Characteristics of Acquired Inhibitors to Factor VIII and Von Willebrand Factor Secondary to Systemic Lupus Erythematosus: Experiences From a Chinese Tertiary Medical Center.

Szczegóły
Abstrakt

Tytuł:: Characteristics of Acquired Inhibitors to Factor VIII and Von Willebrand Factor Secondary to Systemic Lupus Erythematosus: Experiences From a Chinese Tertiary Medical Center.
Autorzy:: Cao XY; From the Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Graduate School of Peking Union Medical College, Beijing.
Li MT; From the Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Graduate School of Peking Union Medical College, Beijing.
Zhang X; From the Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Graduate School of Peking Union Medical College, Beijing.
Zhao Y; From the Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Graduate School of Peking Union Medical College, Beijing.
Zeng XF; From the Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Graduate School of Peking Union Medical College, Beijing.
Zhang FC; From the Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Graduate School of Peking Union Medical College, Beijing.
Hou Y; From the Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Graduate School of Peking Union Medical College, Beijing.
Zhu LX
Źródło:: Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases [J Clin Rheumatol] 2021 Aug 01; Vol. 27 (5), pp. 201-205.
Typ publikacji:: Journal Article
Język:: English
Imprint Name(s):: Publication: Philadelphia, PA : Lippincott Williams & Wilkins
Original Publication: Baltimore, MD : Williams & Wilkins, c1995-
MeSH Terms:: Factor VIII*
Lupus Erythematosus, Systemic*/complications
Lupus Erythematosus, Systemic*/diagnosis
von Willebrand Factor*
Hemophilia A/*etiology
Adult ; Antiphospholipid Syndrome/etiology ; China/epidemiology ; Female ; Hospitals ; Humans ; Middle Aged ; Young Adult
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Substance Nomenclature:: 0 (von Willebrand Factor)
9001-27-8 (Factor VIII)
Entry Date(s):: Date Created: 20191210 Date Completed: 20210818 Latest Revision: 20230927
Update Code:: 20240105
PubMed Central ID:: PMC8300847
DOI:: 10.1097/RHU.0000000000001284
PMID:: 31815811
: Czasopismo naukowe

Objective: Because acquired hemophilia (AH) is a rare entity in systemic lupus erythematosus (SLE), we aimed to investigate the clinical features of SLE-related AH in Chinese patients.
Methods: This is a medical records review study carried out at a large tertiary care hospital in China from years 1986 to 2018. We searched the case database in Peking Union Medical College Hospital using the International Classification of Diseases. The clinical data on SLE-related AH patients were collected.
Results: A total of 9282 SLE patients had been hospitalized. Six female SLE-related AH patients were identified. Four patients had acquired hemophilia A (AHA), and 2 patients had acquired von Willebrand syndrome. Their mean age was 33.67 ± 13.77 years. Five patients had active disease. The mean SLE disease activity index measured at the time of diagnosis of AH was 10.50 ± 5.28. The average level of activated partial thromboplastin time was 86.5 seconds. Coexistence of secondary antiphospholipid syndrome and AHA was found in one case, and pulmonary embolism was observed 3 years later. After immunosuppressive therapy and symptomatic treatment, an overall remission rate of 83.3% was achieved.
Conclusions: The frequency of SLE-related AH was low. The development of AH in SLE patients frequently occurs with active disease. The AH could be the first clinical presentation of SLE. Secondary antiphospholipid syndrome and AHA could appear in the same SLE patient. Early and aggressive treatment contributes to a favorable prognosis.
Competing Interests: The authors declare no conflict of interest. Dr Yong Hou is funded by grants from the National Natural Science Foundation of China (81671614, www.nsfc.gov.cn/), Chinese Academy of Medical Sciences Initiative for Innovative Medicine 2017-12M-3-001.
(Copyright © 2019 The Author(s). Published by Wolters Kluwer Health, Inc.)

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