Yili Xu1, Guang Yang1, Xueqiang Xu1, Yaoyu Huang1, Kang Liu1, Tongfu Yu2, Jun Qian1, Xiufen Zhao1, Jingfeng Zhu1, Ningning Wang3, Changying Xing1. 1. Department of Nephrology, the First Affiliated Hospital with Nanjing Medical University, Jiangsu Province Hospital, Nanjing, China. 2. Department of Imaging, the First Affiliated Hospital with Nanjing Medical University, Jiangsu Province Hospital, Nanjing, China. 3. Department of Nephrology, the First Affiliated Hospital with Nanjing Medical University, Jiangsu Province Hospital, Nanjing, China. wangnn@njmu.edu.cn.
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.
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.
Authors: J C Jennette; R J Falk; P A Bacon; N Basu; M C Cid; F Ferrario; L F Flores-Suarez; W L Gross; L Guillevin; E C Hagen; G S Hoffman; D R Jayne; C G M Kallenberg; P Lamprecht; C A Langford; R A Luqmani; A D Mahr; E L Matteson; P A Merkel; S Ozen; C D Pusey; N Rasmussen; A J Rees; D G I Scott; U Specks; J H Stone; K Takahashi; R A Watts Journal: Arthritis Rheum Date: 2013-01