Skip to main content
NCBI home page
Annals of the Rheumatic Diseases logoLink to Annals of the Rheumatic Diseases
. 2003 Nov;62(11):1088–1093. doi: 10.1136/ard.62.11.1088

Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach

D Mukerjee 1, G St 1, B Coleiro 1, C Knight 1, C Denton 1, J Davar 1, C Black 1, J Coghlan 1
PMCID: PMC1754353  PMID: 14583573

Abstract

Objective: To determine the prevalence of systemic sclerosis associated pulmonary arterial hypertension (SScPAH), evaluate outcome, and identify predictors of mortality in a large patient cohort.

Methods: A prospective four year follow up study of 794 patients (722 from our own unit and 72 referrals). All patients screened for PAH using a combination of echocardiography, lung function testing, and clinical assessment. Patients with suspected raised pulmonary artery systolic pressures of >35 mm Hg, carbon monoxide transfer factor (TLCO) <50% predicted, or a precipitous fall in TLCO >20% over a one year period with no pulmonary fibrosis, and patients with SSc with breathlessness with no pulmonary fibrosis found were investigated with right heart catheterisation. All patients with SScPAH were treated in accordance with current best practice.

Results: The prevalence of PAH was 12% (89/722) by right heart catheter. The survival was 81%, 63%, and 56% at 1, 2, and 3 years from the diagnosis (in 89 patients from our own cohort and 59/72 referrals). Haemodynamic indices of right ventricular failure—raised mRAP (hazard ratio 21), raised mPAP (hazard ratio 20), and low CI (hazard ratio 11) predicted an adverse outcome There was no significant difference in survival between patients with SScPAH with (n=40) and without (n=108) pulmonary fibrosis (p=0.3).

Conclusions: The prevalence of SScPAH in this cohort was similar to that of other catheter based studies and lower than that of previous echo based studies. The 148 patients with SScPAH actively treated had comparable outcomes to those of the cohorts with primary pulmonary hypertension. A high mRAP was the strongest haemodynamic predictor of mortality. To improve prognosis, future treatments need to be implemented at an earlier disease stage to prevent right ventricular decompensation.

Full Text

The Full Text of this article is available as a PDF (324.5 KB).

Figure 1 .

Figure 1

Open in a new tab

Cumulative survivals of 148 patients with SScPAH between March 1998 and September 2002.

Figure 2 .

Figure 2

Open in a new tab

Kaplan-Meier survival curves for 148 patients with SScPAH divided into tertiles—47 with mPAP <32 mm Hg, 48 with mPAP = 32–44 mm Hg, and 53 with mPAP >45 mm Hg. There was a significant difference in mortality between the three groups (p<0.01 between each group).

Figure 3 .

Figure 3

Open in a new tab

Kaplan-Meier survival curves for 108 patients with isolated SScPAH and 40 patients with pulmonary fibrosis related SScPAH (p=0.3).

Figure 4 .

