IgG4-related nephritis and interstitial pulmonary disease complicated by invasive pulmonary fungal infection: a case report

• Main Authors: Yili Xu, Guang Yang, Xueqiang Xu, Yaoyu Huang, Kang Liu, Tongfu Yu, Jun Qian, Xiufen Zhao, Jingfeng Zhu, Ningning Wang, Changying Xing
• Format: Article
• Language: English
• Published: BMC 2021-01-01
• Series: BMC Nephrology
• Subjects: IgG4-Related nephritis; IgG4-Related lung disease; Corticosteroid; Invasive pulmonary fungal infection; Case report
• Online Access: https://doi.org/10.1186/s12882-020-02223-8

Abstract Background 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. Case presentation 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. Conclusions 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.