男性 Mikulicz 病:全面回顾

Venkata Triveni Devi Yerra, Stephanie Margaret Puvvada, Vinod Kumar Mugada, Srinivasa Rao Yarguntla
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摘要

IgG4 相关疾病(IgG4-RD)是一种复杂的自身免疫性疾病,会对人体的多个器官产生影响,包括泪腺和唾液腺、肝脏、胆管、甲状腺、胰腺、胃肠道、肾脏、主动脉、前列腺和腹膜后。Mikulicz 病是 IgG4-RD 的一种亚型,特别针对泪腺和唾液腺,导致腺体肿胀、口干和眼干。Mikulicz病的诊断依据是至少有两对泪腺、颌下腺和腮腺出现比例性和持续性炎症,同时血清IgG4水平升高和/或IgG4阳性浆细胞明显渗入受影响的腺组织。糖皮质激素治疗对控制 IgG4 相关的 MD 有益,但联合治疗对预防复发的疗效仍是一个争论不休的话题。该病的病因可能各不相同,但淋巴上皮性唾液腺炎是最常见的病因。该病的典型病例平均年龄在 55 岁至 65 岁之间,20 岁以下的发病率极为罕见。Mikulicz 病是一种仍在研究中的神秘疾病。然而,确定其特征性症状并将其与其他类似疾病区分开来,对于有效诊断和治疗至关重要。为了更好地理解该病的根本原因,并为Mikulicz病和其他亚型IgG4-RD患者提供更有效的治疗方案,进一步的研究是必不可少的。
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Mikulicz Disease in Males: A Comprehensive Review
IgG4-related disease (IgG4-RD) is an intricate autoimmune disorder that have an effect on multiple organs in the body, including the lachrymal and salivary glands, liver, biliary duct, thyroid, pancreas, gastrointestinal tract, kidney, aorta, prostate and retroperitoneum. Mikulicz disease, a subtype of IgG4-RD, specifically targets the lachrymal and salivary glands, leading to glandular swelling, xerostomia, and xerophthalmia. Diagnosis of Mikulicz disease is established on the presence of proportional and persistent inflammation of at least two pairs of lachrymal, submandibular, and parotid glands, along with exalted serum IgG4 levels and/or marked penetration of IgG4-positive plasmacytes into the affected glandular tissues. Treatment with glucocorticoids has shown to be beneficial in managing IgG4-related MD, but the efficacy of combination therapy in preventing relapse is still a topic of debate. The underlying causes of the disease can vary, but lymphoepithelial sialadenitis is the widespread cause. The disease typically presents in individuals with an average age ranging from 55 to 65 years, and the incidence among individuals under the age of 20 is exceedingly rare. Mikulicz disease is an enigmatic ailment that is still being researched. However, identifying its hallmark symptoms and distinguishing it from other similar conditions is essential for effective diagnosis and treatment. Further research is essential to better comprehend the fundamental causes of the disease and to promote more effective treatment options for patients with Mikulicz disease and other subtypes of IgG4-RD.
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