ANA-positive primary immune thrombocytopaenia: a different clinical entity with increased risk of connective tissue diseases

Objective Primary immune thrombocytopaenia (ITP) is highly heterogeneous. ANA-positive primary ITP may resemble the preclinical stage of connective tissue diseases (CTDs), but is still considered primary ITP due to a controversial CTD risk assessment in this group. The objective of this study was to...

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Main Authors: Yuan Liu, Bin Wang, Shiju Chen, Guixiu Shi, Hongyan Qian, Guomei Yang, Jinying Lan, Fan Dai, Peishi Rao, Puqi Wu
Format: Article
Language:English
Published: BMJ Publishing Group 2021-04-01
Series:Lupus Science and Medicine
Online Access:https://lupus.bmj.com/content/8/1/e000523.full
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author Yuan Liu
Bin Wang
Shiju Chen
Guixiu Shi
Hongyan Qian
Guomei Yang
Jinying Lan
Fan Dai
Peishi Rao
Puqi Wu
author_facet Yuan Liu
Bin Wang
Shiju Chen
Guixiu Shi
Hongyan Qian
Guomei Yang
Jinying Lan
Fan Dai
Peishi Rao
Puqi Wu
author_sort Yuan Liu
collection DOAJ
description Objective Primary immune thrombocytopaenia (ITP) is highly heterogeneous. ANA-positive primary ITP may resemble the preclinical stage of connective tissue diseases (CTDs), but is still considered primary ITP due to a controversial CTD risk assessment in this group. The objective of this study was to clarify the risk of CTD in ANA-positive patients with primary ITP.Methods We performed a retrospective cohort study and a meta-analysis. 586 patients with newly diagnosed primary ITP were followed up and Cox regression analyses were used to analyse the associations of ANA positivity and other immune parameters with CTD development.Results The mean follow-up time was 37 (19–56) months. ANA was positive in 21.33% (125 of 586) of patients with primary ITP in our retrospective cohort, and the overall rate of ANA positivity in the meta-analysis was 17.06% (369 of 2163). The adjusted HR for CTD in ANA-positive primary ITP was 6.15 (95% CI 2.66 to 14.23, p<0.001). Five patients in the ANA-positive group developed SLE (5 of 125, 4.0%), significantly higher than in the ANA-negative group (0 of 461, 0%). A clinical model combining ANA, anti-Sjogren’s syndrome A antibody and C3 was successfully developed to predict the risk of CTD in patients with primary ITP. Increased risk of CTD (risk ratio=12.43, 95% CI 7.91 to 19.55, p<0.00001), especially SLE (risk ratio=30.41, 95% CI 13.23 to 69.86, p<0.00001), among ANA-positive patients with primary ITP was confirmed by a meta-analysis of previous studies and the present study.Conclusions The findings suggest that ANA-positive primary ITP is a clinical entity distinct from other primary ITPs and is associated with increased risk of developing CTDs, especially SLE.
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spelling doaj-art-31e1bf93ae19491997d37bd0430df3d42025-08-20T02:49:17ZengBMJ Publishing GroupLupus Science and Medicine2053-87902021-04-018110.1136/lupus-2021-000523ANA-positive primary immune thrombocytopaenia: a different clinical entity with increased risk of connective tissue diseasesYuan Liu0Bin Wang1Shiju Chen2Guixiu Shi3Hongyan Qian4Guomei Yang5Jinying Lan6Fan Dai7Peishi Rao8Puqi Wu91Emory University, Atlanta, GA, USADepartment of Occupational & Environmental Health, School of Public Health, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, ChinaSchool of Medicine, Xiamen University, Xiamen, China2the First Affiliated Hospital of Xiamen University, Xiamen, ChinaDepartment of Rheumatology and Clinical Immunology, The First Affiliated Hospital of Xiamen University, Xiamen, Fujian, ChinaDepartment of Rheumatology, The Affiliated Hospital of Southwest Medical University, Luzhou, ChinaDepartment of Rheumatology and Clinical Immunology, The First Affiliated Hospital of Xiamen University, Xiamen, Fujian, ChinaDepartment of Rheumatology and Clinical Immunology, The First Affiliated Hospital of Xiamen University, Xiamen, Fujian, ChinaDepartment of Rheumatology and Clinical Immunology, The First Affiliated Hospital of Xiamen University, Xiamen, Fujian, ChinaDepartment of Rheumatology and Clinical Immunology, The First Affiliated Hospital of Xiamen University, Xiamen, Fujian, ChinaObjective Primary immune thrombocytopaenia (ITP) is highly heterogeneous. ANA-positive primary ITP may resemble the preclinical stage of connective tissue diseases (CTDs), but is still considered primary ITP due to a controversial CTD risk assessment in this group. The objective of this study was to clarify the risk of CTD in ANA-positive patients with primary ITP.Methods We performed a retrospective cohort study and a meta-analysis. 586 patients with newly diagnosed primary ITP were followed up and Cox regression analyses were used to analyse the associations of ANA positivity and other immune parameters with CTD development.Results The mean follow-up time was 37 (19–56) months. ANA was positive in 21.33% (125 of 586) of patients with primary ITP in our retrospective cohort, and the overall rate of ANA positivity in the meta-analysis was 17.06% (369 of 2163). The adjusted HR for CTD in ANA-positive primary ITP was 6.15 (95% CI 2.66 to 14.23, p<0.001). Five patients in the ANA-positive group developed SLE (5 of 125, 4.0%), significantly higher than in the ANA-negative group (0 of 461, 0%). A clinical model combining ANA, anti-Sjogren’s syndrome A antibody and C3 was successfully developed to predict the risk of CTD in patients with primary ITP. Increased risk of CTD (risk ratio=12.43, 95% CI 7.91 to 19.55, p<0.00001), especially SLE (risk ratio=30.41, 95% CI 13.23 to 69.86, p<0.00001), among ANA-positive patients with primary ITP was confirmed by a meta-analysis of previous studies and the present study.Conclusions The findings suggest that ANA-positive primary ITP is a clinical entity distinct from other primary ITPs and is associated with increased risk of developing CTDs, especially SLE.https://lupus.bmj.com/content/8/1/e000523.full
spellingShingle Yuan Liu
Bin Wang
Shiju Chen
Guixiu Shi
Hongyan Qian
Guomei Yang
Jinying Lan
Fan Dai
Peishi Rao
Puqi Wu
ANA-positive primary immune thrombocytopaenia: a different clinical entity with increased risk of connective tissue diseases
Lupus Science and Medicine
title ANA-positive primary immune thrombocytopaenia: a different clinical entity with increased risk of connective tissue diseases
title_full ANA-positive primary immune thrombocytopaenia: a different clinical entity with increased risk of connective tissue diseases
title_fullStr ANA-positive primary immune thrombocytopaenia: a different clinical entity with increased risk of connective tissue diseases
title_full_unstemmed ANA-positive primary immune thrombocytopaenia: a different clinical entity with increased risk of connective tissue diseases
title_short ANA-positive primary immune thrombocytopaenia: a different clinical entity with increased risk of connective tissue diseases
title_sort ana positive primary immune thrombocytopaenia a different clinical entity with increased risk of connective tissue diseases
url https://lupus.bmj.com/content/8/1/e000523.full
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