费尔蒂综合征：一例报告。
Felty syndrome: a case report.
机构信息
Touro College of Osteopathic Medicine, 60 Prospect Avenue, Middletown, NY, 10940, USA.
Orange Regional Medical Center, Middletown, NY, USA.
出版信息
J Med Case Rep. 2021 May 27;15(1):273. doi: 10.1186/s13256-021-02802-9.
BACKGROUND
Felty syndrome is a rare manifestation of chronic rheumatoid arthritis in which patients develop extraarticular features of hepatosplenomegaly and neutropenia. The typical presentation of Felty syndrome is in Caucasians, females, and patients with long-standing rheumatoid arthritis of 10 or more years. This case report presents a patient with an early-onset and atypical demographic for Felty syndrome.
CASE PRESENTATION
Our patient is a 28-year-old African American woman with past medical history of rheumatoid arthritis diagnosed in 2017, asthma, pneumonia, anemia, and mild intellectual disability who was admitted to inpatient care with fever, chills, and right ear pain for 7 days. The patient's mother, also her caregiver, brought the patient to the hospital after symptoms of fever and ear pain failed to improve. Our patient was diagnosed with sepsis secondary to pneumonia and urinary tract infection. She had been admitted twice in the past year, both times with a diagnosis of pneumonia. During this visit in September 2019, it was discovered that the patient had leukopenia and neutropenia. Bone marrow biopsy revealed increased immature mononuclear cells with left shift and rare mature neutrophils. During the hospital course, the patient was provisionally diagnosed with Felty syndrome and treated with adalimumab and hydroxychloroquine for her rheumatoid arthritis. Her sepsis secondary to pneumonia and urinary tract infection was treated with ceftriaxone and doxycycline, which was later switched to cefepime because of positive blood and urine cultures for Pseudomonas aeruginosa. She was discharged with stable vital signs and is continuing to control her rheumatoid arthritis with adalimumab.
CONCLUSION
This case report details the clinical course of sepsis secondary to pneumonia and urinary tract infection in the setting of Felty syndrome. Our patient does not fit the conventional profile for presentation given her race, age, and the length of time following diagnosis of rheumatoid arthritis.
背景
费尔蒂综合征是慢性类风湿关节炎的一种罕见表现，患者会出现肝脾肿大和中性粒细胞减少等关节外表现。费尔蒂综合征的典型表现为白种人、女性和患类风湿关节炎 10 年或以上的患者。本病例报告介绍了一例发病早且不典型的费尔蒂综合征患者。
病例介绍
我们的患者是一位 28 岁的非裔美国女性，既往有类风湿关节炎病史，于 2017 年确诊，还有哮喘、肺炎、贫血和轻度智力障碍。她因发热、寒战和右耳痛 7 天而住院。患者的母亲，也是她的照顾者，在患者的发热和耳部疼痛症状未改善后将她带到医院。我们的患者被诊断为肺炎和尿路感染引起的脓毒症。她在过去一年中曾两次住院，均被诊断为肺炎。在 2019 年 9 月的这次就诊中，发现患者出现白细胞减少和中性粒细胞减少。骨髓活检显示幼稚单核细胞增多，左移，罕见成熟中性粒细胞。在住院期间，患者被临时诊断为费尔蒂综合征，并接受阿达木单抗和羟氯喹治疗其类风湿关节炎。她因肺炎和尿路感染引起的脓毒症接受头孢曲松和多西环素治疗，后来因血和尿培养出铜绿假单胞菌而改用头孢吡肟。她出院时生命体征稳定，继续使用阿达木单抗控制类风湿关节炎。
结论
本病例报告详细描述了费尔蒂综合征继发肺炎和尿路感染脓毒症的临床过程。考虑到种族、年龄和类风湿关节炎确诊后的时间，我们的患者不符合常规表现。
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