Anti-Neutrophil Cytoplasmic Antibodies

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Immunofluorescence imaging of c-ANCA

Anti-neutrophil cytoplasmic antibodies , abbreviated to ANCA , are certain autoantibodies , i.e. antibodies produced by the body against the body's own leukocytes . They are detected in the laboratory by means of immunofluorescence . The search for ANCA in serum is indicated for the following diseases:

  1. pulmonary bleeding
  2. cutaneous vasculitis with systemic characteristics
  3. Mononeuritis multiplex
  4. peripheral neuropathy
  5. prolonged sinusitis or otitis
  6. Tracheal stenosis
  7. extracapillary proliferative glomerulonephritis ("kidney corpuscle disease")

If a patient has any of these diseases, a positive ANCA result suggests vasculitis . Based on the fluorescence pattern on ethanol-fixed neutrophilic granulocytes, a distinction is made between c (= c ytoplasmic) -ANCA and p (= p erinuclear) -ANCA.

c-ANCA

c-ANCA are characteristic of granulomatosis with polyangiitis (formerly Wegener's granulomatosis) and are directed against the enzyme proteinase 3 . The latter is a serine protease of the cytoplasmic granules of neutrophil granulocytes. Accordingly, the antibodies are also referred to as PR3-ANCA (proteinase-3 anti-neutrophil cytoplasmic antibodies).

p-ANCA

p-ANCA occur in microscopic polyangiitis , eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome), primary sclerosing cholangitis and idiopathic glomerulonephritis . They are directed against the enzyme myeloperoxidase .

etiology

The cause (etiology) of ANCA formation is unknown. A combination of environmental factors and genetic predisposition may be required. Possible causes of ANCA-associated vasculitis are discussed:

literature

  • Frontiers in Nephrology: ANCA-Associated Vasculitis - Guest Editor Charles D. Pusey. In: J Am Soc Nephrol , 2006, 17, pp. 1221–1223 - Series of review articles on the current state of basic research (English)

Individual evidence

  1. Dimitrios-P Bogdanos, Pietro Invernizzi, Ian-R Mackay, Diego Vergani: Autoimmune liver serology: current diagnostic and clinical challenges . In: World Journal of Gastroenterology: WJG . 14, No. 21, June 7, 2008, ISSN  1007-9327 , pp. 3374-3387. PMID 18528935 .
  2. ^ Robert AF de Lind van Wijngaarden et al .: Hypotheses on the Etiology of Antineutrophil Cytoplasmic Autoantibody Associated Vasculitis: The Cause Is Hidden, but the Result Is Known . In: Clin J Am Soc Nephrol . No. 3 , 2008, p. 237-252 , doi : 10.2215 / CJN.03550807 ( abstract ).