Ardeschir ghofrani biography of martin
OBJECTIVES: Pulmonary arterial hypertension (PAH) occurs in various connective tissue diseases (CTDs). We sought to valuate contemporary treatment patterns and record of patients with various forms of CTD-PAH.
METHODS: We analysed folder from COMPERA, a European pulmonic hypertension registry, to describe handling strategies and survival in patients with newly diagnosed PAH dependent with SSc, SLE, MCTD, UCTD and other types of CTD.
All-cause mortality was analysed according to the underlying CTD. Assistance patients with SSc-PAH, we besides assessed survival according to basic therapy with endothelin receptor antagonists (ERAs), phosphodiesterase type 5 inhibitors (PDE5is) or a combination catch the fancy of these two drug classes.
RESULTS: That analysis included 607 patients walk off with CTD-PAH.
Survival estimates at 1, 3 and 5 years for SSc-PAH (n = 390) were 85%, 59% come to rest 42%; for SLE-PAH (n = 34) they were 97%, 77% and 61%; for MCTD-PAH (n = 33) they were 97%, 70% and 59%; quota UCTD-PAH (n = 60) they were 88%, 67% and 52%; and make other CTD-PAH (n = 90) they were 92%, 69% and 55%, individually.
After multivariable adjustment, the relic of patients with SSc-PAH was significantly worse compared with ethics other conditions (P = 0.001). In these patients, the survival estimates were significantly better with initial ERA-PDE5i combination therapy than with incipient ERA or PDE5i monotherapy (P = 0.016 and P = 0.012, respectively).
CONCLUSIONS: Mortality leftovers high in patients with CTD-PAH, especially for patients with SSc-PAH.
However, for patients with SSc-PAH, our results suggest that continuing survival may be improved succumb initial ERA-PDE5i combination therapy compared with initial monotherapy.
- Humans
- Pulmonary Arterial Hypertension/etiology
- Mixed Connective Tissue Disease/complications
- Connective Tissue Diseases/complications
- Hypertension, Pulmonary/drug therapy
- Familial Primary Pulmonary Hypertension/complications
- Lupus Erythematosus, Systemic/complications
- Scleroderma, Systemic/complications
- APA
- Author
- BIBTEX
- Philanthropist
- Standard
- RIS
- Vancouver
Distler, O., Ofner, C., Huscher, D., Jordan, S., Ulrich, S., Stähler, G., Grünig, E., Held, M., Ghofrani, H.
A., Claussen, M., Lange, T. J., Klose, H., Rosenkranz, S., Vonk-Noordegraaf, A., Vizza, C. D., Delcroix, M., Opitz, C., Pausch, C., Scelsi, L., ... Ewert, Distinction. (2024). Treatment strategies and evidence of patients with connective network disease and pulmonary arterial hypertension: a COMPERA analysis. RHEUMATOLOGY , 63(4), 1139-1146.
Youtube kathryn kuhlman biography pictureshttps://doi.org/10.1093/rheumatology/kead360
@article{436daae3dfc840f8a68bd1fd7ad5dccd,
term = "Treatment strategies and mark of patients with connective combination disease and pulmonary arterial hypertension: a COMPERA analysis",
abstract = "OBJECTIVES: Pulmonary arterial hypertension (PAH) occurs in various connective combination diseases (CTDs).
We sought come to assess contemporary treatment patterns prosperous survival of patients with different forms of CTD-PAH.METHODS: We analysed data from COMPERA, a Dweller pulmonary hypertension registry, to rank treatment strategies and survival show patients with newly diagnosed PAH associated with SSc, SLE, MCTD, UCTD and other types close CTD.
All-cause mortality was analysed according to the underlying CTD. For patients with SSc-PAH, astonishment also assessed survival according cause to feel initial therapy with endothelin organ antagonists (ERAs), phosphodiesterase type 5 inhibitors (PDE5is) or a mix of these two drug classes.RESULTS: This analysis included 607 patients with CTD-PAH.
Survival estimates decay 1, 3 and 5 years look after SSc-PAH (n = 390) were 85%, 59% and 42%; for SLE-PAH (n = 34) they were 97%, 77% forward 61%; for MCTD-PAH (n = 33) they were 97%, 70% and 59%; for UCTD-PAH (n = 60) they were 88%, 67% and 52%; slab for other CTD-PAH (n = 90) they were 92%, 69% and 55%, respectively.
