The incidence and course of pulmonary hypertensionin systemic lupus erythematosus patients. Three years follow up outcome

Authors: D. Tegzová;  L. Kamenik 1;  L. Musilová;  J. Štolfa;  Š. Forejtová;  T. Soukup 2;  A. Dorazilová 3;  H. Al-Hiti 4;  C. Dostál
Authors‘ workplace: Revmatologický ústav, Praha, 1Nemocnice Milosrdných sester, Praha, 2II. interní klinika fakultní nemocnice UK, Hradec Králové, 3Hlavův patologicko-anatomický ústav 1. LF UK, Praha, 4Institut klinické a experimentální medicíny, Praha
Published in: Čes. Revmatol., , 2003, No. 3, p. 117-127.


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 andlaboratory parameters were detected. Methods: One hundred SLE patients with/or without secondaryAPSwereexamined during 3 years.Patients haddetailed clinical examination, laboratory tests,autoantibody screening and echocardiography. If the suspicion for PH was raised, blood pressurein pulmonary arteries has been evaluated by right heart catheterization. To eliminate a possiblepulmonary 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 diedpathological changes of pulmonary arteries were evaluated. Results: PH was diagnosed in 10 SLEpatients on the basis of echocardiography. In 2 of these patients the echocardiography findings werenot confirmed by right heart catheterization. All the patients were women. The mean age at thediagnosis of PH was 40 ± 10 years, the mean SLEDAI score was 9 ± 11. Five women with PH wereantiphospholipid 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.Pulmonaryembolismoccured in 3 patients (38%). Mild (PAP 30–40 mmHg) and severe PH (PAP over 50 mmHg) wereobserved in 4 patients each, moderate PH was not found. Four patients died, 3 of them had severePH as well as secondary APS (75%) and pulmonary embolism occured in 2 patients (50%). Heartfailure caused the death of all cases (100%). The mean time of survival since the PH detection was1,3 years. Autopsy demonstrated pulmonary arteriole vasculopathy typical for PH (media intimaandelastica proliferation, lumen obturation,mikrothrombosis, plexiformandangiomatosis lesions).Vasculitis was not seen. Conclusion: PH in our SLE patients occured exclusively in female in fertileage. The increased incidence of secondary APS, antiphospholipid antibodies, anti-ds DNA antibodies,pulmonary and neuropsychiatric manifestations were detected in PH patients. In the course ofthe first symptoms of dyspnoea, severe PH was already present in 80% of patients. Heart failureensued. Treatment of SLE had no effect on already established PH. Lethal outcome was observed in50% of patients with PH, from whom 50% had pulmonary embolism. A standard vasodilatationtherapy with calcium-channel blockers had been used in all patients who died.

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

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