Abstract
Our case series describes three children who were initially diagnosed as having severe pulmonary arterial hypertension (PAH) and subsequently found to be positive for specific autoantibodies suggestive of an underlying autoimmune process. The signs and symptoms of PAH are subtle and may be part of the initial presentation of childhood connective tissue disease (CTD). Evaluation for connective tissue disease in the newly diagnosed pulmonary hypertension (PH) patient is important because early diagnosis of PH as well as CTD is crucial in the successful management of these complex patients. Ongoing monitoring for CTD in patients with severe PAH is warranted.
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References
Badesch DB, Tapson VF, McGoon MD, Brundage BH, Rubin LJ, Wigley FM et al (2000) Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease: A randomized, controlled trial. Ann Intern Med 132:425–434
Burdt MA, Hoffman RW, Deutscher SL, Wang GS, Johnson JC, Sharp GC (1999) Long-term outcome in mixed connective tissue disease: longitudinal clinical and serologic findings. Arthritis Rheum 42:899–909
Galie` N, Manes A, Branzi A (2004) The endothelin system in pulmonary arterial hypertension. Cardiovasc Res 61:227–237
Hachulla E, Gressin V, Guillevin L, Carpentier P, Diot E, Sibilia J (2005) Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum 52:3792–3800
Jais X, Launay D, Yaici A, Le Pavec J, Tchérakian C, Sitbon O et al (2008) Immunosuppressive therapy in lupus- and mixed connective tissue disease-associated pulmonary arterial hypertension: a retrospective analysis of twenty-three cases. Arthritis Rheum 58:521–531
Mukerjee D, St George D, Coleiro B, Knight C, Denton CP, Davar J et al (2003) Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: Application of a registry approach. Ann Rheum Dis 62:1088–1093
Nashid M, Khanna PP, Furst DE, Clements PJ, Maranian P, Seibold J, et al. (2010) Gender and ethnicity differences in patients with diffuse systemic sclerosis-analysis from three large randomized clinical trials. Rheumatology [Epub ahead of print]
Robbins IM, Gaine SP, Schilz R, Tapson VF, Rubin LJ, Loyd JE (2000) Epoprostenol for treatment of pulmonary hypertension in patients with systemic lupus erythematosus. Chest 117:14–18
Rubin LJ, Badesch DB, Barst RJ, Galie N, Black CM, Keogh A et al (2003) Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 346:896–903
Simonneau G, Robbins IM, Beghetti M, Channick RN, Delcroix M, Denton CP et al (2009) Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 54:S43–S54
Steen VD (1996) Autoantibodies in systemic sclerosis. Bull Rheum Dis 45:6–8
Tanaka E, Harigai M, Tanaka M, Kawaguchi Y, Hara M, Kamatani N (2002) Pulmonary hypertension in systemic lupus erythematosus: evaluation of clinical characteristics and response to immunosuppressive treatment. J Rheumatol 29:282–287
Acknowledgment
This work was supported by the Jayden DeLuca Foundation and M01-RR00069 General Clinical Research Center, National Center for Research Resources, and National Institutes of Health.
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Takatsuki, S., Soep, J.B., Calderbank, M. et al. Connective Tissue Disease Presenting With Signs and Symptoms of Pulmonary Hypertension in Children. Pediatr Cardiol 32, 828–833 (2011). https://doi.org/10.1007/s00246-011-9950-7
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DOI: https://doi.org/10.1007/s00246-011-9950-7