Talwar Deepak, Vadala Rohit, Talwar Surbhi, Pahuja Sourabh, Prajapat Deepak
Pulmonary and Critical Care Medicine, Metro Centre for Respiratory Disease, Noida, IND.
Nephrology, University Hospital Coventry and Warwickshire, Coventry, GBR.
Cureus. 2022 Jan 17;14(1):e21327. doi: 10.7759/cureus.21327. eCollection 2022 Jan.
Pulmonary renal syndrome (PRS) is a simultaneous occurrence of diffuse alveolar hemorrhage (DAH) and glomerulonephritis (GN). The diagnosis of PRS not only requires a high index of clinical suspicion and prompt management, but it is often fatal due to rapidly progressive clinical deterioration despite aggressive treatment. The authors, therefore, share the real-world experience of PRS presenting to tertiary care pulmonary center in north India.
The objectives of the study were to identify etiology, clinical manifestations, treatment modalities and outcomes of patients presenting with PRS.
MATERIALS & METHODS: This was a retrospective observational study undertaken at Metro Centre for Respiratory Diseases of patients diagnosed with PRS during the last two years between 2019 and 2021. The patients diagnosed with PRS based on clinical manifestations, serology and biopsies were included in the study. All cases of non-immunological causes of PRS were excluded from the study. Chi-square and Mann-Whitney U tests were done to look for associations obtained between survivors and non-survivors. Cox regression analysis was done to estimate the hazard ratios of clinical variables on survival in PRS patients.
A total of 12 patients of PRS were included in the study and diagnosis was made based on clinical manifestations, serology as well as biopsies. The mean age of presentation was 45.4 (± 17.8) years and 66.7% of the patients were females. The most common etiology was anti-nuclear cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) seen in 83.3% of the cases. The most common symptoms were coughing and fever (80%) followed by dyspnea and hemoptysis (70%) with the mean duration of symptoms being 17.1 (±8.9) days. The mortality of PRS patients in our study was 41.6% and these patients had a higher acute physiology and chronic health evaluation (APACHE) score (median-26) compared to those patients who survived (median - 15.8).
The occurrence of PRS, although rare, presents with rapid clinical deterioration leading to a high mortality rate. AAV was the most common cause of PRS as observed in our study. Early recognition and prompt aggressive management strategies with immunosuppressant therapies are essential for better outcomes for the patients.
肺肾综合征（PRS）是弥漫性肺泡出血（DAH）和肾小球肾炎（GN）同时出现的病症。PRS的诊断不仅需要高度的临床怀疑指数和及时的处理，而且尽管积极治疗，其临床病情迅速恶化常导致死亡。因此，作者分享了印度北部一家三级医疗肺科中心接诊的PRS的实际病例经验。
本研究的目的是确定PRS患者的病因、临床表现、治疗方式及预后。
这是一项在地铁呼吸疾病中心进行的回顾性观察研究，研究对象为2019年至2021年这两年间被诊断为PRS的患者。基于临床表现、血清学和活检诊断为PRS的患者被纳入研究。所有非免疫性病因导致的PRS病例均被排除在研究之外。采用卡方检验和曼-惠特尼U检验来寻找幸存者与非幸存者之间的关联。进行Cox回归分析以估计临床变量对PRS患者生存的风险比。
本研究共纳入12例PRS患者，诊断基于临床表现、血清学及活检。患者的平均就诊年龄为45.4（±17.8）岁，66.7%为女性。最常见的病因是抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV），见于83.3%的病例。最常见的症状是咳嗽和发热（80%），其次是呼吸困难和咯血（70%），症状的平均持续时间为17.1（±8.9）天。本研究中PRS患者的死亡率为41.6%，与存活患者（中位数 - 15.8）相比，这些患者的急性生理与慢性健康状况评分（APACHE）更高（中位数 - 26）。
PRS的发生虽然罕见，但临床病情迅速恶化，导致死亡率很高。如我们的研究所观察到的，AAV是PRS最常见的病因。早期识别并及时采取积极的免疫抑制治疗管理策略对患者获得更好的预后至关重要。
