Clinical case of delayed systemic sclerosis and pulmonary arterial hypertension diagnostics
DOI:
https://doi.org/10.14739/2310-1210.2019.2.161515Keywords:
pulmonary hypertension, systemic sclerosis, cardiac catheterizationAbstract
Pulmonary hypertension is a frequent and severe complication of systemic sclerosis (SSc). SSc-PAH patients have a worse prognosis than patients with idiopathic PAH. We report a case of a patient with delayed SSc and PAH diagnostics. Limited SSc was established in a 61-year-woman with 20 years of Raynaud’s syndrome anamnesis. But diagnosis of SSc-PAH was determined in severe and late symptomatic stage with signs of right heart congestion, NYHA III FC and mPAP 48 mmHg. This emphasizes the need for clinicians to be aware of limited SSc and have a high index of PH suspicion in such patients.
References
Chifflot, H., Fautrel, B., Sordet, C., Chatelus, E., & Sibilia, J. (2008) Incidence and Prevalence of Systemic Sclerosis: A systematic literature review. Semin Arthritis Rheum., 37(4), 223–35. doi: 10.1016/j.semarthrit.2007.05.003
Abu-Shakra, M., & Lee, P. (1995) Mortality in systemic sclerosis: a comparison with the general population. J. Rheumatol., 22(11), 2100–2102.
Hao, Y., Thakkar, V., Stevens, W., Morrisroe, K., Prior, D., Rabusa, C., et al. (2015) A comparison of the predictive accuracy of three screening models for pulmonary arterial hypertension in systemic sclerosis. Arthritis. Res. Ther., 17, 7. doi: 10.1186/s13075-015-0517-5
Muangchan, C., Baron, M., & Pope, J. (2013) The 15% rule in scleroderma: the frequency of severe organ complications in systemic sclerosis: A systematic review. J. Rheumatol., 40(9), 1545–56. doi: 10.3899/jrheum.121380
Fisher, M. R., Mathai, S. C., Champion, H. C., Girgis, R. E., Housten-Harris, T., Hummers, L., et al. (2006) Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis. Rheum., 54(9), 3043–3050. doi: 10.1002/art.22069
Kawut, S. M., Taichman, D. B., Archer-Chicko, C. L., Palevsky, H. I., Kimmel, S. E., et al. (2003) Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis. Chest, 123(2), 344–350. doi: 10.1378/chest.123.2.344
D’Alonzo, G. E., Barst, R. J., Ayres, S. M., Bergofsky, E. H., Brundage, B. H., Detre, K. M., et al. (1991) Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med., 115(5), 343–9.
Steen, V. D., Lucas, M., Fertig, N., & Medsger, Jr T. A. (2007) Pulmonary arterial hypertension and severe pulmonary fibrosis in systemic sclerosis patients with a nucleolar antibody. J. Rheumatol., 34, 2230–5.
Coghlan, J.G., Denton, C. P., Grünig, E., Bonderman, D., Distler, O., Khanna, D., et al. (2014) Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann. Rheum. Dis., 73(7), 1340–9. doi: 10.1136/annrheumdis-2013-203301
Chung, L., Domsic, R. T., Lingala, B., Alkassab, F., Bolster, M., Csuka, M. E., et al. (2014) Survival and predictors of mortality in systemic sclerosis-associated pulmonary arterial hypertension: outcomes from the pulmonary hypertension assessment and recognition of outcomes in scleroderma registry. Arthritis Care Res., 66(3), 489–95. doi: 10.1002/acr.22121
Semenov, V., Kuryata, O., & Lysunets, T. (2018) Clinical pattern of systemic sclerosis in Central Ukraine. Association between clinical manifestations of systemic sclerosis and hypertension. Reumatologia, 56(1), 24–30. doi: 10.5114/reum.2018.74745
Galiè, N., Humbert, M., Vachiery, J. L., Gibbs, S., Lang, I., Torbicki, A., et al. (2015) 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur. Respir. J., 46(4), 903–75. doi: 10.1183/13993003.01032-2015
Simonneau, G. (2018) PH haemodynamic definitions and clinical classifications and characteristics of specific PAH subgroups. Materials of 6 World Symposium on Pulmonary Hypertension. Nice.
