以低钾血症首发症状的原发性干燥综合征致I型肾小管酸中毒1例报道

doi:10.16150/j.1671-2870.2024.06.010

摘要/Abstract

摘要：

原发性干燥综合征（primary Sjögren syndrome, PSS）作为一种慢性系统性自身免疫性疾病，早期发病往往较为隐匿，患者多以其他系统疾病的临床表现首次就诊，针对其发病机制现暂无明确研究结论。该病除侵犯机体外分泌腺体外，肾脏也常是其受累器官，部分患者可发展为肾小管酸中毒（renal tubular acidosis, RTA），出现难治性低钾血症及相关临床表现，而目前临床尚缺乏有针对性的规范治疗手段。早期诊断该病可以及时干预患者的病情进展，避免或延缓相关系统受累，使患者的临床治疗达最大获益。现报道1例因反复肢体乏力1年入院，最终被诊断为PSS继发Ⅰ型RTA的病例，根据患者自身情况予硫酸羟氯喹调节免疫治疗及对症处理，最终患者的病情好转。

关键词: 原发性干燥综合征, 肾小管性酸中毒, 低钾血症, 诊断

Abstract:

Primary Sjögren syndrome (PSS) as a chronic systemic autoimmune disease, many patients with early hidden symptoms of presented the clinical manifestations of other systemic diseases for the first time, and its pathogenesis was not clear yet. PSS could invade the exocrine glands of body, and the kidney was also the susceptible organ in PSS. Some patients could develop to renal tubular acidosis, and lead to refractory hypokalemia and related clinical manifestations. As lack of effective treatment, the early diagnosis and intervention of the disease could avoid damage or delay the involvement of related organs and maximize the benefits of clinical treatment. This paper reported a patient, who was admitted to hospital with chief complain of repeated limb weakness for one year, was diagnosed as type I renal tubular acidosis secondary to primary Sjögren syndrome. The patients received symptomatic treatment and hydroxychloroquine sulfate immunotherapy, and the symptoms were resolved eventually.

Key words: Primary Sjögren syndrome, Renal tubular acidosis, Hypokalemia, Diagnosis

中图分类号:

R593.2
R445

黄敏, 左莹. 以低钾血症首发症状的原发性干燥综合征致I型肾小管酸中毒1例报道[J]. 诊断学理论与实践, 2024, 23(06): 624-627.

HUANG Min, ZUO Ying. Type Ⅰ renal tubular acidosis caused by primary Sjögren syndrome with first diagnosis of hypokalemia: a case report[J]. Journal of Diagnostics Concepts & Practice, 2024, 23(06): 624-627.

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体位	醛固酮(ng/dL)	肾素(μIU/mL)	醛固酮/肾素
卧位	10.70	10.12	1.06
立位	15.60	39.29	0.40

时间	皮质醇（μg/dL）	促肾上腺皮质激素（pg/mL）
08:00	9.53	8.97
16:00	8.95	8.54
00:00	1.09	3.58

时间	体重（kg）	血压（mmHg）	心率（次/分）	尿量（mL）	尿比重
09:00	55	105/68	69	180	1.004
10:00	55	105/66	66	200	1.005
11:00	55	112/79	72	150	1.005
12:00	55	104/68	76	100	1.006
13:00	55	105/68	69	100	1.006
14:00	54.5	103/68	70	100	1.006
15:00	54.5	102/70	68	110	1.006
16:30	54.2	121/85	57	120	1.007
17:30	54.2	108/75	69	100	1.006