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ACR Open Rheumatology Dec 2023This study aimed to identify risk factors associated with the development of pulmonary arterial hypertension (PAH) in patients with systemic lupus erythematosus (SLE).
OBJECTIVE
This study aimed to identify risk factors associated with the development of pulmonary arterial hypertension (PAH) in patients with systemic lupus erythematosus (SLE).
METHODS
We conducted a systematic literature review of studies focusing on adult patients classified as having SLE-related PAH by searching the electronic databases Embase, Medline, Medline in-progress, Wanfang, China National Knowledge Infrastructure, Ichushi Web, Kmbase, and KoreaMed. Based on the findings, we conducted a Delphi survey to build expert consensus on issues related to screening for PAH in patients with SLE and on the importance and feasibility of measuring the identified factors in clinical practice.
RESULTS
We included 21 eligible studies for data synthesis. Sixteen factors were associated with an increased risk of SLE-PAH: pericardial effusion, serositis, longer duration of SLE, arthritis, acute and subacute cutaneous lupus, scleroderma pattern on nailfold capillaroscopy, diffusion capacity of carbon monoxide in the lungs (DLCO) <70% predicted, interstitial lung disease, thrombocytopenia, and seven serological factors. Six factors were associated with a decreased risk of SLE-PAH: malar/acute rash, hematologic disorder, renal disorder, higher Systemic Lupus Erythematosus Disease Activity Index score, and two serological factors. Among these, there were six risk factors on which the panelists reached strong or general consensus (peak tricuspid regurgitation velocity on echocardiography >2.8 m/s, pericardial effusion, DLCO <70% predicted, scleroderma pattern on nailfold capillaroscopy, brain natriuretic peptide >50 ng/l, and N-terminal pro-brain natriuretic peptide >300 ng/l). The Delphi panel confirmed the need for a screening tool to identify patients with SLE at high risk of developing PAH and provided consensus on the importance and/or practicality of measuring the identified factors.
CONCLUSION
The risk factors we identified could be used in a screening algorithm to identify patients with SLE with a high risk of developing PAH to facilitate early diagnosis, which could improve prognosis and management of these patients.
PubMed: 37794618
DOI: 10.1002/acr2.11611 -
Medicine Dec 2023To investigate the risk factors for the development of pulmonary arterial hypertension (PAH) in patients with systemic lupus erythematosus (SLE). (Meta-Analysis)
Meta-Analysis
BACKGROUND
To investigate the risk factors for the development of pulmonary arterial hypertension (PAH) in patients with systemic lupus erythematosus (SLE).
METHODS
The literature related to risk factors for the development of PAH in SLE patients was searched by the computer on China national knowledge infrastructure (CNKI), PubMed, and Embase, and the literature search was limited to the period of library construction to October 2022. Two researchers independently performed literature screening and literature information extracting, including first author, publication time, case collection time, sample size, and study factors, and used the Newcastle-Ottawa Scale (NOS) to evaluate the quality of the literature. The relationship between each clinical manifestation and laboratory index and the occurrence of PAH in SLE patients was evaluated based on the ratio (OR value) and its 95% CI.
RESULTS
A total of 24 publications were included, including 23 case-control studies and 1 cohort study with NOS ≥ 6, and the overall quality of the literature was high. The risk of PAH was higher in SLE patients who developed Raynaud phenomenon than in those who did not [OR = 2.39, 95% CI (1.91, 2.99), P < .05]; the risk of PAH was higher in SLE patients who were positive for anti-RNP antibodies than in those who were negative for anti-RNP antibodies [OR = 1.77, 95% CI (1.17, 3.2.65), P < .05]; the risk of PAH was higher in SLE patients with interstitial lung lesions than in those without combined interstitial lung lesions [OR = 3.28, 95% CI (2.37, 4.53), P < .05]; the risk of PAH was higher in SLE patients with combined serositis than in those without serositis [OR = 2.28, 95% CI (1.83, 2.84), P < .05]. The risk of PAH was higher in SLE patients with combined pericardial effusion than in those without pericardial effusion [OR = 2.97, 95% CI (2.37, 3.72), P < .05]; the risk of PAH was higher in SLE patients with combined vasculitis than in those without vasculitis [OR = 1.50, 95% CI (1.08, 2.07), P < .05]; rheumatoid factor-positive SLE patients had a higher risk of PAH than those with rheumatoid factor-negative [OR = 1.66, 95% CI (1.24, 2.24), P < .05].
CONCLUSION
Raynaud phenomenon, vasculitis, anti-RNP antibodies, serositis, interstitial lung lesions, rheumatoid factor, and pericardial effusion are risk factors for the development of PAH in patients with SLE.
Topics: Humans; Pulmonary Arterial Hypertension; Cohort Studies; Hypertension, Pulmonary; Serositis; Pericardial Effusion; Rheumatoid Factor; Lupus Erythematosus, Systemic; Familial Primary Pulmonary Hypertension; Risk Factors; Raynaud Disease; Vasculitis
PubMed: 38134088
DOI: 10.1097/MD.0000000000036654 -
Autoimmunity Reviews Apr 2024Childhood Mixed Connective Tissue Disease (cMCTD) is the rarest pediatric connective tissue disease that includes features of systemic lupus erythematosus,... (Review)
Review
OBJECTIVE
Childhood Mixed Connective Tissue Disease (cMCTD) is the rarest pediatric connective tissue disease that includes features of systemic lupus erythematosus, polymyositis/dermatomyositis, juvenile idiopathic arthritis, and systemic sclerosis, identified by Sharp in 1972 and whose diagnosis remains challenging. This systematic review aims to identify clinical features at the onset of cMCTD and manifestations not currently included into the available diagnostic criteria.
METHODS
A systematic literature review was performed in accordance with PRISMA guidelines 2020 using bibliographic databases: MEDLINE via PubMed and EMBASE.
ELIGIBILITY CRITERIA
patients diagnosed with MCTD with onset before 18 years.
STUDIES INCLUDED
registries, retrospective and prospective cohort studies, case series and reports with analysis of data on signs and symptoms of presentation.
RESULTS
39 articles were included (215 subjects, 82.5% female), mean age of 141 months (± 41 months DS, range 2.5-204). The most used criteria for the diagnosis of MCTD were the Kasukawa criteria (54.5%). The clinical manifestations described at onset were Raynaud's phenomenon (69.7%), arthritis (60.9%), muscular involvement (53.5%), dermatological signs (39.5%), swollen fingers or hands (29.3%), arthralgias (25.6%), fever (22.3%), lung involvement (14.4%), sclerodactily (13.5%), lymphadenopathy (10.7%) serositis (10.2%), esophageal involvement (6.9%), nervous system involvement (6.9%), xeroftalmia (3.7%), xerostomia (3.7%), hepatosplenomegaly (2.8%), cardiac involvement (2.8%), hepatitis (2.3%), parotiditis (2.3%), Hashimoto's thyroiditis (0.9%), ocular involvement (0.9%).
CONCLUSIONS
The data from this systematic review suggest great heterogeneity of the clinical presentation of cMCTD for which there are no validated diagnostic criteria that may suggest a new diagnostic approach to allow earlier or more accurate diagnosis in the future.
Topics: Adolescent; Child; Child, Preschool; Female; Humans; Male; Age of Onset; Mixed Connective Tissue Disease
PubMed: 38191065
DOI: 10.1016/j.autrev.2023.103513