AB0556 PARTICULARITIES OF PULMONARY HYPERTENSION IN SYSTEMIC LUPUS ERYTHEMATOSUS

Background: Systemic lupus erythematosus (SLE) is an autoimmune disease that primarily affects young women. It is characterized by the production of autoantibodies and immune complexes. Vascular pulmonary involvement has long been considered rare, a consequence of thromboembolic events. It is in fact a proper entity, often of multifactorial mechanism, with spontaneous evolution to pulmonary arterial hypertension (PAH), which makes its gravity Objectives: The objective of our study is to determine the particuliarities of PAH during SLE. Methods: We conducted a mono-centric, retrospective and descriptive study of the follow up of patients in the internal medicine department of the Military Hospital of Tunis for LES (diagnosis according to the ARA criteria) between January 2010 and December 2015. All patients underwent external echocardiography or during their stay in the department Results: We collected 87 patients diagnosed with SLE. PAH was recorded in 16 patients (38%) including 6 men and 10 women with a F/M sex ratio of 1.66. The average age was 37.23 years with extremes ranging from 16 to 70 years old. Clinically, we observed dyspnea in 10 patients (62%), chest pain in 7 cases (43%), dry cough in 2 cases (12%), palpitations in 1 case (6%),%) and right heart failure in 1 case (6%). At cardiac auscultation, 5 patients were tachycardic (31%), 5 had a tricuspid systolic murmur and 1 had an irregular rhythm. A burst of B2 in the pulmonary focus was noted in 3 patients (18%). Nine of the 16 patients with PAH had electrical signs: five had sinus tachycardia (31%), two had signs of right ventricular hypertrophy. A complete arrhythmia with atrial fibrillation was noted in one patient. The chest X-ray showed cardiomegaly in 6 patients (37%). Among the 16 patients, there was a tricuspid valve insufficiency associated with PAH in 7 patients (43%), 2 had mitral valve insufficiency (12%), 6 patients had pericarditis, one patient had endocarditis (6%) and myocarditis was found in another one (6%). PAH was isolated in 12% of cases. In Immunological tests, NAAs were positive in all patients. Three quarters of the patients (12) had native anti-DNA positive (75%) and ¼ of the patients (4) had anti-Sm positive and anti-RNP positive antibodies (25%). The complement was consumed in 5 cases (31.2%). Anti-phospholipid antibodies were noted in 4 cases (25%). Added to non-specific measures (smoking cessation, elimination of intense physical effort ...) adopted for all patients, oxygen therapy was indicated in four patients. Diltiazem 180 mg/day vasodilator therapy was prescribed in 3 patients. Effective anti-vitamin K anticoagulation was prescribed in a patient with severe PAH at 80 mmHg. Conclusion: Pulmonary arterial hypertension (PAH) is a rare complication of systemic lupus erythematosus, its prevalence varies from 0.5 to 17.5% depending on the series. SLE is the second leading cause of PAH in connective tissue disease after systemic sclerosis. This vascular involvement is essential for the prognosis and is an important evolutionary step in the management. Disclosure of Interests: None declared