Al Arfaj Abdurhman Saud, Khalil Najma
Prof. Abdurhman Saud Al Arfaj, FRCP©, FACP, Division of Rheumatology, Department of Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Najma Khalil, M.Sc., M.Phil, Division of Rheumatology, College of Medicine Research Center, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Pak J Med Sci. 2018 Jan-Feb;34(1):88-93. doi: 10.12669/pjms.341.13881.
To explore clinical and laboratory features, therapy and outcome of antineutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV) patients from our tertiary care center.
This study of AAV patients seen in Rheumatology clinics at King Khalid University hospital (KKUH), King Saud University, Riyadh during the period 1990-2014 was carried out retrospectively. Demographic, clinical, haematological and immunological parameters along with therapy, complications and outcome were retrieved from patients' medical charts. Different characteristics were compared between the three groups of AAV; GPA (Granulomatosis with polyangiitis), MPA (Microscopic polyangiitis) and EGPA (Eosinophilic granulomatosis with polyangiitis).
We identified 34 AAV patients (21 males: 13 females; 31 Saudis: 3 non-Saudis) comprising of 23 GPA, 2 MPA and 9 EGPA cases. The mean age of onset was 42.1±17.6 years (range 11-75) and mean duration of disease was 8.7± 5.1 years (range 1-20). The most frequently affected system was pulmonary in all AAV (73.5%), GPA (65.2%) and EGPA (100%) while it was renal in MPA (100%) patients. Ophthalmological and upper airways involvement was higher in GPA. Neurological involvement was higher in EGPA (p<0.05). ANCA were detected in 79.4% of AAV patients, of them c-ANCA were 77.8% and p-ANCA 22.2%. ANCA was positive in 91.3% GPA, 100% MPA and 44.4% EGPA patients. In GPA c-ANCA were detected in 80.9% and p-ANCA in 17.4%, in MPA, c-ANCA were detected in 50% and p-ANCA in 50%, in EGPA, c-ANCA were observed in 75% and p-ANCA in 25%. GPA patients had PR3 specificity in 93.3%, and MPO in 6.7%, PR3 was present in all MPA patients (100%), while EGPA patients had MPO (100%). Therapy administered were corticosteroids (100%), intravenous cyclophosphamide (58.8%), azathioprine (50%) and rituximab (11.8%). Infections were noted in 29.4%.
The 10-year survival in our AAV patients was 95%. ANCA pattern was similar to Caucasian AAV patients and different from Japanese and Chinese AAV patients.
探索我院三级医疗中心抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）患者的临床和实验室特征、治疗方法及预后情况。
本研究对1990年至2014年期间在利雅得沙特国王大学哈利德王储医院（KKUH）风湿科门诊就诊的AAV患者进行回顾性分析。从患者病历中获取人口统计学、临床、血液学和免疫学参数以及治疗情况、并发症和预后信息。对AAV的三组患者，即肉芽肿性多血管炎（GPA）、显微镜下多血管炎（MPA）和嗜酸性肉芽肿性多血管炎（EGPA）的不同特征进行比较。
我们共纳入34例AAV患者（21例男性，13例女性；31例沙特人，3例非沙特人），其中包括23例GPA、2例MPA和9例EGPA患者。平均发病年龄为42.1±17.6岁（范围11 - 75岁），平均病程为8.7±5.1年（范围1 - 20年）。所有AAV患者中最常受累的系统是肺部（73.5%），GPA患者中为65.2%，EGPA患者中为100%，而MPA患者中最常受累的系统是肾脏（100%）。GPA患者眼部和上呼吸道受累更为常见。EGPA患者神经系统受累更为常见（p<0.05）。79.4%的AAV患者检测到ANCA，其中胞浆型ANCA（c-ANCA）占77.8%，核周型ANCA（p-ANCA）占22.2%。91.3%的GPA患者、100%的MPA患者和44.4%的EGPA患者ANCA呈阳性。在GPA患者中，80.9%检测到c-ANCA，17.4%检测到p-ANCA；在MPA患者中，50%检测到c-ANCA，50%检测到p-ANCA；在EGPA患者中，75%检测到c-ANCA，25%检测到p-ANCA。GPA患者中93.3%具有蛋白酶3（PR3）特异性，6.7%具有髓过氧化物酶（MPO）特异性；所有MPA患者（100%）均有PR3；而EGPA患者均有MPO（100%）。使用的治疗方法包括糖皮质激素（100%）、静脉注射环磷酰胺（58.8%）、硫唑嘌呤（50%）和利妥昔单抗（11.8%）。29.4%的患者出现感染。
我们的AAV患者10年生存率为95%。ANCA模式与白种人AAV患者相似，与日本和中国AAV患者不同。
