SYSTEMIC SCLEROSIS AND PULMONARY HYPERTENSION - CASE REPORT

Ivana Aleksić; Sandra Šarić; Bojan Ilić; Sonja Stojanović; Marina Deljanin llić

Ivana Aleksić Institut za lečenje i rehabilitaciju „Niška Banja“, Srpskih junaka br 2, Niska Banja
Sandra Šarić Institute for Treatment and Rehabilitation „Niška Banja“
Bojan Ilić Institute for Treatment and Rehabilitation „Niška Banja
Sonja Stojanović Institute for Treatment and Rehabilitation „Niška Banja“, Niška Banja, University of Niš, Faculty of Medicine, Niš, Serbia
Marina Deljanin llić Institute for Treatment and Rehabilitation „Niška Banja“, Niška Banja, University of Niš, Faculty of Medicine, Niš, Serbia

Keywords: systemic sclerosis, pulmonary hypertension, therapy

Abstract

Introduction: Pulmonary arterial hypertension(PAH) which occurs in about 15% of patients with SSc is a progressive vasculopathy and despite modern therapeutic options is still one of the leading causes of death in these patients.

Case report:In November 2018 we presented a patient who was diagnosed overlap syndrome(systemic sclerosis and rheumatoid arthritis)with a predominance of systemic sclerosis(SSc).She was initially treated by a rheumatologist with a synthetic antimalarial medication and azathioprine,with corticosteroid therapy.Since January 2020, mycophenolate mofetil was added to the battery of treatments. In January 2020,worsening of the echocardiographic findings was registered(dilated right heart cavity, RVSP72mmHg, tricuspid regurgitation 3+).Due to the worsening of her symptoms in the form of severe fatigue, a feeling of suffocation,in August 2020, a cardiologist started bosentan therapy in a dose of 2x62.5mg per day.After the applied therapy,the patient showed a subjective improvement and a reduction of symptoms.In November 2020, a control echocardiographic examination registered a decrease in right ventricular pressure-RVSP55mmHg. Along with the therapy prescribed by her rheumatologist (mycophenolate mofetil 2g daily, prednisolone 15-20mg daily), it was administered in tandem with the therapy of her cardiologist (bosentan 62.5mg 2x1)this regime included scheduled controls and regular monitoring of laboratory analyses.

Conclusion:Pulmonary hypertension in patients with systemic sclerosis has a worse prognosis than idiopathic PAH, and additionally it is also dependant upon right ventricular pressure and functional class. The process of treating PAH in patients with SSc requires a complex strategy that includes an initial assessment of the severity of the disease and consequential responses to the therapy.

References

Aithala R, Alex AG, Danda D. Pulmonary hypertension in connective tissue diseases: an update. Int J Rheum Dis. 2017 Jan;20(1):5-24.
Aleksić I, Stamenkovic B, Milenkovic S, Stojanović S, Zivkovic V. Artritis u sistemskoj sklerozi-prikaz slucaja. Hronični artritisi 2016. Balneoclimatologia 2016;40(1): 213-219.
Berger M., Haimowitz A., Van Tosh A., Berdoff R.L. and Goldberg E. : "Quantitative assessment of pulmonary hypertension in patients with tricuspid regurgitation using continuous wave Doppler ultrasound". J Am Coll Cardiol 1985; 6: 359.
Coghlan JG, Denton CP, Grunig E, et al, DETECT study group: Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: The DETECT study. Ann Rheum Dis 2014;73(7):1340-9.
Currie P.J., Seward J.B., Chan K.L.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.
Damjanov N. Sistemska skleroza: klinički oblici i rana dijagnoza bolesti. Acta Rheum Belgrad 2005 (2);131-135.
Denton CP, Pope JE, Peter HH, et al. (on behalf of the Tracleer Use in PAH associated with Scleroderma and Connective Tissue Diseases (TRUST) Investigators). Ann Rheum Dis 2008; 67: 1222-1228.
Galiè N, Manes A, Branzi A. Prostanoids for pulmonary arterial hypertension. Am J Respir Med 2003;2:123–137. ESC/ERS Guidelines, Downloaded from http://eurheartj.oxfordjournals. org/ by guest on November 10, 2016.
Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2016;37(1):67-119.
Hansen JE, Oudiz RJ,Wasserman K. Pulmonary function in primary pulmonary hypertension. J Am Coll Cardiol 2003;41:1028–1035.
Hinderliter A.L., Willis P.W., Long W.et al. : "Frequency and prognostic significance of pericardial effusion in primary pulmonary hypertension: PPH Study Group. Primary pulmonary hypertension". Am J Cardiol 1999; 84: 481.
Humbert M, Galiè N, McLaughlin VV, Rubin LJ, Simonneau G. An insider view on the World Symposium on Pulmonary Hypertension. Lancet Respir Med. 2019 Jun;7(6):484-485.
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: 890–896.
Kato M, Atsumi T. Pulmonary arterial hypertension associated with connective tissue diseases: A review focusing on distinctive clinical aspects. Eur J Clin Invest. 2018;48:e12876.
Le Pavec J, Humbert M, Mouthon L, et al. Systemic sclerosis-associated pulmonary arterial hypertension. Am J Respir Crit Care Med 2010;181(12):1285-1293.
LeRoy EC, Black C, Fleischmajer R, et al. Scleroderma (systemic sclerosis): Classification, subsets and pathogenesis. J Rheumatol 1988;15:202-205.
Marina Deljanin Ilić. Ehokardiografija u dijagnostici plućne hipertenzije. Balneoclimatologia 2017;41(1):229-238.
Stamenković B. Najnovije EULAR preporuke za lečenje sistemsk skleroze. Zapaljenske reumatske bolesti. Balneoclimatologia 2018;42(1): 125-130.
Šarić S, Stamenković B, Aleksić I, Mitić V, Stojanović D, Stoičkov V, Marinković D, Rihter M. Značaj evaluacije poremećaja plućne funkcije u sistemskoj sklerozi-prikaz slučaja. Hronični artritisi 2016. Balneoclimatologia 2016;40(1): 221-227.
Taichman DB, Ornelas J, Chung L, et al. Pharmacologic Therapy for Pulmonary Arterial Hypertension in Adults: CHEST Guideline and Expert Panel Report. Chest 2014;146(2):449-475.