Type Ⅰ renal tubular acidosis caused by primary Sjögren syndrome with first diagnosis of hypokalemia: a case report

doi:10.16150/j.1671-2870.2024.06.010

Abstract

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

CLC Number:

R593.2
R445

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.

Figures/Tables 4

Table 1

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References 18

[1]	Mariette X, Criswell LA. Primary Sjögren’s Syndrome[J]. N Engl J Med, 2018, 378(10):931-939.
[2]	中华医学会风湿病学分会. 干燥综合征诊断及治疗指南[J]. 中华风湿病学杂志, 2010, 14(11):766-768.
	Chinese rheumatology association. Guidelines on the diag-nosis and management of Sjögren’s syndrome[J]. Chin J Rheumatol, 2010, 14(11):766-768.
[3]	张文, 厉小梅, 徐东, 等. 原发性干燥综合征诊疗规范[J]. 中华内科杂志, 2020, 59(4):269-276.
	Zhang W, Li XM, Xu D, et al. Recommendations of diagnosis and treatment of primary Sjögren’s syndrome in China[J]. Chin J Intern Med, 2020, 59(4):269-276. doi: 10.3760/cma.j.cn112138-20200113-00021 pmid: 32209192
[4]	姚血明, 侯雷, 曾苹, 等. 干燥综合征误诊原因分析[J]. 风湿病与关节炎, 2020, 9(1):28-30.
	Yao XM, Hou L, Zeng P, et al. Misdiagnosed causes analysis of Sjögren’s syndrome[J]. Rheum Arthritis, 2020, 9(1):28-30.
[5]	韩瑞红, 赵克中. 干燥综合征引发严重低钾血症致心脏骤停一例[J]. 天津医药, 2013, 41(2):180-181.
	Han RH, Zhao KZ. Cardiac arrest due to severe hypokalemia caused by Sjögren’s syndrome: a case report[J]. Tianjin Med J, 2013, 41(2):180-181.
[6]	Varlamov EV, Langlois F, Vila G, et al. Management of endocrine disease: cardiovascular risk assessment, thromboembolism, and infection prevention in Cushing’s syndrome: a practical approach[J]. Eur J Endocrinol, 2021, 184(5):R207-R224. doi: 10.1530/EJE-20-1309 pmid: 33539319
[7]	Torregrosa Prats JV, Santos Rodríguez F, González Parra E, et al. Distar renal tubular acidosis (dRTA): epidemiological, diagnostics, clinical follow-up and therapeutical issues. Nephrologists cohort survey outcome[J]. Nefrologia (Engl Ed), 2021, 41(1):62-68.
[8]	Goules A, Geetha D, Arend LJ, et al. Renal involvement in primary Sjögren’s syndrome: natural history and treatment outcome[J]. Clin Exp Rheumatol, 2019, 37 Suppl 118(3):123-132.
[9]	Furqan S, Banu S, Ram N. Osteoporosis complicating renal tubular acidosis in association with Sjögren’s Syndrome[J]. Cureus. 2021 Sep 29;13(9):e18373.
[10]	Palmer BF, Kelepouris E, Clegg DJ. Renal tubular acidosis and management strategies: a narrative review[J]. Adv Ther, 2021, 38(2):949-968. doi: 10.1007/s12325-020-01587-5 pmid: 33367987
[11]	Zhang L, Xu B, Niu Y, et al. Familial distal renal tubular acidosis[J]. J Int Med Res, 2021, 49(3):3000605211000 533.
[12]	Giglio S, Montini G, Trepiccione F, et al. Distal renal tubular acidosis: a systematic approach from diagnosis to treatment[J]. J Nephrol, 2021, 34(6):2073-2083. doi: 10.1007/s40620-021-01032-y pmid: 33770395
[13]	Liu Y, Hirayama M, Cui X, et al. Effectiveness of autologous serum eye drops combined with punctal plugs for the treatment of Sjögren syndrome-related dry eye[J]. Cornea, 2015, 34(10):1214-1220. doi: 10.1097/ICO.0000000000000542 pmid: 26226470
[14]	Ahn H, Ji YW, Jun I, et al. Comparison of treatment modalities for dry eye in primary Sjögren’s syndrome[J]. J Clin Med, 2022, 11(2):463.
[15]	Ogawa Y, Takeuchi T, Tsubota K. Autoimmune epithelitis and chronic inflammation in Sjögren’s syndrome-related dry eye disease[J]. Int J Mol Sci, 2021, 22(21):11820.
[16]	Negrini S, Emmi G, Greco M, et al. Sjögren’s syndrome: a systemic autoimmune disease[J]. Clin Exp Med, 2022, 22(1):9-25.
[17]	刘霞, 徐婷, 吴敏. 外泌体在自身免疫性疾病中的研究进展[J]. 中国临床研究, 2023, 36(2):238-241.
	LIU X, XU T, WU M. Research progress of extracellular vesicles in autoimmune diseases[J]. Chin Clin Res, 2023, 36(2):238-241.
[18]	孟德钎, 李鞠, 李永胜, 等. 艾拉莫德联合甲泼尼龙、硫酸羟氯喹对原发性干燥综合征合并间质性肺病的疗效[J]. 中国临床研究, 2023, 36(1):85-89.
	MENG D C, LI J, LI Y S, et al. The efficacy of alamod combined with methylprednisolone and hydroxychloroquine sulfate in the treatment of primary Sjogren's syndrome complicated with interstitial lung disease[J]. Chin Clinl Res, 2023, 36(1):85-89.

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