Stéphane Zuily1, Vinicius Domingues2, Christine Suty-Selton3, Valérie Eschwège4, Laurent Bertoletti5, Ari Chaouat6, François Chabot6, Véronique Regnault7, Evelyn M Horn8, Doruk Erkan9, Denis Wahl10. 1. CHRU de Nancy, Regional Competence Centre for Rare Vascular and Systemic Autoimmune Diseases, Vascular Medicine Division, Nancy F-54000, France; Inserm, UMR_S 1116, Nancy F-54000, France; Université de Lorraine, Nancy F-54000, France. Electronic address: s.zuily@chru-nancy.fr. 2. NYU Langone Medical Center, Division of Rheumatology, New York, NY, USA. 3. CHRU de Nancy, Cardiology Department, Nancy F-54000, France. 4. CHRU de Nancy, Hematology Laboratory, Nancy F-54000, France. 5. CHU de Saint-Étienne, Service de Médecine Vasculaire et Thérapeutique and Université Jean-Monnet and Inserm U1059 and INNOVTE Network, Saint-Étienne F-42023, France. 6. CHRU de Nancy, Department of Respiratory Medicine and Lorraine University, INGRES, EA 7298, Nancy F-54000, France. 7. Inserm, UMR_S 1116, Nancy F-54000, France; Université de Lorraine, Nancy F-54000, France; CHRU de Nancy, Contrat d'interface, Nancy F-54000, France. 8. Weill Cornell Medicine, Division of Cardiology, New York, NY, USA. 9. Barbara Volcker Center for Women and Rheumatic Diseases, Hospital for Special Surgery, and Weill Cornell Medicine of Cornell University, New York, NY, USA. 10. CHRU de Nancy, Regional Competence Centre for Rare Vascular and Systemic Autoimmune Diseases, Vascular Medicine Division, Nancy F-54000, France; Inserm, UMR_S 1116, Nancy F-54000, France; Université de Lorraine, Nancy F-54000, France.
Abstract
BACKGROUND: Pulmonary hypertension (PH) is a life-threatening condition that may affect outcomes in patients with systemic lupus erythematosus (SLE). The role of antiphospholipid antibodies (aPL) on the risk of PH is controversial. Therefore our objective was to estimate the risk of PH (WHO groups 1-5) including associated pulmonary arterial hypertension (APAH, WHO group 1 only) related to aPL in patients with SLE. METHODS: Systematic review and meta-analysis were performed: MEDLINE, EMBASE, Cochrane Library, congress abstracts, and reference lists of eligible studies were searched through 2015. Studies were selected if they included SLE patients with descriptions of the exposure to aPL and the outcomes (PH including APAH). Two reviewers extracted study characteristics and outcome data from published reports. Estimates were pooled using random effects models and sensitivity analyses. PROSPERO registration number: CRD42015016872. RESULTS: Of 984 identified abstracts, 31 primary studies (five cohorts, 13 case-control, 13 cross-sectional) met inclusion criteria, including 4480 SLE patients. Prevalence of PH in aPL-positive vs. aPL-negative SLE patients was 12.3% vs. 7.3%, respectively. The overall pooled odds ratio (OR) for PH was 2.28 (95% CI, 1.65 to 3.15) (I2=39%). The risk of APAH was also significantly increased (OR=2.62 [95% CI, 1.11-6.15]). The risk of PH was the highest for lupus anticoagulant (OR=1.96 [95% CI, 1.31-2.92]) and IgG anticardiolipin antibodies (OR=2.64 [95% CI, 1.30-5.36]) while other antibodies were not significantly associated with PH. CONCLUSIONS: Among SLE patients, aPL can identify patients at risk for PH and APAH. These findings warrant implementation of effective screening and early treatment strategies.
BACKGROUND:Pulmonary hypertension (PH) is a life-threatening condition that may affect outcomes in patients with systemic lupus erythematosus (SLE). The role of antiphospholipid antibodies (aPL) on the risk of PH is controversial. Therefore our objective was to estimate the risk of PH (WHO groups 1-5) including associated pulmonary arterial hypertension (APAH, WHO group 1 only) related to aPL in patients with SLE. METHODS: Systematic review and meta-analysis were performed: MEDLINE, EMBASE, Cochrane Library, congress abstracts, and reference lists of eligible studies were searched through 2015. Studies were selected if they included SLEpatients with descriptions of the exposure to aPL and the outcomes (PH including APAH). Two reviewers extracted study characteristics and outcome data from published reports. Estimates were pooled using random effects models and sensitivity analyses. PROSPERO registration number: CRD42015016872. RESULTS: Of 984 identified abstracts, 31 primary studies (five cohorts, 13 case-control, 13 cross-sectional) met inclusion criteria, including 4480 SLEpatients. Prevalence of PH in aPL-positive vs. aPL-negative SLEpatients was 12.3% vs. 7.3%, respectively. The overall pooled odds ratio (OR) for PH was 2.28 (95% CI, 1.65 to 3.15) (I2=39%). The risk of APAH was also significantly increased (OR=2.62 [95% CI, 1.11-6.15]). The risk of PH was the highest for lupus anticoagulant (OR=1.96 [95% CI, 1.31-2.92]) and IgG anticardiolipin antibodies (OR=2.64 [95% CI, 1.30-5.36]) while other antibodies were not significantly associated with PH. CONCLUSIONS: Among SLEpatients, aPL can identify patients at risk for PH and APAH. These findings warrant implementation of effective screening and early treatment strategies.
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