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| __NOTOC__
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| {{Wegener's granulomatosis}}
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| {{CMG}}{{APM}}{{AE}}{{KW}}
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| ==Overview==
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| Vasculitis such as Granulomatosis with polyangiitis is usually only suspected when a patient has had unexplained symptoms for a longer period of time. Determination of [[antineutrophil cytoplasmic antibody|ANCA]]s can aid in the diagnosis, but positivity is not conclusive and negative ANCAs are not sufficient to reject the diagnosis. Cytoplasmic staining ANCAs that react with the enzyme [[proteinase 3]] (cANCA) in [[neutrophil granulocyte|neutrophils]] (a type of [[white blood cell]]) are associated with Granulomatosis with polyangiitis.
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| If the patient has [[chronic renal failure|renal failure]] or cutaneous vasculitis, these are the most logical organs to obtain a [[biopsy]] from. Rarely, [[thoracoscopy|thoracoscopic]] lung biopsy is required. On [[histopathology|histopathological]] examination, a biopsy will show ''leukocytoclastic vasculitis'' with [[necrosis|necrotic]] changes and [[granuloma]]tous [[inflammation]] (clumps of typically arranged white blood cells) on [[light microscopy|microscopy]]. The latter is the main reason for the appellation of "Wegener's granulomatosis", although it is not an essential feature. Unfortunately, many biopsies can be non-specific and 50% provide too little information for the diagnosis of Wegener's.
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| ==Diagnostic Criteria==
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| A diagnosis of Granulomatosis with polyangiitis can be made when three out of the six criteria are established. They are:<ref name="pmid27596099">{{cite journal| author=Noone D, Hebert D, Licht C| title=Pathogenesis and treatment of ANCA-associated vasculitis-a role for complement. | journal=Pediatr Nephrol | year= 2016 | volume= | issue= | pages= | pmid=27596099 | doi=10.1007/s00467-016-3475-5 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27596099 }}</ref>
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| # a histopathology that shows granuloma
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| # the upper respiratory tract is involved
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| # there is a stenosis that is present in larynx, trachea, and the bronchioles
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| # the pulmonary system is involved
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| # the presence of anti-neutrophil cytoplasmic antibodies
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| # the presence of glomerulonephritis
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| In 1990, the American College of Rheumatology accepted classification criteria for Granulomatosis with polyangiitis. They were not intended for diagnosis, but for inclusion in [[randomized controlled trial]]s. Two or more positive criteria have a sensitivity of 88.2% and a specificity of 92.0% of describing Granulomatosis with polyangiitis.<ref name=Leavitt>Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. ''Arthritis Rheum'' 1990;33:1101-7. PMID 2202308.</ref>
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| * Nasal or oral inflammation:
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| ** painful or painless oral ulcers ''or''
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| ** purulent or bloody nasal discharge
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| * Lungs: abnormal chest X-ray with:
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| ** nodules,
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| ** infiltrates ''or''
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| ** cavities
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| * Kidneys: urinary sediment with:
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| ** microhematuria''or''
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| ** red cell [[urinary casts|casts]]
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| * Biopsy: granulomatous inflammation
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| ** within the arterial wall ''or''
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| ** in the perivascular area
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| According to the Chapel Hill Consensus Conference (CHCC) on the nomenclature of systemic vasculitis (1992), establishing the diagnosis of Granulomatosis with polyangiitis demands:<ref name=Jenette>Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, Hagen EC, Hoffman GS, Hunder GG, Kallenberg CG,''et al''. Nomenclature of systemic vasculitides. Proposal of an international consensus conference.''Arthritis Rheum'' 1994;37:187-92. PMID 8129773.</ref>
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| * a granulomatous inflammation involving the respiratory tract, and
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| * a [[vasculitis]] of small- to medium-sized vessels.
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| Several investigators have compared the ACR and Chapel Hill criteria.<ref name=Bruce>Bruce IN, Bell AL. A comparison of two nomenclature systems for primary systemic vasculitis. ''Br J Rheumatol''1997;36:453-8. PMID 9159539.</ref>
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| ==References==
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| {{reflist|2}}
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| [[Category:Arthritis]]
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| [[Category:Diseases involving the fasciae]]
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| [[Category:Rheumatology]]
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| [[Category:Nephrology]]
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| [[Category:Pulmonology]]
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| [[Category:Autoimmune diseases]]
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