Departments of Family Medicine, Radiology Western Pennsylvania Hospital Forbes Regional Campus, Monroeville, PA 15146, USA.
J Clin Rheumatol. 2009 Oct;15(7):354-7. doi: 10.1097/RHU.0b013e3181b5d631.
Great attention has been drawn toward the recently defined IgG4 related sclerosing disease, an entity incorporating autoimmune pancreatitis and other organ involvements, hypergammaglobulinemia and deposition of IgG4 in affected tissues. We demonstrate an index case of this disease with multiple organ involvements and an excellent response to corticosteroid treatment. A 69-year-old white man was diagnosed with IgG4 related sclerosing disease with involvement of lungs, pancreas, submandibular glands, lymph nodes, and kidney. In addition, the patient had polyclonal IgG hypergammaglobulinemia and hypocomplementemia. A renal biopsy showed tubulointerstitial nephritis with extensive lymphoplasmacytic infiltrate. Biopsy of the lung showed "inflammatory pseudotumor" with lymphocyte and plasma cell infiltration. IgG4 positive plasma cells were seen in the biopsy of submandibular gland and the kidney. Immuno-staining of the renal biopsy specimen showed vascular deposition of complement split product C4d. Corticosteroid treatment of 2 months showed complete resolution of the disease process. We demonstrate deposition of C4d in the IgG4 related tubulointerstitial nephritis for the first time.
人们对最近定义的 IgG4 相关硬化性疾病给予了极大关注，该疾病包含自身免疫性胰腺炎和其他器官受累、高丙种球蛋白血症以及受累组织中 IgG4 的沉积。我们展示了一例具有多器官受累和对皮质类固醇治疗反应良好的该病指数病例。一名 69 岁白人男性被诊断为 IgG4 相关硬化性疾病，涉及肺、胰腺、颌下腺、淋巴结和肾脏。此外，患者存在多克隆 IgG 高丙种球蛋白血症和低补体血症。肾活检显示伴有广泛淋巴浆细胞浸润的肾小管间质性肾炎。肺活检显示“炎性假瘤”，伴有淋巴细胞和浆细胞浸润。颌下腺和肾脏活检中可见 IgG4 阳性浆细胞。肾活检标本的免疫染色显示血管补体分裂产物 C4d 的沉积。2 个月的皮质类固醇治疗使疾病过程完全缓解。我们首次证明 IgG4 相关肾小管间质性肾炎中 C4d 的沉积。
