Pancreatic mass as an initial manifestation of polyarteritis nodosa:A case report and review of the literature

• Published in: World journal of gastroenterology : WJG 2015-01, Vol.21 (3), p.1014-1019
• Main Authors: Yokoi, Yoshihiro, Nakamura, Ippei, Kaneko, Takeshi, Sawayanagi, Tomoki, Watahiki, Youichi, Kuroda, Makoto
• Format: Article
• Language: English
• Subjects: Aged; Antibodies, Antineutrophil Cytoplasmic - blood; Biomarkers - blood; Biopsy; Case Report; Classic; Diagnosis, Differential; Female; Fibrosis; Glomerulonephritis - diagnosis; Humans; Immunosuppressive Agents - therapeutic use; Kidney - immunology; Kidney - pathology; Microscopic Polyangiitis - diagnosis; Microscopic Polyangiitis - immunology; Necrosis; nodosa; Microscopic; Pancreas - blood supply; Pancreas - drug effects; Pancreas - immunology; Pancreas - pathology; Pancreas - surgery; Pancreatic Diseases - blood; Pancreatic Diseases - diagnosis; Pancreatic Diseases - immunology; Pancreatic Diseases - therapy; Pancreaticoduodenectomy; Peroxidase - immunology; polyangii; polyarteritis; Polyarteritis Nodosa - blood; Polyarteritis Nodosa - diagnosis; Polyarteritis Nodosa - immunology; Polyarteritis Nodosa - therapy; Predictive Value of Tests; Tomography, X-Ray Computed; Treatment Outcome
• ISSN: 1007-9327; 2219-2840
• DOI: 10.3748/wjg.v21.i3.1014

Classic polyarteritis nodosa(PAN) that targets mediumsized muscular arteries and microscopic polyangiitis(MPA),characterized by inflammation of small-caliber vessels and the presence of circulating myeloperoxidase anti-neutrophil cytoplasmic antibodies(MPO-ANCA),are distinct clinicopathological entities of systemic vasculitis.A 66-year-old woman presented with fever,cholestasis and positive MPO-ANCA.Radiological examination showed a pancreatic mass compressing the bile duct.Therefore,we performed pancreatoduodenectomy.Histopathological examination revealed that necrotizing vasculitis predominantly affecting the medium-sized vessels,spared arterioles or capillaries in the pancreas,a finding consistent with PAN.Unexpectedly,renal biopsy revealed small-caliber vasculitis and glomerulonephritis,supporting MPA.The initial manifestation of a pancreatic mass associated with vasculitis has only been reported in 7 articles.Its diagnosis is challenging because no reliable clinico-radiological findings have been observed.Clinicians should be aware of such cases and early diagnosis followed by immunosuppression is mandatory.Our findings may reflect a polyangiitis overlap syndrome coexisting between pancreatic PAN and renal MPA.