Prevalence, treatment, and outcomes of coexistent pulmonary hypertension and interstitial lung disease in systemic sclerosis
Open accessAbstract
Objective: Systemic sclerosis ( SS c) is associated with interstitial lung disease ( ILD ) and pulmonary hypertension ( PH ). This study was undertaken to determine the prevalence, characteristics, treatment, and outcomes of PH in a cohort of patients with SS c‐associated ILD . Methods: Patients with SS c‐associated ILD on high‐resolution computed tomography ( HRCT ) were included in a prospective observational cohort. Patients were screened for PH based on a standardized screening algorithm and underwent right‐sided heart catheterization ( RHC ) if indicated. PH classification was based on hemodynamic findings and the extent of ILD on HRCT . Summary statistics and survival using the Kaplan‐Meier method were calculated. Results: Of the 93 patients with SS c‐associated ILD included in the study, 76% were women and 65.6% had diffuse cutaneous SS c. The mean age was 54.9 years, and the mean SS c disease duration was 8 years. Twenty‐nine patients (31.2%) had RHC ‐proven PH ; of those 29 patients, 24.1% had PAH , 55.2% had World Health Organization ( WHO ) Group III PH , 34.5% had WHO Group III PH with pulmonary vascular resistance >3.0 Wood units, 48.3% had a PH diagnosis within 7 years of SS c onset, 82.8% received therapy for ILD , and 82.8% received therapy for PAH . The survival rate 3 years after SS c‐associated ILD diagnosis for all patients was 97%. The survival rate 3 years after PH diagnosis for those with SS c‐associated ILD and PH was 91%. Conclusion: In a large cohort of patients with SS c‐associated ILD , a significant proportion of patients had coexisting PH , which often occurs early after SS c diagnosis. Most patients were treated with ILD and PAH therapies, and survival was good. Patients with SS c‐associated ILD should be evaluated for coexisting PH .
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Integrity signals
Registry
Cites
Statistics
Claims the paper makes
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
High-risk pulmonary arterial hypertension profiles include familial PAH, PVOD, CTD-PAH, CTD-PAH with concomitant interstitial lung disease, rapidly progressive disease despite therapy, and development of hemoptysis.
“Other high-risk profiles include familial PAH, PVOD, CTD-PAH, CTD-PAH associated with concomitant ILD, rapidly progressive disease despite therapy, and development of hemoptysis.(282, 291–293)”
From Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation. (2021) · cites this paper as [293]
The Discussion (page unavailable) reports poor survival in systemic-sclerosis–associated PAH and PH with ILD, with survival tending to be worse when PH is due to ILD. This supports the concern about concomitant ILD in this connective-tissue-disease population, but the paper does not establish the broader list of high-risk PAH profiles, including familial PAH, PVOD, progression despite therapy, or hemoptysis. (LLM, conf=0.85)
Experimental — accuracy not yet measured
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
The self-check needs the paper’s full text.
