Factors associated with disease progression in early-diagnosed pulmonary arterial hypertension associated with systemic sclerosis: longitudinal data from the DETECT cohort

Mihai, Carina; Antic, Milos; Dobrotă, Rucsandra; Bonderman, Diana; Chadha‐Boreham, Harbajan; Coghlan, J. Gerry; Denton, Christopher P.; Doelberg, Martin; Grünig, Ekkehard; Khanna, Dinesh; McLaughlin, Vallerie V.; Müller‐Ladner, Ulf; Pope, Janet; Rosenberg, D; Seibold, James R.; Vonk, Madelon C.; Distler, Oliver

doi:10.1136/annrheumdis-2017-211480

Cited by 22 publications

(18 citation statements)

References 12 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…For example, in the previously described EUSTAR study examining cardiac dysfunction in SSc, male sex was independently predictive of new onset PAH (odds ratio of 2.66) (58). In a study that included patients with SSc-PAH enrolled in the DETECT cohort study for up to three years, univariate logistic regression revealed that male sex was associated (odds ratio of 4.1) with disease progression (63). Whereas, in the Spanish RESCLE cohort study, the investigators reported a clear predominance of pulmonary hypertension in females, in particular, in the absence of interstitial lung disease (40)…”

Section: Respiratorymentioning

confidence: 97%

Gender-related differences in systemic sclerosis

Hughes

Pauling

Armstrong-James

et al. 2020

Autoimmunity Reviews

View full text Add to dashboard Cite

Systemic sclerosis (SSc) is a complex autoimmune connective tissue disease which is characterised by widespread tissue fibrosis of the skin and internal organs, widespread vasculopathic alterations and immune system abnormalities. The condition is more common in women, but has a more severe expression of disease and higher mortality in men. Although the aetiopathogenesis of SSc is not yet fully understood, there is emerging evidence of important differences between men and women with the disease. This review brings together the current evidence on sex differences in SSc including epidemiology, pathophysiology, clinical expression of disease, mortality, SSc in transgender individuals, and psychosocial aspects of disease.

show abstract

Section: Respiratorymentioning

confidence: 97%

Gender-related differences in systemic sclerosis

Hughes

Pauling

Armstrong-James

et al. 2020

Autoimmunity Reviews

View full text Add to dashboard Cite

show abstract

“…When compared with European Society of Cardiology/European Respiratory Society 2009 guidelines, the DETECT algorithm recommended more patients for RHC and detected more patients with PAH [ 129 ]. In DETECT study where 57 patients with SSc-PAH were followed [ 130 ], 44% (25/57) of them showed progression during a median follow-up of 12.6 months. Thirteen of the twenty-five showed mild PAH in WHO functional class I or II.…”

Section: Lung Involvement In Systemic Sclerosis (Ssc)mentioning

confidence: 99%

“…Thirteen of the twenty-five showed mild PAH in WHO functional class I or II. Factors associated with progression were male gender, lower DLCO, higher FVC/DLCO ratio, and poor functional capacity [ 130 ].…”

Section: Lung Involvement In Systemic Sclerosis (Ssc)mentioning

confidence: 99%

Lung Involvements in Rheumatic Diseases: Update on the Epidemiology, Pathogenesis, Clinical Features, and Treatment

Lee

Kang

2018

BioMed Research International

View full text Add to dashboard Cite

Lung illness encountered in patients with rheumatic diseases bears clinical significance in terms of increased morbidity and mortality as well as potential challenges placed on patient care. Although our understanding of natural history of this important illness is still limited, epidemiologic knowledge has been accumulated during the past decade to provide useful information on the risk factors and prognosis of lung involvements in rheumatic diseases. Moreover, the pathogenesis particularly in the context of genetics has been greatly updated for both the underlying rheumatic disease and associated lung involvement. This review will focus on the current update on the epidemiologic and genetics features and treatment options of the lung involvements associated with four major rheumatic diseases (rheumatoid arthritis, systemic sclerosis, myositis, and systemic lupus erythematosus), with more attention to a specific form of involvement or interstitial lung disease.

show abstract

“…[78,79]. The DETECT cohort included patients with mild PAH and specific determinants of PAH progression have been elucidated using data from this cohort [80] and align with variables such as Nt-pro-BNP and low DLco identified in other cohorts [52,81,82]. The DETECT algorithm first examines six variables (FVC, telangiectasia, ACA positivity, electrocardiogram right axis deviation and serum levels of Nt-pro-BNP and urate) to generate a score.…”

Section: [H2] Screening and Diagnosis Of Pulmonary Hypertensionmentioning

confidence: 99%

Major lung complications of systemic sclerosis

2018

View full text Add to dashboard Cite

Systemic sclerosis (SSc) is associated with high mortality owing to internal organ complications, and lung disease is the leading cause of SSc-associated death. The most notable lung complications in SSc are fibrosis and pulmonary arterial hypertension (PAH). A major challenge for the management of lung disease in SSc is detecting those patients with severe pathology and those patients who are likely to benefit from available treatments. In the past few years, strategies for managing lung fibrosis and pulmonary hypertension, including PAH, have greatly progressed. For lung fibrosis, the tools to assess risk of progression and severity of the disease have been refined. Clinical trial results support the use of immunosuppression, including high-intensity regimens with autologous stem cell transplantation. New trials are underway to test other potential therapies including treatments that are approved for use in idiopathic lung fibrosis. For PAH, identifying individuals at high risk of disease development is critical. In addition, individuals who have borderline elevation of pulmonary arterial pressure need to be appropriately managed and followed up. Many approved drugs targeting PAH are now available, and results from large-scale clinical trials provide robust evidence that various treatments for SSc-associated PAH are associated with good long-term outcomes.

show abstract

Factors associated with disease progression in early-diagnosed pulmonary arterial hypertension associated with systemic sclerosis: longitudinal data from the DETECT cohort

Cited by 22 publications

References 12 publications

Gender-related differences in systemic sclerosis

Gender-related differences in systemic sclerosis

Lung Involvements in Rheumatic Diseases: Update on the Epidemiology, Pathogenesis, Clinical Features, and Treatment

Major lung complications of systemic sclerosis

Contact Info

Product

Resources

About