Aggressively targeting the inflammatory pathway in SLE-PAH improved hemodynamics and allowed for titration off prostacyclin.

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Pulmonary Arterial hypertension (PAH) is a progressive disease confers a poor prognosis, often leading to right ventricular failure and death. Prostacyclin infusion is Class IA recommendation for the treatment of patients with NYHA FC III and IV symptoms. Despite its pivotal role in management, patients often find it onerous as administration is complex, requiring central venous access, and small errors in administration can be life-threatening. Transition off of parenteral therapy to oral or inhaled agents is often a patient goal, but can be difficult to achieve. CASE PRESENTATION: A 37 year old woman with SLE was diagnosed with Group 1 PAH after a right heart catheterization (RHC) noting mean pulmonary arterial pressure of 42mmHg, a normal wedge pressure and a low cardiac output and index. Her symptoms were consistent with NYHA FC IV. Patient's SLE was uncontrolled at the time of diagnosis as manifested by diffuse arthralgias, alopecia, and a rash. Serology was indicative of flare, with decrease in complement levels and elevated DSDNA titers. (Fig. 1) She was initiated on sequential triple therapy with IV prostacyclin endothelin receptor antagonist and phosphodiesterase type 5 inhibitor. Her immunosuppression, cellcept and prednisone, doses were adjusted to achieve control of disease activity. After 6 years of her diagnosis and control of SLE as shown with normalization of complements and DSDNA titers, her BNP, six minute walk distance, V02 max, and right sided morphology normalized. Her NYHA FC improved to II. RHC that was repeated revealed normalization of her hemodynamics. Decision was to wean off her IV treprostinil (30 ng/kg/min) with ongoing oral therapy. Follow up functional and hemodynamic parameters over the past three years have noted stability of her PAH. DISCUSSION: The pathophysiology of arteriopathy in PAH associated with CT diseases implicates an immune and an inflammatory pathway as evident by presence of macrophages and lymphocytes in the plexiform lesions and the presence of IgG and complements in the pulmonary arterial walls. Treatment of the inflammatory pathway is an avenue to reduce the extent of remodeling that may occur and thus better control of underlying PAH. CONCLUSIONS: We present this case to stress on the importance of control of disease activity in the regression of PAH, and allowance for titration of parenteral prostacyclin.





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