Cites
2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension
Galiè, N., Humbert, M., Vachiery, J.-L., Gibbs, S., Lang, I., Torbicki, A., Simonneau, G., Peacock, A., Vonk Noordegraaf, A., Beghetti, M., et al. (2016) · European Heart Journal · doi:10.1093/eurheartj/ehv317 · Cited by 6,931 · see citing works
Haemodynamic definitions and updated clinical classification of pulmonary hypertension
Simonneau, G., Montani, D., Celermajer, D. S., Denton, C. P., Gatzoulis, M. A., Krowka, M., Williams, P. G., & Souza, R. (2019) · European Respiratory Journal · doi:10.1183/13993003.01913-2018 · Cited by 3,813 · see citing works
van den Hoogen, F. H. J., Khanna, D., Fransen, J., Johnson, S. R., Baron, M. G., Tyndall, A., Matucci‐Cerinic, M., Naden, R. P., Medsger, T. A., Carreira, P. E., et al. (2013) · Arthritis & Rheumatism · doi:10.1002/art.38098 · Cited by 3,399 · see citing works
Self-citation2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension
Galiè, N., Humbert, M. J. C., Vachiéry, J., Gibbs, S., Lang, I. M., Torbicki, A., Simonneau, G., Peacock, A. J., Noordegraaf, A. V., Beghetti, M., et al. (2015) · European Respiratory Journal · doi:10.1183/13993003.01032-2015 · Cited by 3,158 · see citing works
Denton, C. P., & Khanna, D. (2017) · The Lancet · doi:10.1016/s0140-6736(17)30933-9 · Cited by 2,257 · see citing works
Self-citationCyclophosphamide versus placebo in scleroderma lung disease
Tashkin, D. P., Elashoff, R., Clements, P. J., Goldin, J., Roth, M. D., Furst, D. E., Arriola, E., Silver, R., Strange, C., Bolster, M., et al. (2006) · New England Journal of Medicine · doi:10.1056/nejmoa055120 · Cited by 1,677 · see citing works
Changes in causes of death in systemic sclerosis, 1972–2002
Steen, V., & Medsger, T. A. (2007) · Annals of the Rheumatic Diseases · doi:10.1136/ard.2006.066068 · Cited by 1,530 · see citing works
Interstitial Lung Disease in Systemic Sclerosis
Goh, N. S. L., Desai, S. R., Veeraraghavan, S., Hansell, D. M., Copley, S. J., Maher, T. M., Corte, T. J., Sander, C. R., Ratoff, J. C., Devaraj, A., et al. (2008) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200706-877oc · Cited by 1,229 · see citing works
Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension
Galiè, N., Barberà, J. A., Frost, A., Ghofrani, H. A., Hoeper, M. M., McLaughlin, V. V., Peacock, A. J., Simonneau, G., Vachiéry, J., Grünig, E., et al. (2015) · New England Journal of Medicine · doi:10.1056/nejmoa1413687 · Cited by 1,182 · see citing works
Self-citationTashkin, D. P., Roth, M. D., Clements, P. J., Furst, D. E., Khanna, D., Kleerup, E. C., Goldin, J. G., Arriola, E., Volkmann, E. R., Kafaja, S., et al. (2016) · The Lancet Respiratory Medicine · doi:10.1016/s2213-2600(16)30152-7 · Cited by 1,077 · see citing works
Self-citationSelexipag for the Treatment of Pulmonary Arterial Hypertension
Sitbon, O., Channick, R. N., Chin, K., Frey, A., Gaine, S., Galiè, N., Ghofrani, H. A., Hoeper, M. M., Lang, I. M., Preiss, R., et al. (2015) · New England Journal of Medicine · doi:10.1056/nejmoa1503184 · Cited by 1,062 · see citing works
Self-citationEvidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study
Coghlan, G., Denton, C. P., Grünig, E., Bonderman, D., Distler, O., Khanna, D., Müller‐Ladner, U., Pope, J. E., Vonk, M. C., Doelberg, M., et al. (2013) · Annals of the Rheumatic Diseases · doi:10.1136/annrheumdis-2013-203301 · Cited by 832 · see citing works
Self-citationPulmonary Hypertension in Chronic Lung Diseases
Seeger, W., Adir, Y., Barberà, J. A., Champion, H. C., Coghlan, G., Cottin, V., Marco, T. D., Galiè, N., Ghio, S., Gibbs, S., et al. (2013) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2013.10.036 · Cited by 678 · see citing works
Pulmonary Hypertension Due to Left Heart Diseases
Vachiéry, J., Adir, Y., Barberà, J. A., Champion, H. C., Coghlan, G., Cottin, V., Marco, T. D., Galiè, N., Ghio, S., Gibbs, J. S. R., et al. (2013) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2013.10.033 · Cited by 631 · see citing works
Severe restrictive lung disease in systemic sclerosis
Steen, V. D., Conte, C., Owens, G. R., et al. (1994) · Arthritis & Rheumatism · doi:10.1002/art.1780370903 · Cited by 528 · see citing works
Diagnosis of pulmonary hypertension
Frost, A., Badesch, D. B., Gibbs, J. S. R., Gopalan, D., Khanna, D., Manes, A., Oudiz, R. J., Satoh, T., Torres, F., & Torbicki, A. (2018) · European Respiratory Journal · doi:10.1183/13993003.01904-2018 · Cited by 482 · see citing works
Self-citationPrediction of Pulmonary Complications and Long‐Term Survival in Systemic Sclerosis
Nihtyanova, S. I., Schreiber, B. E., Ong, V. H., Rosenberg, D., Moinzadeh, P., Coghlan, G., Wells, A. U., & Denton, C. P. (2014) · Arthritis & Rheumatology · doi:10.1002/art.38390 · Cited by 463 · see citing works
Interstitial lung disease in progressive systemic sclerosis: high-resolution CT versus radiography.