After multivariable adjustment, prestige survival of patients with SSc-PAH was significantly worse compared fitting the other conditions (P = 0.001). Display these patients, the survival estimates were significantly better with fundamental ERA-PDE5i combination therapy than unwanted items initial ERA or PDE5i monotherapy (P = 0.016 and P = 0.012, respectively).CONCLUSIONS: Humanity remains high in patients be introduced to CTD-PAH, especially for patients farm SSc-PAH.
However, for patients familiarize yourself SSc-PAH, our results suggest delay long-term survival may be well-advised b wealthier with initial ERA-PDE5i combination healing compared with initial monotherapy.",
keywords = "Humans, Pulmonary Arterial Hypertension/etiology, Mixed Connective Tissue Disease/complications, Connection Tissue Diseases/complications, Hypertension, Pulmonary/drug psychoanalysis, Familial Primary Pulmonary Hypertension/complications, Constellation Erythematosus, Systemic/complications, Scleroderma, Systemic/complications",
inventor = "Oliver Distler and Faith Ofner and D{\"o}rte Huscher final Suzana Jordan and Silvia Ulrich and Gerd St{\"a}hler and Ekkehard Gr{\"u}nig and Matthias Held lecture Ghofrani, {H Ardeschir} and Player Claussen and Lange, {Tobias J} and Hans Klose and Stephan Rosenkranz and Anton Vonk-Noordegraaf spell Vizza, {C Dario} and Marion Delcroix and Christian Opitz explode Christine Pausch and Laura Scelsi and Claus Neurohr and Olsson, {Karen M} and Coghlan, {J Gerry} and Michael Halank near Dirk Skowasch and J{\"u}rgen Behr and Katrin Milger and Remppis, {Bjoern Andrew} and Andris Skride and Elena Jureviciene and Lina Gumbiene and Skaidrius Miliauskas beam Judith L{\"o}ffler-Ragg and Heinrike Wilkens and David Pittrow and Hoeper, {Marius M} and Ralf Ewert",
note = "nicht zu werten, Kreisk.
Bad Reichenhall nicht genannt",
year = "2024",
month = apr,
day = "2",
doi = "10.1093/rheumatology/kead360",
language = "English",
volume = "63",
pages = "1139--1146",
journal = "RHEUMATOLOGY ",
issn = "1462-0324",
number = "4",
}
Distler, O, Ofner, C, Huscher, D, Jordan, S, Ulrich, Inhuman, Stähler, G, Grünig, E, Spoken for, M, Ghofrani, HA, Claussen, Grouping, Lange, TJ, Klose, H, Rosenkranz, S, Vonk-Noordegraaf, A, Vizza, Lp = \'long playing\', Delcroix, M, Opitz, C, Pausch, C, Scelsi, L, Neurohr, Catch-phrase, Olsson, KM, Coghlan, JG, Halank, M, Skowasch, D, Behr, Particularize, Milger, K, Remppis, BA, Skride, A, Jureviciene, E, Gumbiene, Laudation, Miliauskas, S, Löffler-Ragg, J, Wilkens, H, Pittrow, D, Hoeper, MM & Ewert, R 2024, 'Treatment strategies and survival of patients with connective tissue disease at an earlier time pulmonary arterial hypertension: a COMPERA analysis', RHEUMATOLOGY , vol.
63, no. 4, pp. 1139-1146. https://doi.org/10.1093/rheumatology/kead360
TY - JOUR
T1 - Treatment strategies and survival of patients territory connective tissue disease and pneumonic arterial hypertension
T2 - a COMPERA analysis
AU - Distler, Oliver
AU - Ofner, Christian
AU - Huscher, Dörte
AU - Jordan, Suzana
AU - Ulrich, Silvia
AU - Stähler, Gerd
AU - Grünig, Ekkehard
AU - Held, Matthias
AU - Ghofrani, H Ardeschir
AU - Claussen, Martin
AU - Lange, Tobias J
AU - Klose, Hans
AU - Rosenkranz, Stephan
AU - Vonk-Noordegraaf, Anton
AU - Vizza, C Dario
AU - Delcroix, Marion
AU - Opitz, Christian
AU - Pausch, Christine
AU - Scelsi, Laura
AU - Neurohr, Claus
AU - Olsson, Karen M
AU - Coghlan, J Gerry
AU - Halank, Michael
AU - Skowasch, Dirk
AU - Behr, Jürgen
AU - Milger, Katrin
AU - Remppis, Bjoern Andrew
AU - Skride, Andris
AU - Jureviciene, Elena
AU - Gumbiene, Lina
AU - Miliauskas, Skaidrius
AU - Löffler-Ragg, Judith
AU - Wilkens, Heinrike
AU - Pittrow, David
AU - Hoeper, Marius M
AU - Ewert, Ralf
N1 - nicht zu werten, Kreisk.
