title: Pulmonary hypertension in connective tissue diseases: an update.
abstract: Pulmonary hypertension (PH) is a relatively commoner complication of systemic sclerosis (SSc) with estimated prevalence ranging between 8% and 12% as compared to much lower figures in other connective tissue diseases (CTD). It is a major cause of morbidity and mortality in CTDs. PH is classified into five major groups. CTD-associated PH belongs to group 1 PH, also known as pulmonary arterial hypertension (PAH). Around 30% of scleroderma-related deaths are due to PAH. Underlying pathogenesis is related to pulmonary vasculopathy involving small vessels. The Evidence-based Detection of Pulmonary Arterial Hypertension in Systemic sclerosis (DETECT) algorithm outperforms the current European Society of Cardiology/European Respiratory Society guidelines as a screening tool in SSc-PAH; it can, therefore, suggest when to refer a patient for right heart catheterization. CTD-PAH patients constitute at least 20% of patients included in all major trials of PH-specific therapy and the results are comparable to those of idiopathic PAH. The role of anticoagulation in CTD-PAH is associated with a high risk-benefit ratio with the caveat of its potential role in those with severe disease. There appears to be no role of immunosuppression in scleroderma-PAH; however, immunosuppressive agents, namely the combination of glucocorticoids and pulse cyclophosphamide / possibly mycophenolate, may result in clinical improvement in a subset of patients with systemic lupus erythematosus and mixed connective tissue disease-related PAH.
authors.affiliation: Department ofClinical Immunology & Rheumatology, Christian Medical College, Vellore, Tamil Nadu, India.
authors.forename: Ramya
authors.initials: R
authors.lastname: Aithala
authors.affiliation: Department ofCardiology, Christian Medical College, Vellore, Tamil Nadu, India.
authors.forename: Anoop G
authors.initials: AG
authors.lastname: Alex
authors.affiliation: Department ofClinical Immunology & Rheumatology, Christian Medical College, Vellore, Tamil Nadu, India.
authors.forename: Debashish
authors.initials: D
authors.lastname: Danda
mesh_terms: D000925:Anticoagulants / Q000627:therapeutic use; D000959:Antihypertensive Agents / Q000627:therapeutic use; D062186:Arterial Pressure; D006328:Cardiac Catheterization; D003240:Connective Tissue Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality / Q000503:physiopathology; D004359:Drug Therapy, Combination; D004452:Echocardiography; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality / Q000503:physiopathology; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D011237:Predictive Value of Tests; D012129:Respiratory Function Tests; D012307:Risk Factors; D016896:Treatment Outcome; D016278:Ventricular Function, Right
chemical_list: D000925:Anticoagulants; D000959:Antihypertensive Agents; D007166:Immunosuppressive Agents
keywords: clinical aspects; pulmonary hypertension; systemic sclerosis