Nihon Toseki Igakkai Zasshi
Online ISSN : 1883-082X
Print ISSN : 1340-3451
ISSN-L : 1340-3451
Polyarteritis nodosa with Cushing's disease in a patient starting hemodialysis
Kensei YahataChinatsu OkamotoHirotaka ImamakiKoichi SetaAkira ShimatsuAkira Sugawara
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Keywords: MGUS
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2007 Volume 40 Issue 1 Pages 75-81

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Abstract

A 34-year-old male was admitted for fatigue and appetite loss in June 2004. In 1994, he was diagnosed to have Cushing's disease and underwent surgery to remove pituitary adenoma. In 2001, kidney biopsy was performed, since proteinuria and monoclonal serum protein had been detected. Nearly 60% of glomeruli showed global sclerosis, but there was no vasculitis. At the same time, recurrence of the pituitary tumor was note. In 2003, he underwent surgery again, but the tumor was not completely resected. In February 2004, he was involved in a car accident. After the accident, renal insufficiency worsened gradually. After admission, hemodialysis therapy was started. Fever, leukocytosis and increase in C-reactive protein (CRP) were observed. Initially, the symptoms seemed to arise from the hematoma that occurred when the blood access catheter was pulled out. Thereafter, anti-bacterial therapy was initiated since catheter infection was suspected. However, fever, leukocytosis and increased CRP persisted. Tuberculosis was suspected and he was treated accordingly, but there was no improvement. He complained of hypoesthesia of the extremity in July and weakness of the right foot in August. Since neuropathy worsened gradually, he was diagnosed with motor dominant polyneuropathy and treated with high-dose immunoglobulin therapy, because he may have developed chronic inflammatory demyelinated polyneuropathy. Although neuropathy was not relieved, fever, leukocytosis, increased CRP and the general condition did improve. However, the improvement was temporary and the general condition worsened gradually. The patient died at the beginning of November. Autopsy showed the arteritis of small and medium-sized arteries of the kidney, heart, median nerve, testis, stomach, peritoneum and pancreas, indicating polyarteritis nodosa (PAN). A case of PAN accompanied by Cushing's disease is very rare. It was very difficult to diagnose PAN in this case because the patient showed a very intricate clinical course. A disease with poor prognosis such as PAN should always be considered in the differential diagnosis in order to provide adequate treatment.

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© 2007 The Japanese Society for Dialysis Therapy
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