laboratory data, length of stay in the ICU and hospital, and mortality of the three groups. RESULTS: Twenty-five SLE patients with infection, 45 SLE patients with noninfectious causes, and 1466 non-SLE patients with infection were included in the study. SLE patients with infection had higher APACHE II scores, higher maximum temperature, higher minimum and maximum heart rate (HR), lower minimum and maximum systolic blood pressure (SBP), and longer ICU length of stay in comparison to SLE patients with noninfectious causes. There were no statistical differences in white blood cell (WBC) count. SLE patients with infection had a higher mortality compared to SLE patients with noninfectious causes. There was no difference in mortality between SLE patients with infection and non-SLE patients with infection. CONCLUSION: SLE patients with infection in the ICU had a higher mortality and a higher APACHE II score compared to SLE patients with noninfectious causes in the ICU. Their physiologic signs including temperature, HR, and SBP were more reflective of infection than their WBC count.
