The frequency of pulmonary hypertension in patients with juvenile scleroderma
Juvenile scleroderma (JS) represents a rarely seen group of connective tissue diseases with multiple organ involvement. Cardiac involvement in JSS is well known and, although rare in children, it may be an important cause of mortality and morbidity. Therefore, an early determination of cardio-vascular and pulmonary involvement is of the most relevance to reduce the mortality in patients with juvenile scleroderma. The aim of the study was to explore the non-invasive methods (Doppler echocardiography, pulmonary function tests), Forced vital capacity (FVC) and Carbon monoxide diffusion capacity (DLCO) in the assessment of the cardiopulmonary involvement in patients with JS. The assessment of pulmonary arterial pressure (PAP) and risk factors for pulmonary arterial hypertension (PAH) were made by the measurement of maximum tricuspid insufficiency (TI), end-diastolic pulmonary insufficiency (PI), ratio of acceleration time (AT) to ejection time (ET) (AT/ET), right atrial pressure (RAP) and contraction of vena cava inferior during inspiration. Thirty-five patients with confirmed JS were included in the study. The mean age of onset of the disease was 9.57 years (median 10 years, range 2-18 years). The mean disease duration and follow-up time was 2 years (median 1 year, range 0.5-8 years) and 3.57 years (median 2 years, range 0.5-14.5 years), respectively.The values of all the analyzed parameters including TI, PI, AT/ET, PAP, FVC and DLCO were found to be within normal ranges in all the patients tested, confirming an uncommonness of cardiopulmonary involvement in patients with juvenile scleroderma.
Foeldvari I. Update on juvenile systemic sclerosis. Curr Rheumatol Rep 2015;17(3):17-18. Doi: http://dx.doi.org/10.1007/s11926-014-0491-y.
Martini G, Foeldvari I, Russo R, Cuttica R, Eberhard A, Ravelli A, et al. Systemic Sclerosis in Childhood. Clinical and Immunologic Features of 153 Patients in an International Database. Arthritis & Rheumatism 2006;54(12):3971-3978. Doi: http://dx.doi.org/10.1002/art.22207.
Zulian F, Cuffaro G, Spretto F. Scleroderma in children: an update. Curr Opin Rheumatol 2013;25(5):643-650. Doi: http://dx.doi.org/10.1097/BOR.0b013e3283641f61.
Borowiec A, Dabrowski R, Wozniak J, Jasek S, Chwyczko T, Kowalik I, et al. Cardiovascular assessment of asymptomatic patients with juvenile-onset localized and systemic scleroderma: 10 years prospective observation. Scand J Rheumatol 2012;41(1):33-38. Doi: http://dx.doi.org/10.3109/03009742.2011.609489.
Fischer A, Bull TM, Steen VD. Practical Approach to Screening for Scleroderma-Associated Pulmonary Arterial Hypertension. Arthritis Care & Research 2012;64(3):303-310. Doi: http://dx.doi.org/10.1002/acr.20693.
Coghlan JG, Handler C. Connective tissue associated pulmonary arterial hypertension. Lupus 2006;15(3):138-142 .Doi: http://dx.doi.org/10.1191/0961203306lu2280rr.
Bossone E1, D'Andrea A, D'Alto M, Citro R, Argiento P, Ferrara F, et al. Echocardiography in pulmonary arterial hypertension: from diagnosis to prognosis. J Am Soc Echocardiogr 2013;26(1):1-14. Doi: http://dx.doi.org/10.1016/j.echo.2012.10.009.
Mukerjee D, St George D, Coleiro B, Knight C, Denton CP, Davar J, et al. Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis 2003;62(11):1088-1093. Doi: http://dx.doi.org/10.1136/ard.62.11.1088.
Van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, et al. 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Arthritis Rheum 2013;65(11):2737-2747. Doi: http://dx.doi.org/10.1002/art.38098.
Fischer A, Bull TM, Steen VD. Practical Approach to Screening for Scleroderma-Associated Pulmonary Arterial Hypertension. Arthritis Care Res 2012;64(3):303-310. Doi: http://dx.doi.org/10.1002/acr.20693.
