PURPOSE: Pulmonary arterial hypertension (PAH) is increasingly recognized as a complication of Systemic Lupus Erythematosus (SLE), which usually carries a poor prognosis. The purpose of this study was to determine the prevalence and etiological mechanisms of anatomical signs of PAH in the necropsies of 90 patients died by SLE.
METHODS: A number of 3.400 complete autopsies carried out at Instituto Lanari between 1958 and 2006 were reviewed, in order to extract patients deceased with SLE diagnosis (criteria of American College of Rheumathology). Risk factors for PAH were studied.
RESULTS: Ninety patients were included; eight patients had evidence of PAH in the necropsy: four patients with PAH type I and four with Recurrent Thromboembolic Disease (RTED). Patients with RTED were older (42.13 + 2.25 vs. 31.27 + 1.38, p = 0.001) and had lower prevalence of lupic glomerulonephritis (50% vs. 90.243%, p = 0.010). Patients with PAH type I had longer evolution of SLE (100% vs. 27.906%, p = 0,011)and had higher prevalence of Raynaud phenomenon (75% vs. 19.767%, p =< 0.001). When patients with different types of PAH were compared, there were no clinical or biochemical differences between the two groups in any of the analyzed variables.
CONCLUSIONS: PAH in the setting of SLE is a rare but potentially life-threatening complication. Changes present in all our patients with unexplained PAH were consistent with the current category of pulmonary arteriopathy. Our patients with pulmonary arteriopathy were clinically not different from Chronic Thromboembolic Disease (CTEPH) patients except because they were younger and showed Raynaud phenomenon. Although it has been hypothetized that PAH is a kind of chronic Raynaud’s phenomenon, aided by autoimmune vascular inflammation; our results showed only structural damage closely similar to idiopathic pulmonary hypertension and absence of vasculitis.
CLINICAL IMPLICATIONS: Our data showed PAH is not exceptional in SLE patients. The presence of Raunaud phenomenon must alert of higher risk and encourage a more intensive search of PAH type I.
DISCLOSURE: The following authors have nothing to disclose: Lorena Delisio, Silvia Quadrelli, Luciana Molinari, Edgardo Sobrino
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