Rinsho Shinkeigaku (Clinical Neurology)

Case Report

An autopsy case of polyarteritis nodosa accompanied with multiple immune-specific autoantibodies and rhabdomyolysis

Masako Takeuchi, M.D.1)2), Takenobu Murakami, M.D., Ph.D.1)2), Naoya Noguchi, M.D., Ph.D.3), Yuki Tajiri, M.D., Ph.D.1)2), Masafumi Kishi, M.D., Ph.D.1), Mayuko Sakuwa, M.D.2), Satoshi Kuwamoto, M.D., Ph.D.4), Tadashi Adachi, M.D., Ph.D.5) and Ritsuko Hanajima, M.D., Ph.D.2)

1) Department of Neurology, Tottori Prefectural Kousei Hospital
2) Division of Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University
3) Department of Gastroenterology, Tottori Prefectural Kousei Hospital
4) Department of Pathology, Faculty of Medicine, Tottori University
5) Division of Neuropathology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University

A 72-year-old male complained of fever lasting 1 month and developed muscle weakness and paresthesia in the legs. He presented with muscle weakness, grasping pain, decreased deep tendon reflexes in the extremities, and reduction of tactile sensation in the distal parts of the left leg muscles. Blood tests revealed leukocytosis and inflammatory reactions. Collagen-disease-specific autoantibodies including anti-double-stranded DNA and anti-Scl-70 antibodies were positive, but antineutrophil cytoplastic antibodies were negative. Nerve conduction studies revealed asymmetric axonal degeneration, indicating multiple mononeuropathy. We started intravenous methylprednisolone pulse and plasma exchange therapies. However, the patient developed intestinal necrosis and perforation, and he died 44 days after the onset of fever. An autopsy revealed vasculitis in small- to medium-sized vessels in multiple organs as well as myoglobin casts in the renal tubules, which were suggestive polyarteritis nodosa (PAN) accompanied with rhabdomyolysis. Positivity for collagen-disease-specific autoantibodies and accompanying rhabdomyolysis are atypical findings with PAN. This patient was not clinically diagnosed as PAN, and so promptly starting immunotherapies should be considered when a case presents with evidence of vasculitis.
Full Text of this Article in Japanese PDF (4445K)

(CLINICA NEUROL, 63: 21|26, 2023)
key words: polyarteritis nodosa, rhabdomyolysis, multiple mononeuropathy

(Received: 9-Jun-22)