OBJECTIVE: To evaluate the clinical features, laboratory findings, diagnosis and treatment, and prognosis of children with systemic lupus erythematosus (SLE) accompanied by pulmonary hypertension (PH). METHODS: The clinical symptoms, laboratory findings, echocardiographic features, SLE disease activity index, and treatment outcome of 15 hospitalized children with SLE accompanied by PH were retrospectively analyzed. RESULTS: Among the 15 patients, the median interval from diagnosis of SLE to diagnosis of PH was 0.1 year (range: 0-6.5 years). Aside from PH-related symptoms, Raynaud's phenomenon was observed in 6 (40%) of the 15 patients. There was no significant difference in SLE disease activity (evaluated by complements 3 and 4 levels, erythrocyte sedimentation rate, and positive rate of anti-double-stranded DNA) between patients with mild-to-moderate PH and those with severe PH (P<0.05). As for treatment, 13 patients received immunosuppressive therapy with glucocorticoids, and among them 2 patients received PH-targeted therapy. During a median follow-up of 8.0 years (range: 0.5-18.1 years) since the diagnosis of PH, 2 deaths were noted with class III or IV cardiac function (World Health Organization), while the other patients were in a stable condition. CONCLUSIONS: Raynaud's phenomenon is a common clinical manifestation in children with SLE accompanied by pulmonary hypertension (PH). PH severity is not significantly associated with SLE disease activity, and thus greater focus should be placed upon early screening of pulmonary arterial pressure in SLE patients. Early diagnosis and early treatment can improve the prognosis of children with SLE.
OBJECTIVE: To evaluate the clinical features, laboratory findings, diagnosis and treatment, and prognosis of children with systemic lupus erythematosus (SLE) accompanied by pulmonary hypertension (PH). METHODS: The clinical symptoms, laboratory findings, echocardiographic features, SLE disease activity index, and treatment outcome of 15 hospitalized children with SLE accompanied by PH were retrospectively analyzed. RESULTS: Among the 15 patients, the median interval from diagnosis of SLE to diagnosis of PH was 0.1 year (range: 0-6.5 years). Aside from PH-related symptoms, Raynaud's phenomenon was observed in 6 (40%) of the 15 patients. There was no significant difference in SLE disease activity (evaluated by complements 3 and 4 levels, erythrocyte sedimentation rate, and positive rate of anti-double-stranded DNA) between patients with mild-to-moderate PH and those with severe PH (P<0.05). As for treatment, 13 patients received immunosuppressive therapy with glucocorticoids, and among them 2 patients received PH-targeted therapy. During a median follow-up of 8.0 years (range: 0.5-18.1 years) since the diagnosis of PH, 2 deaths were noted with class III or IV cardiac function (World Health Organization), while the other patients were in a stable condition. CONCLUSIONS: Raynaud's phenomenon is a common clinical manifestation in children with SLE accompanied by pulmonary hypertension (PH). PH severity is not significantly associated with SLE disease activity, and thus greater focus should be placed upon early screening of pulmonary arterial pressure in SLEpatients. Early diagnosis and early treatment can improve the prognosis of children with SLE.
Authors: Nazzareno Galiè; Marc Humbert; Jean-Luc Vachiery; Simon Gibbs; Irene Lang; Adam Torbicki; Gérald Simonneau; Andrew Peacock; Anton Vonk Noordegraaf; Maurice Beghetti; Ardeschir Ghofrani; Miguel Angel Gomez Sanchez; Georg Hansmann; Walter Klepetko; Patrizio Lancellotti; Marco Matucci; Theresa McDonagh; Luc A Pierard; Pedro T Trindade; Maurizio Zompatori; Marius Hoeper Journal: Rev Esp Cardiol (Engl Ed) Date: 2016-02
Authors: Lewis J Rubin; David B Badesch; Robyn J Barst; Nazzareno Galie; Carol M Black; Anne Keogh; Tomas Pulido; Adaani Frost; Sebastien Roux; Isabelle Leconte; Michael Landzberg; Gerald Simonneau Journal: N Engl J Med Date: 2002-03-21 Impact factor: 91.245
Authors: Laura C Price; S John Wort; Frédéric Perros; Peter Dorfmüller; Alice Huertas; David Montani; Sylvia Cohen-Kaminsky; Marc Humbert Journal: Chest Date: 2012-01 Impact factor: 9.410
Authors: N Galiè; Lj Rubin; Mm Hoeper; P Jansa; H Al-Hiti; Gmb Meyer; E Chiossi; A Kusic-Pajic; G Simonneau Journal: Lancet Date: 2008-06-21 Impact factor: 79.321