我们报告了一例IgG4相关疾病(IgG4-RD)伴明显的嗜酸性粒细胞增多。一名79岁的妇女因腹泻和体重减轻而入院。颈淋巴结病,双侧颌下腺肿胀,贫血(Hb8.5g/dl),嗜酸性粒细胞增多症(9750/μl),血清肌酐升高(1.57mg/dl),胰淀粉酶(191IU/l),发现IgG4(3380mg/dl)。磁共振成像的扩散加权图像显示胰腺和肾脏内部都有高强度信号。颌下腺的超声图显示鹅卵石图案。肾活检提示急性肾小管间质性肾炎。嘴唇活检,胃肠道,和骨髓显示淋巴浆细胞和IgG4阳性浆细胞(30-67/HPF)浸润。胃肠道和骨髓活检也显示嗜酸性粒细胞浸润。肾上腺功能不全,风湿性疾病,结核病,寄生虫感染,药物诱导的嗜酸性粒细胞增多,和嗜酸性粒细胞白血病均被排除。我们开始用40mg泼尼松龙(PSL)治疗,她的一般状况迅速改善。嗜酸性粒细胞计数,血清IgG4和血清肌酐下降。我们逐渐减少PSL并维持5mg/天。在5年的治疗中,她没有复发症状。根据2019年美国风湿病学会/欧洲抗风湿病联盟对IgG4-RD的分类标准,嗜酸性粒细胞>3000/μl是排除标准之一。如果我们遵守这个标准,应避免IgG4-RD的诊断.然而,我们的病例符合I型自身免疫性胰腺炎的诊断标准,IgG4相关性唾液腺炎,和IgG4-RD的全球诊断。我们最终诊断为IgG4-RD伴继发性嗜酸性粒细胞增多综合征。这种情况表明,嗜酸性粒细胞>3000/μl的IgG4-RD确实存在于现实世界中。
We report a
case of
IgG4-related disease (
IgG4-RD) with marked eosinophilia. A 79-year-old woman was admitted due to diarrhoea and weight loss. Cervical lymphadenopathy, bilateral submandibular glands swelling, anaemia (Hb8.5 g/dl), hypereosinophilia (9750/μl), elevated serum creatinine (1.57 mg/dl), pancreatic amylase (191 IU/l), and IgG4 (3380 mg/dl) were found. Diffusion-weighted image on magnetic resonance imaging showed high-intensity signals inside both the pancreas and the kidneys. The echogram of submandibular glands revealed cobblestone pattern. Kidney biopsy revealed acute tubulointerstitial nephritis. Biopsies of lip, gastrointestinal tract, and bone marrow showed infiltration of lymphoplasmacytic cells and IgG4-positive plasma cells (30-67/HPF). Gastrointestinal and bone marrow biopsies also showed eosinophilic infiltration. Adrenal insufficiency, rheumatic disease, tuberculosis, parasite infection, drug-induced eosinophilia, and eosinophilic leukaemia were all ruled out. We started treatment with 40 mg of prednisolone (PSL) and her general condition rapidly improved. The eosinophil count, serum IgG4, and serum creatinine decreased. We gradually tapered PSL and maintained 5 mg/day. During the 5 years of treatment, she had no recurrence of the symptom. According to the 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-RD, eosinophils >3000/μl is one of the exclusion criteria. If we comply with this criterion, the diagnosis of IgG4-RD should be avoided. However, our
case fit the diagnostic criteria of type I autoimmune pancreatitis,
IgG4-related sialadenitis, and global diagnosis of
IgG4-RD. We finally diagnosed our
case as
IgG4-RD with secondary hypereosinophilic syndrome. This
case suggests that
IgG4-RD with eosinophils >3000/μl does exist in the real world.