From Sjogrens’ Syndrome to Systemic Scleroderma: A Clinical Case of Disease Manifestation with Severe Pulmonary Arterial Hypertension

Timely recognition and treatment of pulmonary arterial hypertension, a dangerous complication of systemic connective tissue diseases, is extremely important, as these patients have a poor prognosis.

In this article, cardiologists present a clinical case of a diagnostic search for the cause of severe pulmonary arterial hypertension of high functional class and pericardial effusion in a 60-year-old female patient with suspected Sjögren’s syndrome. An assessment of the clinical status and autoimmune markers was performed, echocardiography, computed tomography and magnetic resonance imaging, coronary angiography and right heart catheterization, ophthalmological testing, as well as biopsy, ultrasound examination and salivary gland scintigraphy were performed. Subsequently, Raynaud’s syndrome and scleredema, as well as specific immunological disorders, were verified, which made it possible to diagnose a limited form of systemic scleroderma, prescribe immunosuppressive therapy, and establish the secondary nature of Sjögren’s syndrome.

The diagnostic results, supported by the opinions of the supervising doctors, can be discussed by the medical community and are of practical use for cardiologists, who rarely encounter rheumatological diseases in their routine practice.

1. Sharma R, Chaudhari KS, Kurien BT, et al. Sjögren Syndrome without Focal Lymphocytic Infiltration of the Salivary Glands. J Rheumatol. 2020;47(3):394-399. doi: 10.3899/jrheum.181443

2. Kollert F, Fisher BA. Equal rights in autoimmunity: is Sjögren’s syndrome ever ‘secondary’? Rheumatology (Oxford). 2020;59(6):1218-1225. doi: 10.1093/rheumatology/keaa009

3. Felten R, Meyer A, Gottenberg JE. Non-primary Sjogren’s Syndrome: Secondary or associated? Joint Bone Spine. 2023;90(2):105502. doi: 10.1016/j.jbspin.2022.105502

4. Qin B, Wang J, Yang Z, et al. Epidemiology of primary Sjögren’s syndrome: a systematic review and meta-analysis. Ann Rheum Dis. 2015;74(11):1983-1989. doi: 10.1136/annrheumdis-2014-205375

5. Crawford JD, Wang H, Trejo-Zambrano D, et al. The XIST lncRNA is a sexspecific reservoir of TLR7 ligands in SLE. JCI Insight. 2023;8(20):e169344. Published 2023 Sep 21. doi: 10.1172/jci.insight.169344

6. Atzeni F, Gozza F, Cafaro G, Perricone C, Bartoloni E. Cardiovascular Involvement in Sjögren’s Syndrome. Front Immunol. 2022;13:879516. Published 2022 May 6. doi: 10.3389/fimmu.2022.879516

7. Le Goff M, Cornec D, Jousse-Joulin S, et al. Comparison of 2002 AECG and 2016 ACR/EULAR classification criteria and added value of salivary gland ultrasonography in a patient cohort with suspected primary Sjögren’s syndrome. Arthritis Res Ther. 2017;19(1):269. Published 2017 Dec 6. doi: 10.1186/s13075-017-1475-x

8. Seror R, Theander E, Brun JG, et al. Validation of EULAR primary Sjögren’s syndrome disease activity (ESSDAI) and patient indexes (ESSPRI). Ann Rheum Dis. 2015;74(5):859-866. doi: 10.1136/annrheumdis-2013-204615

9. Seror R, Chiche L, Beydon M, et al. Estimated prevalence, incidence and healthcare costs of Sjögren’s syndrome in France: a national claims-based study. RMD Open. 2024;10(1):e003591. Published 2024 Feb 2. doi: 10.1136/rmdopen-2023-003591

10. Xu D, Tian X, Zhang W, Zhang X, Liu B, Zhang F. Sjogren’s syndromeonset lupus patients have distinctive clinical manifestations and benign prognosis: a case-control study. Lupus. 2010;19(2):197-200. doi: 10.1177/0961203309348235

11. Denton CP, Khanna D. Systemic sclerosis. Lancet. 2017;390(10103):1685-1699. doi: 10.1016/S0140-6736(17)30933-9

12. Tuhy T, Hassoun PM. Clinical features of pulmonary arterial hypertension associated with systemic sclerosis. Front Med (Lausanne). 2023;10:1264906. Published 2023 Sep 27. doi: 10.3389/fmed.2023.1264906

13. Rose-Jones LJ, Mclaughlin VV. Pulmonary hypertension: types and treatments. Curr Cardiol Rev. 2015;11(1):73-79. doi: 10.2174/1573403x09666131117164122

14. Avouac J, Airò P, Meune C, et al. Prevalence of pulmonary hypertension in systemic sclerosis in European Caucasians and metaanalysis of 5 studies. J Rheumatol. 2010;37(11):2290-2298. doi: 10.3899/jrheum.100245

15. Hassan HJ, Naranjo M, Ayoub N, et al. Improved Survival for Patients with Systemic Sclerosis-associated Pulmonary Arterial Hypertension: The Johns Hopkins Registry. Am J Respir Crit Care Med. 2023;207(3):312-322. doi: 10.1164/rccm.202204-0731OC

16. Drosos AA, Pennec YL, Elisaf M, et al. Sjögren’s syndrome in patients with the CREST variant of progressive systemic scleroderma. J Rheumatol. 1991;18(11):1685-1688.

17. Oddis CV, Eisenbeis CH Jr, Reidbord HE, Steen VD, Medsger TA Jr. Vasculitis in systemic sclerosis: association with Sjögren’s syndrome and the CREST syndrome variant. J Rheumatol. 1987;14(5):942-948.