原发性胆汁性胆管炎与巴塞杜氏病的关联及治疗难题:一名 36 岁赞比亚黑人女性患者的病例报告。

Sydney Mpisa, E. Sinkala, Wamundila Kawana, Brown Kamanga
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Examination revealed moist skin, scleral jaundice, generalised scratch marks, wasting with a body mass index (BMI) of 15kg/m2, a small goitre with a bruit heard over it and fine tremors. She did not have proptosis, finger clubbing or cervical lymphadenopathy or pretibial myxoedema. File review showed no prior goitre. Ultrasound of the neck confirmed the homogenous thyroid gland with increased vascularity. Blood tests revealed a suppressed thyroid stimulating hormone (TSH) (<0.005Uiu/ml) elevated free triiodothyronine (FT3) (10.9pmol/l) and elevated free thyroxine (FT4) (53.4pmol/l). TSH receptor antibodies were also elevated. The clinical features and blood tests led to the confirmation of Graves’ disease in a patient with PBC. 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引用次数: 0

摘要

文献记载原发性胆道胆管炎(PBC)患者发生其他自身免疫性疾病,甲状腺疾病就是这样一个例子。然而,这种情况在非洲,特别是在赞比亚很少见到。在此,我们报告一例36岁赞比亚黑人女性患者,她于2014年在赞比亚卢萨卡大学教学医院(UTH)被诊断为PBC。直到2020年11月,她才出现PBC发作。在发作期间,患者报告有2周的瘙痒、疲劳、尿色深、便色淡的病史,并伴有进行性体重减轻。检查发现皮肤湿润,巩膜黄疸,全身划痕,消瘦,体重指数(BMI) 15kg/m2,小甲状腺肿块,肿块上方有肿块,轻微震颤。她没有突出,手指棍棒或颈椎淋巴结病或胫前黏液水肿。档案回顾显示先前没有甲状腺肿。颈部超声证实均质甲状腺伴血管增多。血液检查显示促甲状腺激素(TSH)抑制(<0.005Uiu/ml),游离三碘甲状腺原氨酸(FT3)升高(10.9pmol/l),游离甲状腺素(FT4)升高(53.4pmol/l)。TSH受体抗体也升高。临床特征和血液检查证实了PBC患者的Graves病。我们的患者随后开始使用卡马唑、心得安和持续的熊去氧胆酸,对PBC和Graves病的治疗都有很好的临床反应。本例患者在Graves甲亢治疗开始后6个月内体重增加,BMI恢复正常,为22.6 Kg/m2。治疗一年后,她出现了粒细胞缺乏症,可能是由于卡马唑的作用,后来停用了卡马唑,导致她的甲状腺激素再次上升。考虑到肝脏疾病,患者不能服用丙硫尿嘧啶(PTU),因为放射性碘(不易获得)是唯一可行的选择。我们的病例报告为PBC倾向于与自身免疫性甲状腺疾病共存的知识体系做出了贡献,应该高度怀疑PBC,特别是在PBC治疗效果良好但体重减轻的PBC患者。我们还试图强调在低资源环境下管理和随访这些患者的挑战。虽然PBC在非洲黑人中并不常见,但在患有胆汁淤积性黄疸的患者中,尤其是年轻女性患者中,应怀疑PBC。
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The association of Primary biliary cholangitis and Graves’ disease coupled with management challenges: a case report of a 36-year-old black Zambian female patient.
The occurrence of other autoimmune conditions in primary biliary cholangitis (PBC) patients is documented in literature and thyroid disease is such an example. Such cases are however rarely seen in the African setting and particularly in Zambia. Here we report a case of a 36-year-old black Zambian female patient who was diagnosed with PBC in 2014 at the University Teaching Hospital(UTH), Lusaka, Zambia. She was lost to follow-up until November 2020 when she presented with a flare of PBC. During the flare the patient reported a 2-weeks history of pruritus, fatigue, passing dark urine and pale stools, coupled with progressive weight loss. Examination revealed moist skin, scleral jaundice, generalised scratch marks, wasting with a body mass index (BMI) of 15kg/m2, a small goitre with a bruit heard over it and fine tremors. She did not have proptosis, finger clubbing or cervical lymphadenopathy or pretibial myxoedema. File review showed no prior goitre. Ultrasound of the neck confirmed the homogenous thyroid gland with increased vascularity. Blood tests revealed a suppressed thyroid stimulating hormone (TSH) (<0.005Uiu/ml) elevated free triiodothyronine (FT3) (10.9pmol/l) and elevated free thyroxine (FT4) (53.4pmol/l). TSH receptor antibodies were also elevated. The clinical features and blood tests led to the confirmation of Graves’ disease in a patient with PBC. Our patient was subsequently started on carbimazole, propranolol and continued ursodeoxycholic acid with great clinical response to treatment of both the PBC and Graves’ disease. Our patient managed to gain weight within six months of commencement of Graves’ hyperthyroidism treatment, with her BMI improving to normal, at 22.6 Kg/m2. One year into her treatment she developed agranulocytosis likely from the carbimazole which was subsequently stopped resulting in her thyroid hormones going up again. The patient could not take propylthiouracil (PTU) in view of the liver disease as such radio-active iodine (which is not readily available) was the only feasible option. Our case report contributes to the body of knowledge that PBC tends to coexist with autoimmune thyroid disease and should be looked out for with a high index of suspicion especially in patients with PBC who report weight loss despite doing well on PBC therapy.  We also seek to highlight the challenges in management and follow up of such patients in low resource setting. Although PBC is not so common in Black Africans, it should be suspected in patients who present with cholestatic jaundice especially in young female patients.
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