Gokhale Yojana, Rathod Raosaheb, Trivedi Trupti, Awadh N T, Deshmukh Utkarsh, Jadhav Lalana, Pawar Amol
Professor of Medicine, In-Charge of Rheumatology Services,Lokmanya Tilak Municipal Medical College, Mumbai, Maharashtra;Corresponding Author.
Resident in Medicine, Lokmanya Tilak Municipal Medical College, Mumbai, Maharashtra.
J Assoc Physicians India. 2018 Jul;66(7):13-17.
Pulmonary Renal Syndrome (PRS), is characterized by diffuse alveolar haemorrhage (DAH) and glomerulonephritis (GN), occurring simultaneously. It has high mortality and dialysis dependence at one year, if not timely diagnosed and aggressively treated.
To study etiology and short term outcome of PRS in India.
This study included patients of PRS seen in a tertiary care center in Mumbai, by one consultant from 1997- 2013, analyzed retrospectively and from January 2014 to December 2015 collected prospectively from six medical units, intensive care unit, nephrology and respiratory units. Patients with DAH (haemoptysis, breathlessness and x-ray chest with bilateral alveolar shadows with sparing of apices) and glomerulonephritis (Proteinuria, heamaturia, hypertension with or without raised serum Creatinine) were included in the study after carefully excluding other causes of haemoptysis and breathless like tuberculosis, pulmonary oedema, pneumonia, ARDS. During prospective enrollment of patients, in all admitted patients with haemoptysis, urine examination was carried out to specifically look for proteinuria and red blood cells in urine, same was also followed in those admitted for breathlessness with chest x-ray suggestive of alveolar haemorrhage. Patients were extensively investigated for etiology and were treated with steroids and pulse cyclophosphamide (after ruling out infectious etiology). Supportive care with ventilator or dialysis was given as per usual indications. Palsmapheresis was initiated in those with serum Creatinine ≥ 5.7mg/dl. Rituximab was used in refractory cases, as per treating physicians' choice. Final outcome was death or discharge.
There were 25 patients of PRS (13 retrospective, 12 prospective), with following etiology : Granulomatosis with polyangiitis (GPA) 7, Microscopic polyangiitis (MPO) 4, Churg Strauss Syndrome (EGPA) 1, Goodpasture's syndrome 1, lupus 5, leptospirosis 5, dengue 2. All were given steroids, 18 (72%) were given pulse Cyclophosphamide (barring those with leptospirosis and dengue), ventilator support in 14 (56%) patients (8 invasive, 6 non-invasive), haemodialysis 3, plasmapheresis 1, Rituximab 2. Seventeen (68%) patients survived, mortality was high in those requiring invasive ventilator.
Most common etiology of PRS is ANCA positive vasculitis in India. With high degree of suspicion for DAH in patients presenting with haemoptysis, breathlessness and alveolar opacities in chest x-ray and carefully investigating by simple urine examination for evidence of GN, timely diagnosis of PRS can be made. With timely appropriate treatment survival is 68%. Patients with PRS due to leptospirosis or dengue have features suggestive of underlying disease (like icterus with raised bilirubin but < 200U SGOT/SGPT, subconjunctival haemorrhage, typical rash of dengue with thrombocytopenia).
肺肾综合征（PRS）的特征是弥漫性肺泡出血（DAH）和肾小球肾炎（GN）同时发生。如果不及时诊断和积极治疗，其一年死亡率和透析依赖率较高。
研究印度PRS的病因及短期预后。
本研究纳入1997年至2013年在孟买一家三级医疗中心由一位顾问诊治的PRS患者，进行回顾性分析，并前瞻性收集2014年1月至2015年12月来自六个医疗科室、重症监护室、肾病科和呼吸科的患者资料。在仔细排除咯血和呼吸困难的其他病因（如肺结核、肺水肿、肺炎、急性呼吸窘迫综合征）后，纳入有DAH（咯血、呼吸困难及胸部X线显示双侧肺泡阴影且肺尖未受累）和肾小球肾炎（蛋白尿、血尿、高血压伴或不伴血清肌酐升高）的患者。在患者前瞻性入组期间，对所有咯血入院患者进行尿液检查，以特别查找蛋白尿和尿中红细胞，对胸部X线提示肺泡出血的呼吸困难入院患者也进行同样检查。对患者进行广泛的病因调查，并在排除感染性病因后用类固醇和环磷酰胺冲击治疗。根据常规指征给予呼吸机或透析支持治疗。血清肌酐≥5.7mg/dl的患者开始进行血浆置换。根据治疗医生的选择，难治性病例使用利妥昔单抗。最终结局为死亡或出院。
共有25例PRS患者（13例回顾性病例，12例前瞻性病例），病因如下：肉芽肿性多血管炎（GPA）7例，显微镜下多血管炎（MPO）4例，变应性肉芽肿性血管炎（EGPA）1例，肺出血肾炎综合征1例，狼疮5例，钩端螺旋体病5例，登革热2例。所有患者均给予类固醇治疗，18例（72%）给予环磷酰胺冲击治疗（钩端螺旋体病和登革热患者除外），14例（56%）患者接受呼吸机支持（8例有创，6例无创），3例接受血液透析，1例接受血浆置换，2例使用利妥昔单抗。17例（68%）患者存活，需要有创呼吸机支持的患者死亡率较高。
在印度，PRS最常见的病因是抗中性粒细胞胞浆抗体（ANCA）阳性血管炎。对于咯血、呼吸困难且胸部X线有肺泡混浊的患者高度怀疑DAH，并通过简单的尿液检查仔细排查GN证据，可及时诊断PRS。及时恰当治疗后生存率为68%。由钩端螺旋体病或登革热引起的PRS患者有潜在疾病的特征（如黄疸伴胆红素升高但<200U SGOT/SGPT、结膜下出血、典型登革热皮疹伴血小板减少）。