Schurawitzki, H., Stiglbauer, R., Graninger, W. B., et al. (1990) · Radiology · doi:10.1148/radiology.176.3.2389033 · Cited by 295 · see citing works
Reveille, J. D., Fischbach, M., McNearney, T. A., Friedman, A., Aguilar, M. B., LISSE, J. R., Fritzler, M. J., Ahn, C. W., & Arnett, F. C. (2001) · Seminars in Arthritis and Rheumatism · doi:10.1053/sarh.2001.20268 · Cited by 275 · see citing works
Mathai, S. C., Hummers, L. K., Champion, H. C., Wigley, F. M., Zaiman, A., Hassoun, P. M., & Girgis, R. E. (2009) · Arthritis & Rheumatism · doi:10.1002/art.24267 · Cited by 265 · see citing works
Medsger, T. A. (2003) · Rheumatic Disease Clinics of North America · doi:10.1016/s0889-857x(03)00023-1 · Cited by 223 · see citing works
Suliman, Y. A., Dobrota, R. D., Huscher, D., Nguyen‐Kim, T. D. L., Maurer, B., Jordan, S., Speich, R., Frauenfelder, T., & Distler, O. (2015) · Arthritis & Rheumatology · doi:10.1002/art.39405 · Cited by 222 · see citing works
Khanna, D., Gladue, H. S., Channick, R. N., Chung, L. S., Distler, O., Furst, D. E., HACHULLA, E., Humbert, M. J. C., Langleben, D., Mathai, S. C., et al. (2013) · Arthritis & Rheumatism · doi:10.1002/art.38172 · Cited by 210 · see citing works
Self-citationTrad, S., Amoura, Z., Beigelman‐Aubry, C., et al. (2005) · Arthritis & Rheumatism · doi:10.1002/art.21538 · Cited by 151 · see citing works
The Fifth World Symposium on Pulmonary Hypertension
Galiè, N., & Simonneau, G. (2013) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2013.10.030 · Cited by 148 · see citing works
Pavec, J. L., Girgis, R. E., Lechtzin, N., et al. (2011) · Arthritis & Rheumatism · doi:10.1002/art.30423 · Cited by 128 · see citing works
Is Pulmonary Arterial Hypertension Really a Late Complication of Systemic Sclerosis?
