Objective: Pulmonary hypertension (PH) was followed in systemic lupus erythematosus (SLE) patients. Risk factors for the initiation of PH, its incidence, course and relation to some clinical and laboratory parameters were detected. Methods: One hundred SLE patients with/or without secondaryAPSwere examined during 3 years.Patients haddetailed clinical examination, laboratory tests, autoantibody screening and echocardiography. If the suspicion for PH was raised, blood pressure in pulmonary arteries has been evaluated by right heart catheterization. To eliminate a possible pulmonary interstitial damage further radiographic and functional investigation were carried out. Patients with PH were then followed up and their case reports are discussed. In patients who died pathological changes of pulmonary arteries were evaluated. Results: PH was diagnosed in 10 SLE patients on the basis of echocardiography. In 2 of these patients the echocardiography findings were not confirmed by right heart catheterization. All the patients were women. The mean age at the diagnosis of PH was 40 ± 10 years, the mean SLEDAI score was 9 ± 11. Five women with PH were antiphospholipid antibody positive, secondary APS was detected in 3 patients (38%). Positive test for anti-ds DNA occured in 6 patients (75%), Raynaud’s phenomenon was found in 2 patients (25%). Out of internal organ manifestations, 1 case of lupus nephritis (13%), 3 cases of pulmonary impairment (38%),and4 cases of neuropsychiatric manifestation (50%)were detected.Pulmonaryembolism occured in 3 patients (38%). Mild (PAP 30–40 mmHg) and severe PH (PAP over 50 mmHg) were observed in 4 patients each, moderate PH was not found. Four patients died, 3 of them had severe PH as well as secondary APS (75%) and pulmonary embolism occured in 2 patients (50%). Heart failure caused the death of all cases (100%). The mean time of survival since the PH detection was 1,3 years. Autopsy demonstrated pulmonary arteriole vasculopathy typical for PH (media intima andelastica proliferation, lumen obturation,mikrothrombosis, plexiformandangiomatosis lesions). Vasculitis was not seen. Conclusion: PH in our SLE patients occured exclusively in female in fertile age. The increased incidence of secondary APS, antiphospholipid antibodies, anti-ds DNA antibodies, pulmonary and neuropsychiatric manifestations were detected in PH patients. In the course of the first symptoms of dyspnoea, severe PH was already present in 80% of patients. Heart failure ensued. Treatment of SLE had no effect on already established PH. Lethal outcome was observed in 50% of patients with PH, from whom 50% had pulmonary embolism. A standard vasodilatation therapy with calcium-channel blockers had been used in all patients who died.

        Key words: systemic lupus erythematosus, pulmonary hypertension, vasculopathy, antiphospholipid antibodies, pulmonary embolism, organ manifestation, heart failure, therapy