Severe Pulmonary Artery Hypertension in Otherwise Silent Lupus: A Unique Hybrid Treatment Approach Using Hydroxychloroquine and Sildenafil

Abstract

Pulmonary hypertension (PH) is a rare manifestation of systemic lupus erythematosus (SLE). Even more rare is pulmonary artery hypertension (PAH) presenting as the initial manifestation of SLE and may be a cause of diagnostic delay. As symptoms of PAH are very mild in the early stages, prompt diagnosis is crucial to prevent the progression of the disease. Echocardiographic evaluation involving the measurement of different right-sided heart variables in addition to estimated pulmonary artery pressure helps in reducing the false-positive rates of detection of PAH. The role of immunosuppression in addition to PAH-specific vasodilator therapy is one of the key aspects of management to minimize flares and improve hemodynamics. Equally important is the choice of a regimen best suited to minimize complications. We present a case of PAH in newly diagnosed SLE and the diagnostic and treatment challenges as a safety net hospital.