Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV)는 large-vessel vasculitis, medium-vessel vasculitis가 아닌 small-vessel vasculitis에 해당합니다. AAV는 주로 작은 혈관에 영향을 미치며 특별한 myeloperoxidase (MPO-ANCA)와 proteinase 3 (PR3-ANCA)와 관련이 있습니다.
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV)는 면역복합제 침착과 관련이 상당히 없는 necrotizing vasculitis입니다.
ANCA-negative AAV ?
ANCA-negative AAV는 특히 eosinophilic granulomatosis with polyangiitis (EGPA)에서 생기는데 어느 정도는 granulomatosis with polyangiitis (GPA)에서도 생깁니다. 이것은 AAV에 대한 다른 기준들은 만족하지만 ANCA에 대한 혈청학적 검사가 음성입니다.
AAV의 주된 임상병리학적 유형으로는 microscopic polyangiitis (MPA), GPA, EGPA가 있으며 또한 AAV는 여러 기관이 아닌 유일하게 한 기관에서만 발생할 수도 있습니다. 예) renal-limited AAV
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Microscopic polyangiitis
MPA is a necrotizing vasculitis that primarily affects capillaries, venules, or arterioles, most commonly manifesting as necrotizing glomerulonephritis and/or pulmonary capillaritis. Involvement of medium- and small- sized arteries may also be present. Granulomatous inflammation is usually absent. ANCA is present in >90 percent of patients with MPA. |
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Granulomatosis with polyangiitis
GPA is a necrotizing vasculitis predominantly involving small- to medium-sized vessels (eg, capillaries, venules, arterioles, arteries, and veins). It typically produces granulomatous inflammation of the upper and lower respiratory tracts as well as necrotizing, pauci-immune glomerulonephritis. ANCA is present in >80 percent of patients with GPA
While MPA and GPA continue be regarded as distinct entities within AAV, they have markedly overlapping manifestations and it can be sometimes extremely difficult to differentiate between these two diseases within a patient. Furthermore, there is a growing recognition that ANCA type (anti-MPO or anti-PR3) has more prognostic and clinical meaning rather than the disease type (MPA or GPA), leading some experts to refer to MPO-AAV or PR3-AAV, and many clinical trials in AAV now stratify enrollment by ANCA type (MPO or PR3) and report results for each subgroup. |
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Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
EGPA is an eosinophilic-rich necrotizing vasculitis predominantly affecting small- to medium-sized vessels. Patients often have chronic rhinosinusitis, asthma, and prominent peripheral blood eosinophilia. ANCA is present in approximately 40 percent of patients with EGPA, usually anti-MPO ANCA. The presence of ANCA is more frequent in patients with glomerulonephritis. |
REF. UpToDate 2020.08.22
