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一名年轻男性系统性红斑狼疮的罕见表现:慢性原发性肾上腺功能不全、亚临床甲状腺功能减退和双血细胞减少症的组合:病例报告

Rare combination of chronic primary adrenal insufficiency, subclinical hypothyroidism, and bicytopenia as features of systemic lupus erythematosus in a young man: A case report.

作者信息

Mulya Deshinta Putri, Leo Benedreky, Ikhsan Mohammad Robikhul, Hutajulu Susanna Hilda

机构信息

Division of Allergy and Immunology, Department of Internal Medicine, Faculty of Medicine, Public Health, and Nursing, Gadjah Mada University/Dr Sardjito Hospital, Yogyakarta, Indonesia.

Department of Internal Medicine, Faculty of Medicine, Public Health, and Nursing, Gadjah Mada University/Dr Sardjito Hospital, Yogyakarta, Indonesia.

出版信息

SAGE Open Med Case Rep. 2023 Aug 1;11:2050313X231190006. doi: 10.1177/2050313X231190006. eCollection 2023.

DOI:10.1177/2050313X231190006
PMID:37539357
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10395155/
Abstract

Adrenal insufficiency in systemic lupus erythematosus is rarely detected, especially in male patients. Nevertheless, such coexistence can occur, and screening for systemic lupus erythematosus should be considered in primary adrenal insufficiency with symptoms of systemic multiorgan involvement. We report a 22-year-old Asian man, initially diagnosed with bicytopenia, developed severe unintentional weight loss, skin and mucosal hyperpigmentation, along with persistent fatigue. Laboratory examination showed positive antinuclear antibody-indirect immunofluorescence, elevated anti-double-stranded DNA, extremely low morning serum cortisol, and mildly elevated thyroid stimulating hormone with normal free T4. He was diagnosed with systemic lupus erythematosus, manifesting as chronic primary adrenal insufficiency, subclinical hypothyroidism, and bicytopenia. He was treated with mycophenolic acid of 180 mg b.i.d, methylprednisolone of 4 mg q.d, and vitamin D3 1000 IU q.d. Methylprednisolone was given for its anti-inflammatory property and as a simple once-daily regimen to supplement glucocorticoid deficiency. Levothyroxine was not prescribed for our patient since his thyroid stimulating hormone was only mildly elevated, and supplementation of levothyroxine in the setting of adrenal insufficiency might precipitate an adrenal crisis. At the 6-month follow-up, he was no longer fatigued, he regained his body weight, his skin and mucosal hyperpigmentation improved significantly, his thyroid stimulating hormone level normalized (without levothyroxine supplementation), and his complete blood count stabilized, remitting him from the need for transfusion.

摘要

系统性红斑狼疮合并肾上腺功能不全很少被发现,尤其是在男性患者中。然而,这种共存情况可能会发生,对于出现系统性多器官受累症状的原发性肾上腺功能不全患者,应考虑筛查系统性红斑狼疮。我们报告一名22岁的亚洲男性,最初被诊断为双血细胞减少症,随后出现严重的非故意体重减轻、皮肤和黏膜色素沉着,以及持续疲劳。实验室检查显示抗核抗体间接免疫荧光阳性、抗双链DNA升高、清晨血清皮质醇极低、促甲状腺激素轻度升高而游离T4正常。他被诊断为系统性红斑狼疮,表现为慢性原发性肾上腺功能不全、亚临床甲状腺功能减退和双血细胞减少症。给予他霉酚酸180毫克每日两次、甲泼尼龙4毫克每日一次和维生素D3 1000国际单位每日一次进行治疗。给予甲泼尼龙是因其抗炎特性以及作为一种简单的每日一次方案来补充糖皮质激素缺乏。由于我们的患者促甲状腺激素仅轻度升高,且在肾上腺功能不全的情况下补充左甲状腺素可能会引发肾上腺危象,因此未给他开左甲状腺素。在6个月的随访中,他不再感到疲劳,体重恢复,皮肤和黏膜色素沉着明显改善,促甲状腺激素水平恢复正常(未补充左甲状腺素),全血细胞计数稳定,无需输血。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c15/10395155/f9e61e65288d/10.1177_2050313X231190006-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c15/10395155/9c598c6024ff/10.1177_2050313X231190006-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c15/10395155/083f6c8a82a9/10.1177_2050313X231190006-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c15/10395155/f9e61e65288d/10.1177_2050313X231190006-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c15/10395155/9c598c6024ff/10.1177_2050313X231190006-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c15/10395155/083f6c8a82a9/10.1177_2050313X231190006-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c15/10395155/f9e61e65288d/10.1177_2050313X231190006-fig3.jpg

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本文引用的文献

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Adrenal insufficiency.肾上腺功能不全。
Lancet. 2021 Feb 13;397(10274):613-629. doi: 10.1016/S0140-6736(21)00136-7. Epub 2021 Jan 20.
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Delayed diagnosis adversely affects outcome in systemic lupus erythematosus: Cross sectional analysis of the LuLa cohort.延迟诊断对系统性红斑狼疮的预后有不良影响:LuLa 队列的横断面分析。
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