Document resource
Objectives To describe the clinical case of severe pulmonary hypertension (PH) as the initial manifestation of systemic lupus erythematosus (SLE).Methods Case report A 21-year-old woman, with a family history of SLE, in the immediate postpartum period, was admitted to the Coronary Care Unit due to progressive dyspnea, palpitations, and lower limb edema.Physical examination blood pressure 140/90 mmHg, heart rate 110 bpm, respiratory rate 19 rpm, O2 saturation 95%, temperature 36.5 °C. A grade 5/6 holosystolic regurgitant murmur was heard in the pulmonary area. Bibasilar crackles were present. Bilateral infrapatellar pitting edema ++.Echocardiography: severely dilated right chambers, dilated pulmonary trunk (43 mm), tricuspid and pulmonary regurgitation, mild pericardial effusion, and LVEF 45%. Chest CT angiography: multiple bilateral subsegmental filling defects. Right heart catheterization: pulmonary artery systolic pressure 83 mmHg, pre-capillary pattern, consistent with chronic thromboembolic disease. Spirometry: reduced FVC (69.6%), reduced FEV1 (56.6%), preserved FEV1/FVC ratio. Treatment with enoxaparin, furosemide, sildenafil, ivabradine and bosentan was initiated.After 3 months, she developed malar rash, polyarthralgias, and Raynaud’s phenomenon. Laboratory tests: WBC 3900/mm3, platelets 123,000/mm3, ANA 1/160 speckled, C3 107, C4 7, anti-dsDNA negative, lupus anticoagulant, anti-B2GP1 and anticardiolipin antibodies negative. Anti-SSA/Ro60 kD was positive. Capillaroscopy was normal. A diagnosis of SLE-associated severe PH was made, and hydroxychloroquine and prednisone were added.At 1-year follow-up with chest CT angiography and V/Q scan, no thromboembolic lesions were observed. However, she persisted with right heart failure symptoms and estimated PASP 60 mmHg. Subcutaneous treprostinil infusion was initiated, with partial symptomatic improvement, and cardiopulmonary transplantation was considered.Results PH persisted despite thromboembolism resolution, supporting its classification as Group 1 PH in the context of SLE.Therapeutic options include calcium channel blockers, phosphodiesterase inhibitors, endothelin receptor antagonists and, in refractory cases, riociguat or prostanoids. Evidence supporting the role of deep immunosuppression is limited, highlighting the need for further research.Conclusions Severe pulmonary hypertension with right ventricular dysfunction is a rare but life-threatening manifestation of SLE. Despite current therapies, prognosis remains poor, and a multidisciplinary approach, including consideration of advanced therapies such as prostanoids and transplantation, is essential.