Coghlan, J. G., Wolf, M., Distler, O., Denton, C. P., Doelberg, M., Harutyunova, S., et al. (2018) Incidence of pulmonary hypertension and determining factors in patients with systemic sclerosis. Eur. Respir. J., 51(4), pii: 1701197. doi: 10.1183/13993003.01197-2017
Valerio, C. J., Schreiber, B. E., Handler, C. E., Denton, C. P., & Coghlan, J. G. (2013) Borderline Mean Pulmonary Artery Pressure in Patients With Systemic Sclerosis: Transpulmonary Gradient Predicts Risk of Developing Pulmonary Hypertension. Arthritis and Rheumatology, 65(4), 1074–84. doi: 10.1002/art.37838
Fox, B. D., Shimony, A., Langleben, D., Hirsch, A., Rudski, L., Schlesinger, R., et al. (2013) .High prevalence of occult left heart disease in scleroderma-pulmonary hypertension. Eur. Respir. J., 42(4), 1083–91. doi: 10.1183/09031936.00091212
Avouac, J., Airò, P., Meune, C., Beretta, L., Dieude, P., Caramaschi, P., et al. (2010) Prevalence of pulmonary hypertension in systemic sclerosis in European Caucasians and metaanalysis of 5 studies. J. Rheumatol., 37(11), 2290–8. doi: 10.3899/jrheum.100245
Tedford, R. J., Hassoun, P. M., Mathai, S. C., Girgis, R. E., Russell, S. D., Thiemann, D. R., et al. (2012) Pulmonary capillary wedge pressure augments right ventricular pulsatile loading. Circulation., 125(2), 289–97. doi: 10.1161/CIRCULATIONAHA.111.051540
Dai, Z., & Matsui, Y. (2014) Pulmonary veno-occlusive disease: an 80-year-old mystery. Respiration., 88(2), 148–57. doi: 10.1159/000359973
Montani, D., Achouh, L., Dorfmüller, P., Le Pavec, J., Sztrymf, B., Tchérakian, C., et al. (2008) Pulmonary veno-occlusive disease: clinical, functional, radiologic, and hemodynamic characteristics and outcome of 24 cases confirmed by histology. Medicine (Baltimore), 87(4), 220–33. doi: 10.1097/MD.0b013e31818193bb
Eyries, M., Montani, D., Girerd, B., Perret, C., Leroy, A., Lonjou, C., et al. (2014) EIF2AK4 mutations cause pulmonary veno-occlusive disease, a recessive form of pulmonary hypertension. Nat Genet., 46(1), 65–9. doi: 10.1038/ng.2844
Lammi, M. R., Saketkoo, L. A., Gordon, J. K., & Steen, V. D. (2018) Changes in hemodynamic classification over time are common in systemic sclerosis-associated pulmonary hypertension: insights from the PHAROS cohort. Pulmonary Circulation., 8(2), 2045893218757404. doi: 10.1177/2045893218757404
Frost, A. E., Farber, H. W., Barst, R. J., Miller, D. P., Elliott, C. G., & McGoon, M. D. (2013) Demographics and outcomes of patients diagnosed with pulmonary hypertension with pulmonary capillary wedge pressures 16 to 18 mm Hg: insights from the REVEAL Registry. Chest., 143(1), 185–195. doi: 10.1378/chest.11-1387
Ciurzynski, M. B. P., Bienias, P., Lichodziejewska, B., Szewczyk, A., Glińska-Wielochowska, M., Jankowski, K., et al. (2008) Assessment of left and right ventricular diastolic function in patients with systemic sclerosis. Kardiol. Pol., 66, 269–276.
Lazar, J. M., Flores, A. R., Grandis, D. J., Orie, J. E., & Schulman, D. S. (1993) Effects of chronic right ventricular pressure overload on left ventricular diastolic function. Am J Cardiol., 72(15), 1179–82. https://doi.org/10.1016/0002-9149(93)90990-T
Rubenfire, M., Huffman, M. D., Krishnan, S., Seibold, J. R., Schiopu, E., & McLaughlin, V. V. (2013) Survival in systemic sclerosis with pulmonary arterial hypertension has not improved in the modern era. Chest., 144(4), 1282–1290. doi: 10.1378/chest.12-0653
DETECT - DETECTion of PAH in SSC. Retrieve from http://detect-pah.com.
Thibodeau, J. T., Turer, A. T., Gualano, S. K., Ayers, C. R., Velez-Martinez, M., Mishkin, J. D., et al. (2014) Characterization of a novel symptom of advanced heart failure: bendopnea. JACC Heart Fail., 2(1), 24–31. doi: 10.1016/j.jchf.2013.07.009
Brandon, N., & Mehra, M. R. (2013) «Flexo-dyspnea»: A novel clinical observation in the heart failure syndrome. J Heart Lung Transplant., 32(8), 844–5. doi: 10.1016/j.healun.2013.06.006
van den Hoogen, F., Khanna, D., Fransen, J., Johnson, S. R., Baron, M., Tyndall, A., et al. (2013) 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis., 72(11), 1747–55. doi: 10.1136/annrheumdis-2013-204424
Downloads
How to Cite
Issue
Section
License
Authors who publish with this journal agree to the following terms:- Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
- Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.
- Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work (See The Effect of Open Access)
