Systemic Lupus Erythematosus-associated Pulmonary Arterial Hypertension: Serial Case Report
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Published:
Dec 14, 2021
Keywords:
connective tissue disease, pulmonary arterial hypertension, right heart failure, systemic lupus erythematosus
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Abstract
One of the causes of pulmonary arterial hypertension (PAH) is connective tissue disease, including systemic lupus erythematosus (SLE). The prevalence of PAH in patients with SLE according to cohort studies varies widely between 0.5% and 43%. In some cases, PAH is the initial manifestation leading to the diagnosis of SLE. However, PAH can develop as a complication in patients who are initially diagnosed with SLE. We report two cases with a different approach to the diagnosis of pulmonary hypertension in SLE patients. These cases emphasize the importance of investigating examination results according to the guidelines to establish the diagnosis of PAH in SLE.
Article Details
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Case Report
References
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2. Simonneau G, Gatzoulis MA, Adatia I, Celermajer D, Denton C, Ghofrani A, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25 SUPPL.).
3. Galiè N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2016;37(1):67–119.
4. McLaughlin V V., Shah SJ, Souza R, Humbert M. Management of pulmonary arterial hypertension. J Am Coll Cardiol. 2015;65(18):1976–97.
5. Tselios K, Gladman DD, Urowitz MB. Systemic lupus erythematosus and pulmonary arterial hypertension: Links, risks, and management strategies. Open Access Rheumatol Res Rev. 2017;9:1–9.
6. Rich JD, Rich S. Clinical diagnosis of pulmonary hypertension. Circulation. 2014;130(20):1820–30.
7. Dhala A. Pulmonary arterial hypertension in systemic lupus erythematosus: Current status and future direction. Clin Dev Immunol. 2012;2012.