PDF(Pubmed)
Abstract：
Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory condition characterized by significant infiltration of immunoglobulin G4 (IgG4)-positive plasma cells within affected tissues, with or without elevated serum IgG4 levels. The prevalence of IgG4-RD remains largely undetermined due to diagnostic challenges, as the condition is frequently unrecognized or misdiagnosed. This report describes a case of a 63-year-old man who was ultimately diagnosed with this rare condition after an extensive two-year period of elusive symptoms. Initially presenting with intermittent body pains and fluctuating fever, his condition progressively evolved to include severe right orbital swelling with marked tenderness and ecchymosis, recurrent non-tender nodules on his arm, and diminished vision. A detailed review of his medical history prompted the consideration of IgG4-RD, leading to the measurement of serum human IgG4 levels, which were found to be significantly elevated at 1504 mg/L (normal range: 39.2-864 mg/L). Following his diagnosis, treatment with glucocorticoids (0.6 mg/kg for one month) was initiated, resulting in a positive clinical response. This case emphasizes the critical importance of considering less common conditions in the differential diagnosis of patients presenting with complex, multi-system symptoms.
摘要：
免疫球蛋白G4相关疾病（IgG4-RD）是一种全身性纤维炎性疾病，其特征是受影响组织内免疫球蛋白G4（IgG4）阳性浆细胞的明显浸润，有或没有升高的血清IgG4水平。由于诊断挑战，IgG4-RD的患病率仍未确定。因为这种情况经常无法识别或误诊。本报告描述了一名63岁男子的病例，该男子在经历了长达两年的难以捉摸的症状后最终被诊断出患有这种罕见疾病。最初表现为间歇性身体疼痛和波动性发热，他的病情逐渐演变为包括严重的右眶肿胀，伴有明显的压痛和瘀斑，他手臂上反复出现的非触痛结节,视力下降。对他的病史的详细回顾促使人们考虑IgG4-RD，导致血清人IgG4水平的测量，在1504mg/L（正常范围：39.2-864mg/L）时发现显着升高。根据他的诊断,开始糖皮质激素治疗（0.6mg/kg，持续一个月），导致积极的临床反应。该案例强调了在对表现为复杂，多系统症状。