Figure 4

Open in a new tab

Survival according to degree of vasoreactivity to acute iloprost challenge in 129 patients with SScPAH divided into tertiles of PVR change (ΔPVR)—40 patients had a ΔPVR of <20%, 50 had a ΔPVR of 20–34%, and 49 had a ΔPVR of ⩾35%. There was no significant difference in survival between the three groups (p=0.8).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Alpert M. A., Pressly T. A., Mukerji V., Lambert C. R., Mukerji B., Panayiotou H., Sharp G. C. Acute and long-term effects of nifedipine on pulmonary and systemic hemodynamics in patients with pulmonary hypertension associated with diffuse systemic sclerosis, the CREST syndrome and mixed connective tissue disease. Am J Cardiol. 1991 Dec 15;68(17):1687–1691. doi: 10.1016/0002-9149(91)90330-n. [DOI] [PubMed] [Google Scholar]
  2. Badesch D. B., Tapson V. F., McGoon M. D., Brundage B. H., Rubin L. J., Wigley F. M., Rich S., Barst R. J., Barrett P. S., Kral K. M. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med. 2000 Mar 21;132(6):425–434. doi: 10.7326/0003-4819-132-6-200003210-00002. [DOI] [PubMed] [Google Scholar]
  3. Battle R. W., Davitt M. A., Cooper S. M., Buckley L. M., Leib E. S., Beglin P. A., Tischler M. D. Prevalence of pulmonary hypertension in limited and diffuse scleroderma. Chest. 1996 Dec;110(6):1515–1519. doi: 10.1378/chest.110.6.1515. [DOI] [PubMed] [Google Scholar]
  4. D'Alonzo G. E., Barst R. J., Ayres S. M., Bergofsky E. H., Brundage B. H., Detre K. M., Fishman A. P., Goldring R. M., Groves B. M., Kernis J. T. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med. 1991 Sep 1;115(5):343–349. doi: 10.7326/0003-4819-115-5-343. [DOI] [PubMed] [Google Scholar]
  5. Denton C. P., Cailes J. B., Phillips G. D., Wells A. U., Black C. M., Bois R. M. Comparison of Doppler echocardiography and right heart catheterization to assess pulmonary hypertension in systemic sclerosis. Br J Rheumatol. 1997 Feb;36(2):239–243. doi: 10.1093/rheumatology/36.2.239. [DOI] [PubMed] [Google Scholar]
  6. Dupuis J. Endothelin-receptor antagonists in pulmonary hypertension. Lancet. 2001 Oct 6;358(9288):1113–1114. doi: 10.1016/S0140-6736(01)06298-5. [DOI] [PubMed] [Google Scholar]
  7. Hinderliter A. L., Willis P. W., 4th, Barst R. J., Rich S., Rubin L. J., Badesch D. B., Groves B. M., McGoon M. D., Tapson V. F., Bourge R. C. Effects of long-term infusion of prostacyclin (epoprostenol) on echocardiographic measures of right ventricular structure and function in primary pulmonary hypertension. Primary Pulmonary Hypertension Study Group. Circulation. 1997 Mar 18;95(6):1479–1486. doi: 10.1161/01.cir.95.6.1479. [DOI] [PubMed] [Google Scholar]
  8. Homma A., Anzueto A., Peters J. I., Susanto I., Sako E., Zabalgoitia M., Bryan C. L., Levine S. M. Pulmonary artery systolic pressures estimated by echocardiogram vs cardiac catheterization in patients awaiting lung transplantation. J Heart Lung Transplant. 2001 Aug;20(8):833–839. doi: 10.1016/s1053-2498(01)00274-1. [DOI] [PubMed] [Google Scholar]
  9. LeRoy E. C., Black C., Fleischmajer R., Jablonska S., Krieg T., Medsger T. A., Jr, Rowell N., Wollheim F. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol. 1988 Feb;15(2):202–205. [PubMed] [Google Scholar]
  10. MacDonald S. L., Rubens M. B., Hansell D. M., Copley S. J., Desai S. R., du Bois R. M., Nicholson A. G., Colby T. V., Wells A. U. Nonspecific interstitial pneumonia and usual interstitial pneumonia: comparative appearances at and diagnostic accuracy of thin-section CT. Radiology. 2001 Dec;221(3):600–605. doi: 10.1148/radiol.2213010158. [DOI] [PubMed] [Google Scholar]