Mukerjee D, St George D, Knight C, Davar J, Wells AU, Du Bois RM. Echocardiography and pulmonary function as screening tests for pulmonary arterial hypertension in systemic sclerosis. Rheumatology 2004;43(4):461-466. Doi: http://dx.doi.org/10.1093/rheumatology/keh067.
Shah AA, Chung SE, Wigley FM, Wise RA, Hummers LK. Changed in estimated right ventricular systolic pressure predict mortality and pulmonary hypertension in a cohort of scleroderma patients. Ann Rheum Dis 2013;72(7):1136-1140. Doi: http://dx.doi.org/10.1136/annrheumdis-2012-201861.
Armstrong WF, Ryan T. Feigenbaum’s Echocardiography, seventh ed. Philadelphia: Lippincott, Williams & Wilkins; 2003.
Litwin SE. Noninvasive assessment of pulmonary artery pressures. Moving beyond tricuspid regurgitation velocities. Circ Cardiovasc Imaging 2010;3(2):132-133. Doi: http://dx.doi.org/10.1161/CIRCIMAGING.110.945121.
Sweiss NJ, Hushaw L, Thenappan T, Sawaqed R, Machado RF, Patel AR, et al. Diagnosis and management of pulmonary hypertension in systemic sclerosis. Curr Rheumatol Rep 2010;12(1):8-18. Doi: http://dx.doi.org/10.1007/s11926-009-0078-1.
Martini G, Vittadello F, Kasapçopur O, Magni MS, Corona F, Duarte-Salazar C, et al. Factors affecting survival in juvenile systemic sclerosis. Rheumatology (Oxford) 2009;48(2):119-122. Doi: http://dx.doi.org/10.1093/rheumatology/ken388.
Misra R, Singh G, Aggarwal P, Aggarwal A. Juvenile onset systemic sclerosis: a single center experience of 23 cases from Asia. Clin Rheumatol 2007;26(8):1259-1262.Doi: http://dx.doi.org/10.1007/s10067-006-0483-z.
Valerio CJ, Schreiber BE, Handler CE, Denton CP, Coghlan JG. Borderline mean pulmonary artery pressure in systemic sclerosis patients: trans-pulmonary gradient predicts risk of developing pulmonary hypertension. Arthritis Rheum 2013;65(4):1074-1084. Doi: http://dx.doi.org/10.1002/art.37838.
Quartier P, Bonnet D, Fournet JC, Bodemer C, Acar P, Ouachée-Chardin M, et al. Severe cardiac involvement in children with systemic sclerosis and myositis. J Rheumatol 2002;29(8):1767-1773.
Foeldvari I. New developments in juvenile systemic and localized scleroderma. Rheum Dis Clin North Am 2013;39(4):905-920. Doi: http://dx.doi.org/10.1016/j.rdc.2013.05.003.
Fraisse A, Jais X, Schleich JM, di Filippo S, Maragnes P, Beghetti M, et al. Characteristics and prospective 2-year-follow –up of children with pulmonary arterial hypertension in France. Arch Cardiovasc Dis 2010;103(2):66-74. Doi: http://dx.doi.org/10.1016/j.acvd.2009.12.001.
Langleben D, Orfanos SE, Givinazzo M, Hirsch A, Baron M, Senécal JL, et al. Pulmonary capillary endothelial metabolic dysfunction: Severity in pulmonary arterial hypertension related to connective tissue disease versus idiopathic pulmonary arterial hypertension. Arthritis Rheum 2008;58(4):1156-1164. Doi: http://dx.doi.org/10.1002/art.23405.
Le Pavec J, Humbert M, Mouthon L, Hasooun PM. Systemic sclerosis associated pulmonary hypertension. Am J Respir Crit Care Med 2010;181(12):1285-1293. Doi: http://dx.doi.org/10.1164/rccm.200909-1331PP.
© Association of Basic Medical Sciences of FBIH.