29 Management of pulmonary hypertension in systemic lupus erythematosus patients

Pulmonary arterial hypertension (PAH) is a complex and devastating disease. According to a longitudinal United States-based registry, connective tissue disease (CTD) accounted for more than 50% of all patients with PAH, in which systemic sclerosis comprised the largest CTD-related PAH.1 In contrast to Western countries, systemic lupus erythematosus (SLE) is a more common CTD than systemic sclerosis in the Asia-Pacific region.2 A cohort study from China has shown that SLE, instead of systemic sclerosis, comprised the largest proportion of all CTD-related PAH.3 The prevalence of PAH in SLE is estimated at 0.5%–17.5%.4 The pathogenesis of PAH involves multiple mechanisms including vasculitis, in situ thrombosis to interstitial lung disease which may all increase pulmonary vascular resistance and lead to right heart failure. The leading risk factors for the development of PAH in SLE patients include Raynaud’s phenomenon, anti-U1 RNP antibody and anti-cardiolipin antibodies positivity. Since PAH is potentially life-threatening, early detection is crucial to improve the outcomes of this condition. Currently, the diagnostic algorithm for PAH in SLE patients follows that of international guidelines. Diagnosis is confirmed by right heart catheterisation. Treatments are similar to the therapeutic interventions for patients with idiopathic PAH. Since inflammatory and dysregulated immune components may play a major role in the pathogenesis of PAH in SLE, glucocorticoids and immunosuppressive therapies including cyclophosphamide are used, although the immunosuppressive therapy trials were small, uncontrolled studies only. Regular follow-up with prognostic evaluation and risk assessment should be performed and the treatment should be individualised accordingly. References McGoon MD, Miller DP. REVEAL: a contemporary US pulmonary arterial hypertension registry. Eur Respir Rev 2012;21:8–18. Jakes RW, Bae SC, Louthrenoo W, Mok CC, Navarra SV, Kwon N. Systematic review of the epidemiology of systemic lupus erythematosus in the Asia-Pacific region: prevalence, incidence, clinical features, and mortality. Arthritis Care Res (Hoboken) 2012;64:159–168. Zhao J, Wang Q, Liu Y, Tian Z, Guo X, Wang H, Lai J, Huang C, Yang X, Li M, Zeng X. Clinical characteristics and survival of pulmonary arterial hypertension associated with three major connective tissue diseases: A cohort study in China. Int J Cardiol 2017;236:432–437. Condliffe R, Kiely DG, Peacock AJ, Corris PA, Gibbs JS, Vrapi F, Das C, Elliot CA, Johnson M, DeSoyza J, Torpy C, Goldsmith K, Hodgkins D, Hughes RJ, Pepke-Zaba J, Coghlan JG. Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med 2009;179:151–157.