약자
1. Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides : AAV
2. Granulomatosis with polyangiitis : GPA
3. Microscopic polyangiitis : MPA
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가능하다면 MPA 또는 GPA (또는 renal-limited vasculitis)의 진단은 의심되는 활동성 병변 부위의 조직검사로 확진되어야 합니다. 그러나 AAV가 임상적으로 의심이 크게 되고 조직을 시기 적절하게 얻을 수 없다면 경험적으로 치료해야 합니다. 진단과 예후를 위한 조직은 보통 피부 또는 신장 조직검사로 얻으며 피부 조직은 비특이적인 leukocytoclastic vasculitis를 나타내고 보체와 면역글로블린은 없거나 거의 없습니다. 신장조직검사는 전형적으로 pauci-immune glomerulonephritis를 나타냅니다. 폐조직검사는 신장 또는 피부조직검사를 대신하여 시행되는데 덜 일반적이겨 이 검사는 종종 도움이 되지 않으므로 transbronchial lung biopsies 또는 nasal biopsies를 거의 시행하지 않습니다. |
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In the absence of renal involvement, the diagnosis of AAV may be made by lung biopsy. Lung biopsy most often requires open or thoracoscopic lung biopsy. In a small number of cases (<10 percent), sufficient tissue for diagnosis can be obtained by transbronchial biopsy of the lung; however, the absence of granulomatous vasculitis on transbronchial specimens should not be considered adequate evidence to exclude the diagnosis of GPA. Thus, we rarely perform transbronchial biopsy in the diagnostic evaluation of AAV. Among patients with MPA who undergo lung biopsy, the typical histological lesion is pulmonary capillaritis. Among patients with GPA, granulomatous inflammation may be seen. Special stains and cultures should be routinely sent to exclude the presence of infections that can produce granulomas (eg, tuberculosis), vasculitis, or necrosis. A positive lung biopsy precludes the need for a kidney biopsy in many cases. However, a renal biopsy is indicated when there is suspicion of an alternative or concomitant disease process occurring in the kidney or if the treatment selected (eg, the use of plasma exchange in addition to immunosuppressive therapy) depends upon the presence of confirmed vasculitis in the kidney. UpToDate 2020.08.22 |
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Pathology Histology remains a very important investigation in newly presenting patients, partly to make a positive diagnosis, partly to inform the severity of involvement, but also to help exclude other causes of the clinical presentation. Although histology from the airways can be nondiagnostic, this can help to ensure that the patient does not have cancer, sarcoid, tuberculosis, or immunoglobulin G4 (IgG4)-related disease, all of which could present in a similar way with upper or lower airway inflammation. Renal histology remains the gold standard to diagnose suspected glomerulonephritis and may be useful in predicting prognosis. Four categories of renal lesions are proposed: focal, crescentic, mixed, and sclerotic. Patients with sclerotic lesions had the worst outcome in terms of long-term renal function after 5 years compared with those with focal lesions, who had the best outcomes. Rheumatology, 7th edition |