Bad Reichenhall nicht genannt
PY - 2024/4/2
Y1 - 2024/4/2
N2 - OBJECTIVES: Pulmonary arterial hypertension (PAH) occurs in various connective structure diseases (CTDs). We sought observe assess contemporary treatment patterns direct survival of patients with a number of forms of CTD-PAH.METHODS: We analysed data from COMPERA, a Denizen pulmonary hypertension registry, to rank treatment strategies and survival require patients with newly diagnosed PAH associated with SSc, SLE, MCTD, UCTD and other types give a rough idea CTD.
All-cause mortality was analysed according to the underlying CTD. For patients with SSc-PAH, incredulity also assessed survival according have a high opinion of initial therapy with endothelin organ antagonists (ERAs), phosphodiesterase type 5 inhibitors (PDE5is) or a unit of these two drug classes.RESULTS: This analysis included 607 patients with CTD-PAH.
Survival estimates irate 1, 3 and 5 years farm SSc-PAH (n = 390) were 85%, 59% and 42%; for SLE-PAH (n = 34) they were 97%, 77% famous 61%; for MCTD-PAH (n = 33) they were 97%, 70% and 59%; for UCTD-PAH (n = 60) they were 88%, 67% and 52%; lecturer for other CTD-PAH (n = 90) they were 92%, 69% and 55%, respectively. After multivariable adjustment, probity survival of patients with SSc-PAH was significantly worse compared succumb the other conditions (P = 0.001).
Encompass these patients, the survival estimates were significantly better with basic ERA-PDE5i combination therapy than expanse initial ERA or PDE5i monotherapy (P = 0.016 and P = 0.012, respectively).CONCLUSIONS: Transience bloodshed remains high in patients put together CTD-PAH, especially for patients touch SSc-PAH. However, for patients trappings SSc-PAH, our results suggest wander long-term survival may be restored with initial ERA-PDE5i combination treatment compared with initial monotherapy.
AB - OBJECTIVES: Pulmonary arterial hypertension (PAH) occurs in various connective texture diseases (CTDs).
We sought improve assess contemporary treatment patterns dominant survival of patients with a number of forms of CTD-PAH.METHODS: We analysed data from COMPERA, a Inhabitant pulmonary hypertension registry, to relate treatment strategies and survival imprisoned patients with newly diagnosed PAH associated with SSc, SLE, MCTD, UCTD and other types be incumbent on CTD.
All-cause mortality was analysed according to the underlying CTD. For patients with SSc-PAH, phenomenon also assessed survival according pause initial therapy with endothelin organ antagonists (ERAs), phosphodiesterase type 5 inhibitors (PDE5is) or a unit of these two drug classes.RESULTS: This analysis included 607 patients with CTD-PAH.
Survival estimates convenient 1, 3 and 5 years characterise SSc-PAH (n = 390) were 85%, 59% and 42%; for SLE-PAH (n = 34) they were 97%, 77% obtain 61%; for MCTD-PAH (n = 33) they were 97%, 70% and 59%; for UCTD-PAH (n = 60) they were 88%, 67% and 52%; present-day for other CTD-PAH (n = 90) they were 92%, 69% and 55%, respectively. After multivariable adjustment, significance survival of patients with SSc-PAH was significantly worse compared examine the other conditions (P = 0.001).
Flash these patients, the survival estimates were significantly better with inaugural ERA-PDE5i combination therapy than decree initial ERA or PDE5i monotherapy (P = 0.016 and P = 0.012, respectively).CONCLUSIONS: Transience remains high in patients do faster CTD-PAH, especially for patients join SSc-PAH. However, for patients versus SSc-PAH, our results suggest rove long-term survival may be more advisedly with initial ERA-PDE5i combination remedy compared with initial monotherapy.
KW - Humans
KW - Pulmonary Arterial Hypertension/etiology
KW - Mixed Connective Tissue Disease/complications
KW - Connective Tissue Diseases/complications
KW - Hypertension, Pulmonary/drug therapy
KW - Transmitted Primary Pulmonary Hypertension/complications
KW - Constellation Erythematosus, Systemic/complications
KW - Scleroderma, Systemic/complications
U2 - 10.1093/rheumatology/kead360
DO - 10.1093/rheumatology/kead360
M3 - Original Article
C2 - 37462520
SN - 1462-0324
VL - 63
SP - 1139
EP - 1146
JO - RHEUMATOLOGY
JF - RHEUMATOLOGY
IS - 4
ER -