HACHULLA, E., Launay, D., Mouthon, L., Sitbon, O., BEREZNE, A., Guillevin, L., Hatron, P., Simonneau, G., Clerson, P., & Humbert, M. J. C. (2009) · CHEST Journal · doi:10.1378/chest.08-3042 · Cited by 123 · see citing works
Clinical and serological features of systemic sclerosis in a multicenter African American cohort
Morgan, N. D., Shah, A. A., Mayes, M. D., Domsic, R. T., Medsger, T. A., Steen, V. D., Varga, J., Carns, M. A., Ramos, P. S., Silver, R. M., et al. (2017) · Medicine · doi:10.1097/md.0000000000008980 · Cited by 115 · see citing works
Self-citationHINCHCLIFF, M., Fischer, A., Schiopu, E., et al. (2011) · The Journal of Rheumatology · doi:10.3899/jrheum.101243 · Cited by 102 · see citing works
Launay, D., Humbert, M., Berezne, A., Cottin, V., Allanore, Y., Couderc, L.-J., Bletry, O., Yaici, A., Hatron, P.-Y., Mouthon, L., et al. (2011) · Chest · doi:10.1378/chest.10-2473 · Cited by 99 · see citing works
Volkmann, E. R., Saggar, R., Khanna, D., et al. (2014) · Arthritis & Rheumatology · doi:10.1002/art.38623 · Cited by 49 · see citing works
Self-citationPulmonary Hypertension Associated with Chronic Respiratory Disease
Girgis, R. E., & Mathai, S. C. (2007) · Clinics in Chest Medicine · doi:10.1016/j.ccm.2006.11.006 · Cited by 48 · see citing works
Severity of Systemic Sclerosis-Associated Pulmonary Arterial Hypertension in African Americans
Blanco, I., Mathai, S. C., Shafiq, M., Boyce, D. M., Kolb, T. M., Chami, H. E., Hummers, L. K., Housten, T., Chaisson, N. F., Zaiman, A., et al. (2014) · Medicine · doi:10.1097/md.0000000000000032 · Cited by 35 · see citing works
Michelfelder, M., Becker, M. O., Riedlinger, A., Siegert, E., Drömann, D., Yu, X. F., Petersen, F., & Riemekasten, G. (2016) · Clinical Rheumatology · doi:10.1007/s10067-016-3504-6 · Cited by 29 · see citing works
Fischer, A., Swigris, J. J., Bolster, M. B., et al. (2015) · PubMed · Cited by 26 · see citing works
Flagged first, then by citations
Cited by
Sorted by citations
Leard, L. E., Holm, A. M., Valapour, M., Glanville, A. R., Attawar, S., Aversa, M., Campos, S. V., Christon, L. M., Cypel, M., Dellgren, G., et al. (2021) · The Journal of Heart and Lung Transplantation · doi:10.1016/j.healun.2021.07.005 · Cited by 821 · see citing works
- Authors
- Young, A., Vummidi, D., Visovatti, S., Homer, K., Wilhalme, H., White, E. S., Flaherty, K., McLaughlin, V., & Khanna, D.
- Affiliations
- University of Michigan (US)
- University of California, Los Angeles (US)
- Corresponding author
- Khanna, Dinesh
- ORCID
- Venue
- Arthritis & Rheumatology
- Publisher
- Wiley
- Published
- 2019-06-18
- Volume
- 71
- Issue
- 8
- Pages
- 1339-1349
- ISSN
- 2326-5191 (ISSN-L)
- 2326-5205
- PMC
- PMC6663644
- OpenAlex
- W2913696824
- Cited by
- 85
- References
- 35
- Citations by year
- 2026: 7 · 2025: 14 · 2024: 11 · 2023: 14 · 2022: 9 · 2021: 12 · 2020: 11 · 2019: 7
- FWCI
- 7.16
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Arthritis & Rheumatology (publishedVersion)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- Deep Blue (University of Michigan) (submittedVersion)
- Funders
- National Institutes of Health (T32‐AR‐007080‐38)
- National Heart, Lung, and Blood Institute (K24‐HL‐111316)
- National Institute of Arthritis and Musculoskeletal and Skin Diseases (K24‐AR‐063120, R01‐AR‐07047)
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
- Health Sciences › Medicine › Pathology and Forensic Medicine › Systemic Sclerosis and Related Diseases
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Keywords
- systemic sclerosis, interstitial lung disease, pulmonary hypertension, pulmonary arterial hypertension, right heart catheterization, survival, prevalence, treatment outcomes, diffuse cutaneous systemic sclerosis
- MeSH terms
- Adult, Female, Cardiac Catheterization, Humans, Hypertension, Pulmonary, Male, Middle Aged, Prospective Studies, Scleroderma, Systemic, Tomography, X-Ray Computed, Comorbidity, Prevalence, Survival Rate, Treatment Outcome, Lung Diseases, Interstitial, Kaplan-Meier Estimate
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
