患者進行抗中性粒細胞質抗體檢測的臨床特徵及他們的表現

Clinical characteristics of the patients testing for anti-neutrophil cytoplasmic antibody and their performances

目的：研評估不同科別進行抗嗜中性白血球細胞質抗體（ANCA）檢測的頻率，辨識接受檢測的患者群體，探討與ANCA相關的血管炎（AAV）患者的預後情況，並研究ANCA濃度與臨床表現的相關性。
方法：我們使用電子病歷系統，在2016年1月至2020年8月期間檢索佛教大林慈濟醫院的ANCA檢測患者。我們分析他們接受ANCA檢測的專科部門，以及同時並存的疾病診斷。對於ANCA陽性的患者，我們分析他們的診斷、受影響的器官、後續隨訪情況以及已故患者的死亡原因。
結果：我們發現最常開立ANCA檢測的科別是過敏免疫風濕科（66.2%）、腎臟科（14.2%）和神經科（7.0%）。患有過敏相關診斷的患者佔42%，自體免疫性疾病佔26.3%，慢性腎臟病佔7.1%。在7名anti-PR3-ANCA陽性的患者中，2名腎臟受影響，2名患有潰瘍性結腸炎，1名患有EGPA。而在16名anti-MPO-ANCA陽性的患者中，93.8%腎臟受影響，62.5%患有MPA或AAV，50%患有肺出血，18.8%患有間質性肺疾病。Anti-MPO-ANCA陽性患者（50%）的死亡率高於PR3-ANCA陽性患者（0%）。除了肺出血外，感染性疾病也可能直接導致MPA患者的死亡。
結論：患有MPA且出現肺出血的患者通常面臨著較高的死亡率，尤其考慮到免疫抑制療法後隨之而來的感染性疾病風險。各科醫師應對AAV的可能性保持警惕，並考慮對這些患者進行ANCA血清學檢測。

Objective: To assess the frequency of anti-neutrophil cytoplasmic antibody (ANCA) testing across specialties, identify tested patient populations, explore the prognoses of patients with ANCA-associated vasculitis (AAV), and investigate the correlation of ANCA titers with clinical presentation.
Methods: We searched for ANCA test patients at Buddhist Dalin Tzu Chi Hospital between January 2016 and August 2020 using electronic medical record systems. The specialty departments in which they received ANCA tests and their concurrent diagnoses were analyzed. In patients with positive ANCA, we analyzed their diagnoses, involved organs, follow-up condition and causes of mortality for deceased patients.
Results: We found that ANCA tests were most frequently ordered by the allergy, immunology, and rheumatology department (66.2%), nephrology department (14.2%), and neurology department (7.0%). Patients with allergy-related diagnoses accounted for 42%, autoimmune diseases 26.3%, and chronic kidney disease 7.1%. Among 7 patients with positive proteinase 3 (PR3)-ANCA, 2 had kidney involvement, 2 had ulcerative colitis, 1 had EGPA. Whereas among sixteen patients with positive anti-myeloperoxidase (MPO)-ANCA, 93.8% had kidney involvement, 81.3% had MPA or AAV, 50% had pulmonary hemorrhage and 18.8% had interstitial lung disease. The mortality rate was higher in patients with positive anti-MPO-ANCA (50%) compared to positive anti-PR3-ANCA (0%). Mortality rate was higher in patients with pulmonary hemorrhage and infectious diseases.
Conclusion: Patients with MPA who experience pulmonary hemorrhage typically face a high mortality rate, particularly considering the risk of subsequent infectious diseases following immunosuppressive therapy. Physicians across various departments should remain vigilant for the possibility of AAV and consider testing for the presence of ANCA serology in these patients.