The effectiveness of upfront pulmonary vasodilators combination therapy in addition to corticosteroids for pulmonary arterial hypertension associated with systemic lupus erythematosus: two successful cases utilising both corticosteroids and an upfront combination of vasodilators

Abstract

The effectiveness of an upfront combination therapy of pulmonary arterial vasodilators on pulmonary arterial hypertension (PAH) associated with systemic lupus erythematosus (SLE) has not yet been established. Here we present two cases of SLE-PAH successfully treated by such a regimen. Case 1 was a 42-year-old Chinese female who was diagnosed as SLE based on 1997 American College of Rheumatology (ACR) criteria for arthritis, lymphocytopenia, thrombocytopenia, and positivity for anti-nuclear antibody (ANA) and anti-DNA antibody (ADNA). PAH was diagnosed simultaneously based on increased mean pulmonary arterial pressure (mPAP) (44mmHg) in right heart catheterisation. She was treated with macitentan, tadalaphil, and beraprost sodium, as an upfront triple combination therapy in addition to 45mg/day of prednisolone (PSL). She was successfully treated, resulting in a decrease of right ventricular systolic pressure (RVSP) on echocardiography from 68.4mmHg to 31.4mmHg within 4 months. Case 2 was a 34-year-old Japanese woman diagnosed as SLE based on 1997 ACR criteria, presenting with arthritis, pericarditis, and positivity for ANA and ADNA. PAH was diagnosed simultaneously based on increased mPAP (40mmHg). She was treated with beraprost sodium and bosentan as an upfront dual combination therapy, in addition to 60mg/d of PSL. Her RVSP was dramatically decreased from 98mmHg to 32.1mmHg. Although beraprost sodium and bosentan were eventually discontinued due to flushing and hypotension within a 7-month period, her PAH remained stable with decreased RVSP (28mmHg) under PSL monotherapy. Our two cases suggest a promising therapeutic value of the upfront combination of pulmonary vasodilators in addition to immunosuppressive therapy for SLE-PAH.