Department of Nephrology, the First Affiliated Hospital with Nanjing Medical University, Jiangsu Province Hospital, Nanjing, China.
Department of Imaging, the First Affiliated Hospital with Nanjing Medical University, Jiangsu Province Hospital, Nanjing, China.
BMC Nephrol. 2021 Jan 11;22(1):22. doi: 10.1186/s12882-020-02223-8.
IgG4-related kidney disease (IgG4-RKD) can affect multiple organs, which was first reported as a complication or extra-organ manifestation of autoimmune pancreatitis in 2004. It is characterized by abundant IgG4-positive plasma cells infiltration in tissues involved.
A 69-year-old man presented with cough and renal dysfunction with medical history of hypertension and diabetes. Pathological findings revealed interstitial nephritis and he was initially diagnosed with IgG4-RKD. Prednisone helped the patient to get a remission of cough and an obvious decrease of IgG4 level. However, he developed invasive pulmonary fungal infection while steroid theatment. Anti-fungal therapy was initiated after lung puncture (around cavitary lung lesion). Hemodialysis had been conducted because of renal failure and he got rid of it 2 months later. Methylprednisolone was decreased to 8 mg/day for maintenance therapy. Anti-fungal infection continued for 4 months after discharge home. On the 4th month of follow-up, Chest CT revealed no progression of lung lesions.
The corticosteroids are the first-line therapy of IgG4-RD and a rapid response helps to confirm the diagnosis. This case should inspire clinicians to identify IgG4-related lung disease and secondary pulmonary infection, pay attention to the complications during immunosuppressive therapy for primary disease control.
IgG4 相关肾脏疾病（IgG4-RKD）可影响多个器官，于 2004 年首次报道为自身免疫性胰腺炎的并发症或额外器官表现。其特征是受累组织中存在丰富的 IgG4 阳性浆细胞浸润。
一名 69 岁男性，因咳嗽和肾功能不全就诊，有高血压和糖尿病病史。病理检查显示间质性肾炎，最初诊断为 IgG4-RKD。泼尼松有助于患者咳嗽缓解和 IgG4 水平明显下降。然而，在激素治疗期间，他发生了侵袭性肺部真菌感染。在肺部穿刺（空洞性肺病变周围）后开始抗真菌治疗。由于肾衰竭，患者进行了血液透析，2 个月后摆脱了透析。为维持治疗，将甲泼尼龙减少至 8mg/天。出院后继续抗真菌治疗 4 个月。在随访的第 4 个月，胸部 CT 显示肺部病变无进展。
皮质类固醇是 IgG4-RD 的一线治疗药物，快速反应有助于确诊。本病例应启发临床医生识别 IgG4 相关肺部疾病和继发性肺部感染，注意在治疗原发性疾病以控制疾病时发生的并发症。
