Autoimmune polyendocrine syndrome type 4 with systemic lupus erythematosus and anti-phospholipid syndrome  

作　　者：Yu Wang Juan Zhao Ting-Ting Zhang Xiao-Wei Ma Jun-Qing Zhang Zhuo-Li Zhang 

机构地区：[1]Department of Rheumatology and Clinical Immunology,Peking University First Hospital,Beijing 100034,China [2]Department of Endocrinology,Peking University First Hospital,Beijing 100034,China

出　　处：《Chinese Medical Journal》2020年第8期994-995,共2页中华医学杂志（英文版）

摘　　要：A 50-year-old Chinese woman who experienced intermittent fever with xerostomia and xerophthalmia for 24 years,paroxysmal loss of consciousness for 11 years,aggravation for 1 year was admitted to the Peking University First Hospital in 2019.She started to have intermittent fever(38-39°C)every 2 to 3 months 24 years before first rheumatological evaluation,and the temperature can return to normal without intervention.After she had an episode of epilepsy with abdominal pain 23 years ago,she had severe weight loss,fatigue,weakness,skin pigmentation,and low blood pressure(80/45 mmHg).Laboratory test showed normal potassium,hyponatremia 133 mmol/L(normal range 135-145 mmol/L),low serum cortisol concentration in the early morning 0.56μg/mL(normal range 4.40-9.20μg/dL),low 24-h urine-free cortisol 259.6μg/d(normal range 370.0-639.0μg/d)and high adrenocortico-tropic-hormone(ACTH)128.37 pg/mL(normal range 7.20-63.30 pg/mL).She also had anemia with hemoglobin 77 g/L,thrombocytopenia with platelet 70×109/L to 90×109/L and dramatically prolonged activated partial thromboplastin time(APTT)105.1 s(normal range 22.7-31.8 s).Serum creatinine was elevated from 110 to 135μmol/L(normal range 44-133μmol/L).Both blood cultures and purified protein derivative test were negative.With prednisolone 30 mg daily along with supportive therapy,the patient’s blood pressure was back to normal with no more fever,and serum creatinine decreased to 120μmol/L.Meanwhile,the patient’s fatigue and skin pigmentation were also alleviated,but her armpit and pubic hairs shed.Further autoimmune tests showed positive anti-nuclear antibodies(ANA)1:1000 and anti-SSA antibody;however,normal serum complements levels.Since she was diagnosed as autoimmune adrenal insufficiency(AI),prednisone replacement therapy(5 mg 8 AM and 5 mg 4 PM each day)has been maintained for more than 20 years.

关 键 词：admitted EPILEPSY FATIGUE 

分 类 号：R593.24[医药卫生—内科学]