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Pulmonary hypertension in scleroderma and its relation to disease activity
Egyptian Rheumatology and Rehabilitation volume 40, pages 173–180 (2013)
The aim of this study was to screen for pulmonary hypertension (PH) in scleroderma patients using Doppler echocardiography, and correlate it with inflammatory parameters of the disease.
Patients and methods
Doppler echocardiography was performed in 39 patients with scleroderma (three men and 36 women) and was compared with 20 age-matched and sex-matched healthy controls. Fifteen (38.5%) patients had limited scleroderma and 24 (61.5%) patients had diffuse scleroderma. PH was diagnosed if the peak systolic pressure gradient at the tricuspid valve was more than 25 mmHg. All patients were subjected to full assessment of history, rheumatological examination, laboratory studies, chest radiography, and pulmonary function tests.
In 39 scleroderma patients, PH was detected in five patients (12.8%); four of them had limited scleroderma and one had diffuse scleroderma. The range of pulmonary artery systolic pressure was 37–63 mmHg (mean ± SD = 49.2 ± 10.1). There was a highly statistically significant difference between patients and controls (P< 0.0001). There were no significant differences between patients with and without PH in clinical features, except for arthritis and Raynaud’s phenomena (P < 0.04, 0.01). Significant differences in the level of erythrocyte sedimentation rate, complement, and anticentromere antibodies were found in patients with PH versus those without PH (P < 0.04, <0.03, <0.002, respectively). There was a statistically significant correlation between pulmonary artery pressure with disease duration (P < 0.001) and Valentini Disease Activity Index (P< 0.001).
Patients with scleroderma have an increased risk of pulmonary arterial hypertension. Echocardiography should be used as a screening tool in patients who are at a higher risk of developing PH.
Jimenez SA, Derk CT. Following the molecular pathways toward an understanding of the pathogenesis of systemic sclerosis. Ann Intern Med 2004; 140:37–50.
Tan FK Systemic sclerosis: the susceptible host (genetics and environment). Rheum Dis Clin North Am 2003; 29:211–237.
Walker UA, Tyndall A, Czirják L, Denton C, Farge-Bancel D, Kowal-Bielecka O, et al. Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group database. Ann Rheum Dis 2007; 66:754–763.
Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972–2002. Ann Rheum Dis 2007; 66:940–944.
Mayes MD, Lacey JV Jr, Beebe-Dimmer J, Gillespie BW, Cooper B, Laing TJ, et al. Prevalence, incidence, survival and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum 2003; 48:2246–2255.
Matsuura E, Ohta A, Kanegae F, Haruda Y, Ushiyama O, Koarada S, et al. Frequency and analysis of factors closely associated with the development of depressive symptoms in patients with scleroderma. J Rheumatol 2003; 30:1782–1787.
Danieli E, Airò P, Bettoni L, Cinquini M, Antonioli CM, Cavazzana I, et al. Health-related quality of life measured by the Short Form 36 (SF-36) in systemic sclerosis: correlations with indexes of disease activity and severity, disability and depressive symptoms. Clin Rheumatol 2005; 24:48–54.
Le Pavec J, Humbert M, Mouthon L, Hassoun PM. Systemic sclerosis-associated pulmonary arterial hypertension. Am J Respir Crit Care Med 2010; 181:1285–1293.
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; 12:8–18.
Simonneau G, Robbins IM, Beghetti M, Channick RN, Delcroix M, Denton CP, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009; 54 (Suppl 1): S43–S54.
Fisher MR, Mathai SC, Champion HC, Girgis RE, Housten-Harris T, Hummers L, et al. Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum 2006; 54:3043–3050.
Hachulla E, Carpentier P, Gressin V, Diot E, Allanore Y, Sibilia J, et al. Risk factors for death and the 3-year survival of patients with systemic sclerosis: the French ItinérAIR-Sclérodermie study. Rheumatology 2009; 48:304–308.
Valentini G, Silman AJ, Veale D. Assessment of disease activity. Clin Exp Rheumatol 2003; 21 (Suppl 29): S39–S41.
Bombardieri S, Medsger TA Jr, Silman AJ, Valentini G. The assessment of the patient with systemic sclerosis. Introduction. Clin Exp Rheumatol 2003; 21 (Suppl 29): S2–S4.
Yock PG, Popp RL. Noninvasive estimation of right ventricular systolic pressure by Doppler ultrasound in patients with tricuspid regurgitation. Circulation 1984; 70:657–662.
Currie PJ, Seward JB, Chan KL, Fyfe DA, Hagler DJ, Mair DD, et al. Continuous wave Doppler determination of right ventricular pressure: a simultaneous Doppler-catheterization study in 127 patients. J Am Coll Cardiol 1985;6:750–756.
SPSS Inc. . Statistical package for social sciences incorporation for windows. Version 11th ed. Chicago, IL: SPSS Inc.; 2001.
Wigley FM. Vascular disease in scleroderma. Clin Rev Allergy Immunol 2009; 36:150–175.
McGoon M, Gutterman D, Steen V, Barst R, McCrory DC, Fortin TA, et al. Screening, early detection and diagnosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004; 126 (Suppl): 14S–34S.
Hachulla E, Gressin V, Guillevin L, Carpentier P, Diot E, Sibilia J, et al. Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum 2005; 52:3792–3800.
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;62:1088–1093.
Gaine SP, Rubin LJ. Primary pulmonary hypertension. Lancet 1998; 352:719–725.
Humbert M, Sitbon O, Chaouat A, Bertocchi M, Habib G, Gressin V, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med 2006; 173:1023–1030.
Mayes MD. Scleroderma epidemiology. Rheum Dis Clin North Am 2003; 29:239–254.
Thenappan T, Shah SJ, Rich S, Gomberg-Maitland M. A USA-based registry for pulmonary arterial hypertension: 1982–2006. Eur Respir J 2007;30:1103–1110.
Naslund MJ, Pearson TA, Ritter JM. A documented episode of pulmonary vasoconstriction in systemic sclerosis. Johns Hopkins Med J 1981;148:78–80.
Chang B, Schachna L, White B, Wigley FM, Wise RA. Natural history of mild–moderate pulmonary hypertension and the risk factors for severe pulmonary hypertension in scleroderma. J Rheumatol 2006; 33:269–274.
Cox SR, Walker JG, Coleman M, Rischmueller M, Proudman S, Smith MD, et al. Isolated pulmonary hypertension in scleroderma. Intern Med J 2005; 35:28–33.
Steen V. Predictors of end stage lung disease in systemic sclerosis. Ann Rheum Dis 2003; 62:97–99.
Plastiras SC, Karadimitrakis SP, Kampolis C, Moutsopoulos HM, Tzelepis GE. Determinants of pulmonary arterial hypertension in scleroderma. Semin Arthritis Rheum 2007;36:392–396.
Ong YY, Nikoloutsopoulos T, Bond CP, Smith MD, Ahern MJ, Roberts-Thomson PJ. Decreased nailfold capillary density in limited scleroderma with pulmonary hypertension. Asian Pac J Allergy Immunol 1998; 16 (2–3): 81–86.
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Elshereef, R.R., Hassan, A.A., Darwish, A.F. et al. Pulmonary hypertension in scleroderma and its relation to disease activity. Egypt Rheumatol Rehabil 40, 173–180 (2013). https://doi.org/10.4103/1110-161X.123789
- pulmonary hypertension