PDF(Pubmed)
Abstract：
UNASSIGNED: IgG4-related disease is a rare and emerging pathology, characterized by the appearance of pseudotumors. Due to the ability to mimic other pathologies, it is essential to consider it as a differential diagnosis in multisystemic processes. The diagnosis is challenging, requiring a multidisciplinary approach, to minimize the associated morbidity and mortality.
UNASSIGNED: IgG4-related disease (IgG4-RD) is a rare, emerging, systemic and chronic pathology, characterized by the appearance of pseudotumors resulting from tissue infiltration by IgG4-positive plasma cells that promote eosinophilic inflammation of the tissue with subsequent fibrosis. We present the case of a male, 45-year-old patient, with marked weight loss and skin pallor detected by his family doctor during a child health consultation of his daughter. When questioned, the patient referred complaints of postprandial discomfort in the left hypochondrium with a feeling of fullness, weight loss, chronic fatigue and hyperhidrosis that had lasted for a month. On physical examination, he was pale, and had pain at palpation of the left hypochondrium. Laboratory data showed increased inflammation markers, abdominal ultrasound and CT demonstrated numerous enlarged lymph nodes in the upper quadrants, raising concern for a malignant lymphoproliferative process. Serological, imaging, clinical and laparoscopic excisional biopsy revealed features of IgG4-related disease and excluded malignant lymphoproliferative disease. The immediate response to treatment with oral prednisolone 30 mg/day also contributed for diagnosis confirmation. Due to refractory disease after gradual prednisolone reduction, second-line therapy with rituximab was initiated. Over the 6 years of follow-up, the patient presented multiple exacerbations characterized by the emergence of systemic symptoms, being maintained under close clinical and imaging follow-up by reumathology, infectious diseases, and family medicine specialists.
UNASSIGNED: IgG4-related disease (IgG4-RD) is a rare, emerging, systemic and chronic pathology, characterized by the appearance of pseudotumors resulting from tissue infiltration by IgG4-positive plasma cells that promote eosinophilic inflammation of the tissue with subsequent fibrosis. We present the case of a male, 45-year-old patient, with marked weight loss and skin pallor detected by his family doctor during a child health consultation of his daughter. When questioned, the patient referred complaints of postprandial discomfort in the left hypochondrium with a feeling of fullness, weight loss, chronic fatigue and hyperhidrosis that had lasted for a month. On physical examination, he was pale, and had pain at palpation of the left hypochondrium. Laboratory data showed increased inflammation markers, abdominal ultrasound and CT demonstrated numerous enlarged lymph nodes in the upper quadrants, raising concern for a malignant lymphoproliferative process. Serological, imaging, clinical and laparoscopic excisional biopsy revealed features of IgG4-related disease and excluded malignant lymphoproliferative disease. The immediate response to treatment with oral prednisolone 30 mg/day also contributed for diagnosis confirmation. Due to refractory disease after gradual prednisolone reduction, second-line therapy with rituximab was initiated. Over the 6 years of follow-up, the patient presented multiple exacerbations characterized by the emergence of systemic symptoms, being maintained under close clinical and imaging follow-up by reumathology, infectious diseases, and family medicine specialists.
摘要：
■IgG4相关疾病是一种罕见且新兴的病理，以伪肿瘤的出现为特征。由于模仿其他病理的能力，将其视为多系统过程的鉴别诊断至关重要。诊断很有挑战性,需要多学科的方法，尽量减少相关的发病率和死亡率。
■IgG4相关疾病（IgG4-RD）是一种罕见的疾病，新兴,系统和慢性病理学，其特征是由于IgG4阳性浆细胞的组织浸润而出现假瘤，从而促进组织的嗜酸性粒细胞炎症并随后纤维化。我们介绍一个男性的案例，45岁的病人,他的家庭医生在女儿的儿童健康咨询中发现了明显的体重减轻和皮肤苍白。当被质疑时，患者以饱胀的感觉转述了左软骨下餐后不适的抱怨，减肥,持续一个月的慢性疲劳和多汗症。在体检时，他脸色苍白，触诊左侧软骨下有疼痛。实验室数据显示炎症标志物增加，腹部超声和CT显示上象限有许多肿大的淋巴结，引起对恶性淋巴增生过程的关注。血清学,成像,临床和腹腔镜切除活检显示IgG4相关疾病的特征,并排除恶性淋巴增生性疾病.对口服泼尼松龙30mg/天治疗的即时反应也有助于诊断确认。由于难治性疾病后逐渐减少泼尼松龙，利妥昔单抗的二线治疗开始.在6年的随访中，患者出现了以出现全身症状为特征的多次加重，通过病理学保持密切的临床和影像学随访，传染病,和家庭医学专家。
■IgG4相关疾病（IgG4-RD）是一种罕见的疾病，新兴,系统和慢性病理学，其特征是由于IgG4阳性浆细胞的组织浸润而出现假瘤，从而促进组织的嗜酸性粒细胞炎症并随后纤维化。我们介绍一个男性的案例，45岁的病人,他的家庭医生在女儿的儿童健康咨询中发现了明显的体重减轻和皮肤苍白。当被质疑时，患者以饱胀的感觉转述了左软骨下餐后不适的抱怨，减肥,持续一个月的慢性疲劳和多汗症。在体检时，他脸色苍白，触诊左侧软骨下有疼痛。实验室数据显示炎症标志物增加，腹部超声和CT显示上象限有许多肿大的淋巴结，引起对恶性淋巴增生过程的关注。血清学,成像,临床和腹腔镜切除活检显示IgG4相关疾病的特征,并排除恶性淋巴增生性疾病.对口服泼尼松龙30mg/天治疗的即时反应也有助于诊断确认。由于难治性疾病后逐渐减少泼尼松龙，利妥昔单抗的二线治疗开始.在6年的随访中，患者出现了以出现全身症状为特征的多次加重，通过病理学保持密切的临床和影像学随访，传染病,和家庭医学专家。
