Rajagopala Srinivas, Parameswaran Sreejith, Ajmera Jail Singh, Ganesh Rajesh Nachiappa, Katrevula Anudeep
Department of Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research, Gorimedu, Puducherry, India.
Department of Nephrology, Jawaharlal Institute of Postgraduate Medical Education and Research, Gorimedu, Puducherry, India.
Int J Rheum Dis. 2017 Jan;20(1):109-121. doi: 10.1111/1756-185X.12818. Epub 2016 Feb 4.
To describe the spectrum of pulmonary involvement in immunoglobulin A nephropathy (IgAN).
We describe two patients with pulmonary renal syndrome related to IgAN and a systematic review of previously reported cases of IgAN and lung involvement.
We identified 23 reports of IgAN-related pulmonary disease, including 19 reports of alveolar hemorrhage and two cases of organizing pneumonia. Dyspnea (84%), hemoptysis (74%), cough (53%) and fever (47%) were common presenting complaints. Simultaneous involvement of kidneys and lung was the most common presentation (42%) but alveolar hemorrhage occurred independent of renal disease in one-fifth (21%). Azotemia was seen in 55.5% at presentation. Mesangio-proliferative glomerulonephritis was the most common biopsy finding and crescentic glomerulonephritis was seen in 27.7%. Among patients undergoing lung biopsy, capillaritis was seen in 72.7%; 37.5% of these had IgA deposits. Steroids with cyclophosphamide, followed by maintenance with methotrexate or azathioprine was used in 44%. Mechanical ventilation, dialysis and plasmapheresis were other adjunctive therapies used. IgAN-related alveolar hemorrhage was associated with a mortality of 26.3% and significant morbidity, with 52.7% having end-stage kidney disease despite immunosuppression. Organizing pneumonia with pulmonary IgA deposition is a well-described association of IgAN.
These findings are similar to our previous observations of Henoch-Schonlein purpura (HSP)-related alveolar hemorrhage, highlighting the similarities of these related syndromes. Multicentric studies of IgAN and HSP-related pulmonary renal syndrome with a standard protocol are needed to define their similarities and differences, optimum suppression and its role in preventing renal progression in this setting.
描述免疫球蛋白A肾病（IgAN）肺部受累的情况。
我们描述了2例与IgAN相关的肺肾综合征患者，并对先前报道的IgAN合并肺部受累病例进行了系统回顾。
我们确定了23篇关于IgAN相关肺部疾病的报告，包括19篇肺泡出血报告和2例机化性肺炎病例。常见的临床表现为呼吸困难（84%）、咯血（74%）、咳嗽（53%）和发热（47%）。肾脏和肺部同时受累是最常见的表现（42%），但五分之一（21%）的肺泡出血独立于肾脏疾病发生。就诊时55.5%的患者出现氮质血症。系膜增生性肾小球肾炎是最常见的活检发现，新月体性肾小球肾炎见于27.7%的患者。在接受肺活检的患者中，72.7%可见毛细血管炎；其中37.5%有IgA沉积。44%的患者使用了类固醇联合环磷酰胺，随后用甲氨蝶呤或硫唑嘌呤维持治疗。机械通气、透析和血浆置换是其他辅助治疗方法。IgAN相关的肺泡出血死亡率为26.3%，且发病率较高，尽管进行了免疫抑制，仍有52.7%的患者发展为终末期肾病。伴有肺部IgA沉积的机化性肺炎是IgAN的一种常见关联表现。
这些发现与我们之前对过敏性紫癜（HSP）相关肺泡出血的观察结果相似，突出了这些相关综合征的相似性。需要采用标准方案对IgAN和HSP相关的肺肾综合征进行多中心研究，以明确它们的异同、最佳抑制方法及其在预防这种情况下肾脏疾病进展中的作用。
