Vasculitic Neuropathy With Iron Deficiency-Related Chronic Inflammation Followed by Elevated Serum Vascular Endothelial Growth Factor: A POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes) Syndrome Mimic.

The causes of peripheral neuropathy are diverse and include numerous systemic diseases. Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a pertinent condition and is characterized by elevated serum vascular endothelial growth factor (VEGF). However, several other diseases with elevated VEGF have been reported besides POEMS syndrome. We report a case of vasculitic neuropathy with iron deficiency-related chronic inflammation, refractory ascites, and high VEGF levels, which was difficult to differentiate from POEMS syndrome. A 66-year-old female presented with fever, lower leg edema, and intractable ascites. She had also experienced tingling sensations and muscle weakness in the right hand and lower limbs in the course of her illness. Neurological findings revealed distal muscle weakness, abnormal sensations, and loss of lower limb tendon reflexes and vibrations. Blood tests revealed anemia with iron deficiency, low unsaturated iron-binding capacity, and high ferritin. The serum VEGF level was markedly elevated, and myeloperoxidase anti-neutrophil cytoplasmic antibody, M protein, and anti-Sjögren's syndrome-B antibodies were positive. Nerve conduction studies showed axonal impairment, predominantly in the lower limbs, whereas nerve ultrasonography revealed extensive nerve thickening in the extremities. A definitive diagnosis was not reached despite these investigations. The lower limb nerve and muscle biopsies led to the diagnosis of vasculitic neuropathy. In this case, a nerve biopsy was important for a definitive diagnosis. It should be noted that serum VEGF may be elevated not only in POEMS syndrome but also in other disorders such as vasculitic neuropathy and iron deficiency-related chronic inflammation.