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Objective To report a case of refractory Takayasu arteritis (TAK) treated with tofacitinib (TOF) and evaluated by contrast-enhanced ultrasound, and perform a literature review to better understand this disorder. Methods The data of a case of TAK treated with TOF were analyzed retrospectively, the treatment and follow-up experience summarized by searching the database(CNKI, Wanfang Data, PubMed) and the literature results analyzed comprehensively. Results The patient, a 28-year-old female with a 10-year history of TAK, presented with fever, neck pain and joints pain. Ultrasonography revealed intimal thickening and stenosis of the carotid artery. Laboratory tests demonstrated an elevated erythrocyte sedimentation rate (ESR) and a C-reactive protein (CRP) level. The symptoms were improved after glucocorticoid (GC) treatment. However, it relapsed during the course of GC tapering, even with the combination of disease modifying anti-rheumatic drugs (DMARDs) and tocilizumab. The DMARDs and tocilizumab were not used continuously due to poor response and adverse drug reactions. As disease progressing, left subclavian aneurysm appeared. The treatment of this patient is a big challenge. After adjusting the treatment with tofacitinib (10 mg/d, then adjust to 7.5 mg/d) and GC (methylprednisolone 14 mg/d, then reduce to 8 mg/d), the patient was successfully relieved without any serious adverse events. Contrast-enhanced ultrasound showed that the thickness of arterial wall decreased (the thickness of left common carotid artery decreased from 8.9 mm to 1.2 mm, and the thickness of left subclavian artery decreased from 7.4 mm to 0.8 mm), the contrast intensity decreased (the total score decreased from 5 to 1), and the aneurysm disappeared. By March 2021 (searching CNKI, Wanfang Data and PubMed), a total of 10 cases of TAK patients treated with TOF. Nine patients were women. Seven patients improved and GC reduced. Only one patient had adverse reactions. Conclusions Janus kinase inhibitor is expected to be a new drug for the treatment of TAK. Contrast-enhanced ultrasound can detect the thickness of the vessel wall and the enhancement of the thickened wall in real time. Contrast-enhanced ultrasound will be an effective tool for evaluating vascular inflammation.
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| 科 Family | 属数 Number of genus | 种数 Number of species | 占总种数比例 Percentage of total species (%) | 属 Genus | 种数 Number of species | 占总种数比例 Percentage of total species (%) |
|---|---|---|---|---|---|---|
| 鹅膏菌科Amanitaceae | 2 | 11 | 5.26 | 鹅膏菌属 Amanita | 10 | 4.78 |
| 小菇科 Mycenaceae | 2 | 12 | 5.74 | 丝盖伞属 Inocybe | 5 | 2.39 |
| 多孔菌科 Polyporaceae | 8 | 14 | 6.70 | 蜡蘑属 Laccaria | 5 | 2.39 |
| 红菇科 Russulaceae | 3 | 23 | 11.00 | 小皮伞属 Marasmius | 6 | 2.87 |
| 小菇属 Mycena | 11 | 5.26 | ||||
| 光柄菇属 Pluteus | 5 | 2.39 | ||||
| 红菇属 Russula | 17 | 8.13 | ||||
| 栓菌属 Trametes | 5 | 2.39 |
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