Rationale and design of the screening of pulmonary hypertension in systemic lupus erythematosus (SOPHIE) study

Abstract

Current guideline-recommended screening for pulmonary hypertension in patients with systemic sclerosis has not been evaluated in systemic lupus erythematosus (SLE), which is disproportionately prevalent in Asians.This multicentre, cross-sectional screening study aims to study the prevalence of pulmonary hypertension among SLE patients using these guidelines, and identify independent predictors and develop a prediction model for pulmonary hypertension in SLE patients.SLE patients from participating centres will undergo an echocardiography- and biomarker-based pulmonary hypertension screening procedure as in the DETECT study. Standard right heart catheterisation will be provided to patients with intermediate or high echocardiographic probability of pulmonary hypertension. Those with low echocardiographic probability will rescreen within 1 year. The primary measure will be the diagnosis and types of pulmonary hypertension and prevalence of pulmonary hypertension in SLE patients. The secondary measures will be the predictors and prediction models for pulmonary hypertension in SLE patients. The estimated sample size is approximately 895 participants.The results of the SOPHIE study will be an important contribution to the literature of SLE-related pulmonary hypertension and may be immediately translatable to real clinical practice. Ultimately, this study will provide the necessary evidence for establishing universal guidelines for screening of pulmonary hypertension in SLE patients.

Controversies and advances in connective tissue disease‐related pulmonary arterial hypertension

“…6 However, there is a multicenter trial currently underway in China to investigate the role of PAH screening in SLE using TTE and a biomarker-based PH screening procedure as used in the DETECT study described above. 32 The low prevalence of PAH occurring in the other CTDs, including pSS, IIM and RA, does not justify the extensive screening of asymptomatic patients with these conditions and thus there are currently no PAH screening recommendations in place for these CTDs.…”

Pulmonary Hypertension Associated with Connective Tissue Disease

“…16 There are no convincing data to support screening in other CTDs-a study is currently underway to see if a screening program can be developed for systemic lupus erythematosus (SLE) where there is less certainty that the frequency of PH is sufficient to render such a program useful. 17 Screening clearly should not be used in RA or Sjogren's syndrome where the prevalence is far too low to justify such effort.…”

Histology of Pulmonary and Bronchiolar Disorders in Connective Tissue Diseases

“…It has been estimated in a recent metaanalysis that the prevalence in SLE is around 8% 28 with significant differences between gender, age, and diagnostic methods, but it can be actually higher in Asian populations in which the disease is more common. 29 Predictive factors of higher incidence are prolonged SLE disease duration (often 5 years), female under the age of 40 years, 30 and the presence of Raynaud's phenomenon, antiphospholipid antibodies (anticardiolipin and lupus anticoagulant), and anti-U1 ribonucleoprotein antibodies. 31 The pathogenesis is multifactorial and incompletely understood, but involves hypoxia, inflammatory mediators, cytokines, thrombosis, and dysregulation of bone morphogenetic protein receptor 2 (BMPR2).…”