Pulmonary arterial hypertension (PAH), which occurs in about 15% of patients with systemic sclerosis (SSc), is a progressive vasculopathy and despite modern therapeutic options is still one of the leading causes of death in these patients. We presented a patient diagnosed with the overlap syndrome (systemic sclerosis and rheumatoid arthritis) with a predominance of the clinical picture of systemic sclerosis (SSc), established in November 2018. She was initially treated by a rheumatologist with an antimalarial, which was soon discontinued by an ophthalmologist, followed by azathioprine which was excluded due to an allergic reaction. She has been continuously on corticosteroid therapy, and since January 2020, mycophenolate mofetil has been added to treatment. The patient was diagnosed with primary biliary cirrhosis by a gastroenterologist after clinical findings and additional examination methods; also, pulmonary fibrosis was diagnosed by a pulmonologist. In January 2020, deterioration of echocardiographic findings was registered (dilated right heart cavity, right ventricular systolic pressure (RVSP) 72 mmHg, tricuspid regurgitation 3+). Sildenafil was proposed by a responsible cardiologist for therapy that was not approved by gastroenterologist. Due to worsening of her symptoms in the form of pronounced fatigue, shortness of breath, in August 2020, a cardiologist of the Institute "Niška Banja" started bosentan therapy in a dose of 2 x 62.5 mg per day. After the applied therapy, the patient had a subjective improvement and reduction of symptoms. In November 2020, a control echocardiographic examination registered a decrease in RVSP to 55 mmHg. In addition to the therapy proposed by the responsible rheumatologist (mycophenolate mofetil 2 g daily, prednisolone 15 - 20 mg daily), the therapy prescribed by her cardiologist was also continued (bosentan 62.5 mg 2 x 1), with regular controls and monitoring of laboratory analyses. PAH in patients with SSc has a worse prognosis than idiopathic PAH, and additionally depends on RVSP and functional class. The process of treating PAH in patients with SSc requires a complex strategy that includes initial assessment of disease severity and subsequent responses to the therapy.
References
1.
LeRoy EC, Black C, Fleischmajer R. Scleroderma (systemic sclerosis. Classification, subsets and pathogenesis J Rheumatol. 1988;15:202–5.
2.
Kato M, Atsumi T. Pulmonary arterial hypertension associated with connective tissue diseases: A review focusing on distinctive clinical aspects. European Journal of Clinical Investigation. 2018;48(2).
3.
Coghlan JG, Denton CP, Grünig E, Bonderman D, Distler O, Khanna D, et al. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Annals of the Rheumatic Diseases. 2014;73(7):1340–9.
4.
Aithala R, Alex AG, Danda D. Pulmonary hypertension in connective tissue diseases: an update. International Journal of Rheumatic Diseases. 2017;20(1):5–24.
5.
Denton CP, Pope JE, Peter HH, Gabrielli A, Boonstra A, van den Hoogen FHJ, et al. Long-term effects of bosentan on quality of life, survival, safety and tolerability in pulmonary arterial hypertension related to connective tissue diseases. Annals of the Rheumatic Diseases. 2008;67(9):1222–8.
6.
Le Pavec J, Humbert M, Mouthon L, Hassoun PM. Systemic Sclerosis-associated Pulmonary Arterial Hypertension. American Journal of Respiratory and Critical Care Medicine. 2010;181(12):1285–93.
7.
Šarić S, Stamenković B, Aleksić I. Značaj evaluacije poremećaja plućne funkcije u sistemskoj sklerozi-prikaz slučaja. Hronični artritisi 2016. Balneoclimatologia. 2016;40(1):221–7.
8.
Ilić MD. Ehokardiografija u dijagnostici plućne hipertenzije. Balneoclimatologia. 2017;41(1):229–38.
9.
Damjanov N. Sistemska skleroza: klinički oblici i rana dijagnoza bolesti. Acta Rheum Belgrad. 2005:2 131-5.
10.
Aleksić I, Stamenković B, Milenković S. Artritis u sistemskoj sklerozi -prikaz slučaja Hronični artritisi 2016. Balneoclimatologia. 2016;40(1):213–9.
11.
Stamenković B. Najnovije EULAR preporuke za lečenje sistemske skleroze. Zapaljenske reumatske bolesti. Balneoclimatologia. 2018;42(1):125–30.
12.
Jones DA, Benjamin CW, Linseman DA. Activation of thromboxane and prostacyclin receptors elicits opposing effects on vascular smooth muscle cell growth and mitogen-activated protein kinase signaling cascades. Mol Pharmacol. 1995;48:890–6.
13.
Galiè N, Manes A, Branzi A. Prostanoids for Pulmonary Arterial Hypertension. American Journal of Respiratory Medicine. 2003;2(2):123–37.
14.
Taichman DB, Ornelas J, Chung L, Klinger JR, Lewis S, Mandel J, et al. Pharmacologic Therapy for Pulmonary Arterial Hypertension in Adults. Chest. 2014;146(2):449–75.
15.
Sun XG, Hansen JE, Oudiz RJ, Wasserman K. Pulmonary function in primary pulmonary hypertension. Journal of the American College of Cardiology. 2003;41(6):1028–35.
16.
Humbert M, Galiè N, McLaughlin VV, Rubin LJ, Simonneau G. An insider view on the World Symposium on Pulmonary Hypertension. The Lancet Respiratory Medicine. 2019;7(6):484–5.
17.
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. European Heart Journal. 2016;37(1):67–119.
18.
Hinderliter AL, Willis PW, Long W, Clarke WR, Ralph D, Caldwell EJ, et al. Frequency and prognostic significance of pericardial effusion in primary pulmonary hypertension. The American Journal of Cardiology. 1999;84(4):481–4.
19.
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. Journal of the American College of Cardiology. 1985;6(4):750–6.
20.
Berger M, Haimowitz A, Van Tosh A, Berdoff RL, Goldberg E. Quantitative assessment of pulmonary hypertension in patients with tricuspid regurgitation using continuous wave doppler ultrasound. Journal of the American College of Cardiology. 1985;6(2):359–65.
The statements, opinions and data contained in the journal are solely those of the individual authors and contributors and not of the publisher and the editor(s). We stay neutral with regard to jurisdictional claims in published maps and institutional affiliations.
,
