Systemic Sclerosis and Systemic Lupus Erythematosus Overlap Syndrome with Pulmonary Arterial Hypertension Successfully Treated with Immunosuppressive Therapy and Riociguat

Abstract

We report the case of a 40-year-old patient with systemic sclerosis (SSc) and systemic lupus erythematosus (SLE) overlap syndrome with pulmonary arterial hypertension (overlap-PAH) that was successfully treated with a combination of immunosuppressive therapy and the soluble guanylate cyclase stimulator riociguat. She was diagnosed with mixed connective tissue disease (MCTD) two years prior to admission. She was admitted to our hospital with dyspnea on exertion and progressive skin sclerosis. She fulfilled both SLE and SSc classification criteria and was re-diagnosed with overlap syndrome. The tricuspid valve pressure gradient (TRPG) on echocardiography was 64 mmHg at admission. On right heart catheterization, mean pulmonary arterial pressure (mPAP) was 43 mmHg and pulmonary capillary wedge pressure was 15 mmHg. We diagnosed her with SSc-SLE overlap-PAH and started treatment with corticosteroids and intravenous cyclophosphamide. We also started treatment with riociguat because we speculated she had a component of SSc-PAH and that immunosuppressive therapy alone may be insufficient. We chose riociguat because of its favorable treatment effect on SSc-PAH. Two months after treatment, her TRPG improved to 33 mmHg and the skin sclerosis improved dramatically, suggesting the efficacy of multi-drug treatment and the importance of early intervention.

Systemic Lupus Erythematosus-related Lung Disease

“…[103][104][105][106][107][108] Riociguat, the guanylate cyclase stimulator, is effective in a small number of SLE-associated PAH cases, with some benefits also seen from the use of corticosteroids and immunosuppressive therapy, including cyclophosphamide, cyclosporine, and MMF. [109][110][111][112][113][114] There is some consensus that a combination of standard PAH therapy alongside immunosuppression is best for treating SLE-related PAH. Overall, observational reports suggest that a combination of both immunosuppression and traditional PAH treatment should be used together to enhance long-term outcomes.…”

Lupus and the Lungs: The Assessment and Management of Pulmonary Manifestations of Systemic Lupus Erythematosus

“…Drugs such as phosphodiesterase-5 inhibitors, endothelin receptor antagonists and prostacyclin pathway agonists have all shown to be effective in SLE associated PAH to varying degrees ( 59 – 64 ). More recently, the guanylate cyclase stimulator riociguat has shown to be effective in a small number of SLE-associated PAH cases ( 65 , 66 ).…”

Respiratory Manifestations in Systemic Lupus Erythematosus

“…Immunosuppressants can be combined with vasoactive agents in more severe forms. Supportive treatments such as diuretics, anticoagulants and oxygen may be beneficial [ 88 , 94 , 107 , 108 , 109 , 110 ].…”