  11. MacGregor A. J., Canavan R., Knight C., Denton C. P., Davar J., Coghlan J., Black C. M. Pulmonary hypertension in systemic sclerosis: risk factors for progression and consequences for survival. Rheumatology (Oxford) 2001 Apr;40(4):453–459. doi: 10.1093/rheumatology/40.4.453. [DOI] [PubMed] [Google Scholar]
  12. McLaughlin V. V., Genthner D. E., Panella M. M., Rich S. Reduction in pulmonary vascular resistance with long-term epoprostenol (prostacyclin) therapy in primary pulmonary hypertension. N Engl J Med. 1998 Jan 29;338(5):273–277. doi: 10.1056/NEJM199801293380501. [DOI] [PubMed] [Google Scholar]
  13. McLaughlin Vallerie V., Shillington Alicia, Rich Stuart. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation. 2002 Sep 17;106(12):1477–1482. doi: 10.1161/01.cir.0000029100.82385.58. [DOI] [PubMed] [Google Scholar]
  14. Morelli S., Giordano M., De Marzio P., Priori R., Sgreccia A., Valesini G. Pulmonary arterial hypertension responsive to immunosuppressive therapy in systemic lupus erythematosus. Lupus. 1993 Dec;2(6):367–369. doi: 10.1177/096120339300200606. [DOI] [PubMed] [Google Scholar]
  15. Murata I., Kihara H., Shinohara S., Ito K. Echocardiographic evaluation of pulmonary arterial hypertension in patients with progressive systemic sclerosis and related syndromes. Jpn Circ J. 1992 Oct;56(10):983–991. doi: 10.1253/jcj.56.983. [DOI] [PubMed] [Google Scholar]
  16. Raffy O., Azarian R., Brenot F., Parent F., Sitbon O., Petitpretz P., Hervé P., Duroux P., Dinh-Xuan A. T., Simonneau G. Clinical significance of the pulmonary vasodilator response during short-term infusion of prostacyclin in primary pulmonary hypertension. Circulation. 1996 Feb 1;93(3):484–488. doi: 10.1161/01.cir.93.3.484. [DOI] [PubMed] [Google Scholar]
  17. Rich S., Dantzker D. R., Ayres S. M., Bergofsky E. H., Brundage B. H., Detre K. M., Fishman A. P., Goldring R. M., Groves B. M., Koerner S. K. Primary pulmonary hypertension. A national prospective study. Ann Intern Med. 1987 Aug;107(2):216–223. doi: 10.7326/0003-4819-107-2-216. [DOI] [PubMed] [Google Scholar]
  18. Rich S., Kaufmann E., Levy P. S. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N Engl J Med. 1992 Jul 9;327(2):76–81. doi: 10.1056/NEJM199207093270203. [DOI] [PubMed] [Google Scholar]
  19. Salerni R., Rodnan G. P., Leon D. F., Shaver J. A. Pulmonary hypertension in the CREST syndrome variant of progressive systemic sclerosis (scleroderma). Ann Intern Med. 1977 Apr;86(4):394–399. doi: 10.7326/0003-4819-86-4-394. [DOI] [PubMed] [Google Scholar]
  20. Sanchez O., Humbert M., Sitbon O., Simonneau G. Treatment of pulmonary hypertension secondary to connective tissue diseases. Thorax. 1999 Mar;54(3):273–277. doi: 10.1136/thx.54.3.273. [DOI] [PMC free article] [PubMed] [Google Scholar]
  21. Sandoval J., Bauerle O., Palomar A., Gómez A., Martínez-Guerra M. L., Beltrán M., Guerrero M. L. Survival in primary pulmonary hypertension. Validation of a prognostic equation. Circulation. 1994 Apr;89(4):1733–1744. doi: 10.1161/01.cir.89.4.1733. [DOI] [PubMed] [Google Scholar]
  22. Stupi A. M., Steen V. D., Owens G. R., Barnes E. L., Rodnan G. P., Medsger T. A., Jr Pulmonary hypertension in the CREST syndrome variant of systemic sclerosis. Arthritis Rheum. 1986 Apr;29(4):515–524. doi: 10.1002/art.1780290409. [DOI] [PubMed] [Google Scholar]
  23. Ungerer R. G., Tashkin D. P., Furst D., Clements P. J., Gong H., Jr, Bein M., Smith J. W., Roberts N., Cabeen W. Prevalence and clinical correlates of pulmonary arterial hypertension in progressive systemic sclerosis. Am J Med. 1983 Jul;75(1):65–74. doi: 10.1016/0002-9343(83)91169-5. [DOI] [PubMed] [Google Scholar]

Articles from Annals of the Rheumatic Diseases are provided here courtesy of BMJ Publishing Group

RESOURCES