title: Anti-neutrophil cytoplasmic antibody (ANCA) associated small-vessel vasculitis in a patient with diabetic nephropathy and autoimmune polyendocrinopathy syndrome (APS) Type 2: a case report.
abstract: We present a 42-year-old woman with pre-existing autoimmune polyendocrinopathy syndrome (APS) Type 2 and chronic kidney disease due to Type 1 diabetic nephropathy, who developed a rapid deterioration in renal function due to perinuclear anti-neutrophil cytoplasmic antibody (pANCA)-associated vasculitis. Although possibly a chance occurrence, ANCA have been detected more frequently in patients with a history of certain autoimmune diseases. Such an association may simply reflect an underlying tendency to immune system dysfunction in these patients and the finding of positive ANCA serology does not reliably herald the development of ANCA-associated vasculitis. However, our case illustrates that positive ANCA serology in such circumstances is not always a benign phenomenon and should still be interpreted within the clinical context. Moreover, clinicians managing patients with pre-existing autoimmune disease should maintain a low threshold for appropriate assessment should such patients develop evidence suggestive of vasculitis.
authors.forename: Jonathan S
authors.initials: JS
authors.lastname: Murray
authors.forename: Laura A
authors.initials: LA
authors.lastname: Baines
authors.forename: Simon H S
authors.initials: SH
authors.lastname: Pearce
authors.forename: Steve
authors.initials: S
authors.lastname: Ball
authors.forename: Nicola
authors.initials: N
authors.lastname: Leech
authors.forename: Katrina M
authors.initials: KM
authors.lastname: Wood
authors.forename: Nigel S
authors.initials: NS
authors.lastname: Kanagasundaram
mesh_terms: D000328:Adult; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000097:blood / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D015415:Biomarkers / Q000097:blood; D001706:Biopsy; D003928:Diabetic Nephropathies / Q000097:blood / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy; D018450:Disease Progression; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007668:Kidney / Q000473:pathology; D007676:Kidney Failure, Chronic / Q000276:immunology / Q000628:therapy; D010530:Peritoneal Dialysis; D010951:Plasma Exchange; D016884:Polyendocrinopathies, Autoimmune / Q000097:blood / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy; D016896:Treatment Outcome
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D015415:Biomarkers; D007166:Immunosuppressive Agents