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Riociguat for idiopathic interstitial pneumonia-associated pulmonary hypertension (RISE-IIP): a randomised, placebo-controlled phase 2b study.

Nathan SD, Behr J, Collard HR, Cottin V, Hoeper MM, Martinez FJ, Corte TJ, Keogh AM, Leuchte H, Mogulkoc N, Ulrich S, Wuyts WA, Yao Z, Boateng F, Wells AU.

Lancet Respir Med. 2019 Aug 12. pii: S2213-2600(19)30250-4. doi: 10.1016/S2213-2600(19)30250-4. [Epub ahead of print]


Outcomes following decline in forced vital capacity in patients with idiopathic pulmonary fibrosis: Results from the INPULSIS and INPULSIS-ON trials of nintedanib.

Richeldi L, Crestani B, Azuma A, Kolb M, Selman M, Stansen W, Quaresma M, Stowasser S, Cottin V.

Respir Med. 2019 Aug 6;156:20-25. doi: 10.1016/j.rmed.2019.08.002. [Epub ahead of print]


Angiotensin Receptor Blockers and Subclinical Interstitial Lung Disease: The MESA Study.

Gannon WD, Anderson MR, Podolanczuk AJ, Kawut SM, Michos ED, Cottin V, Kreuter M, Raghu G, Barr RG, Lederer DJ.

Ann Am Thorac Soc. 2019 Jul 31. doi: 10.1513/AnnalsATS.201903-198RL. [Epub ahead of print] No abstract available.


Clinical Characteristics of Subependymal Giant Cell Astrocytoma in Tuberous Sclerosis Complex.

Jansen AC, Belousova E, Benedik MP, Carter T, Cottin V, Curatolo P, Dahlin M, D'Amato L, d'Augères GB, de Vries PJ, Ferreira JC, Feucht M, Fladrowski C, Hertzberg C, Jozwiak S, Lawson JA, Macaya A, Marques R, Nabbout R, O'Callaghan F, Qin J, Sander V, Sauter M, Shah S, Takahashi Y, Touraine R, Youroukos S, Zonnenberg B, Kingswood JC.

Front Neurol. 2019 Jul 3;10:705. doi: 10.3389/fneur.2019.00705. eCollection 2019.


Progressive fibrosing interstitial lung diseases: current practice in diagnosis and management.

Wijsenbeek M, Kreuter M, Olson A, Fischer A, Bendstrup E, Wells CD, Denton CP, Mounir B, Zouad-Lejour L, Quaresma M, Cottin V.

Curr Med Res Opin. 2019 Aug 2:1-10. doi: 10.1080/03007995.2019.1647040. [Epub ahead of print]


Biomarkers of extracellular matrix turnover in patients with idiopathic pulmonary fibrosis given nintedanib (INMARK study): a randomised, placebo-controlled study.

Maher TM, Stowasser S, Nishioka Y, White ES, Cottin V, Noth I, Selman M, Rohr KB, Michael A, Ittrich C, Diefenbach C, Jenkins RG; INMARK trial investigators.

Lancet Respir Med. 2019 Jul 17. pii: S2213-2600(19)30255-3. doi: 10.1016/S2213-2600(19)30255-3. [Epub ahead of print]


Cost-effectiveness of pirfenidone compared to all available strategies for the treatment of idiopathic pulmonary fibrosis in France.

Clay E, Cristeau O, Chafaie R, Pinta A, Mazaleyrat B, Cottin V.

J Mark Access Health Policy. 2019 Jun 24;7(1):1626171. doi: 10.1080/20016689.2019.1626171. eCollection 2019.


Diagnostic Likelihood Thresholds that Define a Working Diagnosis of Idiopathic Pulmonary Fibrosis.

Walsh SLF, Lederer DJ, Ryerson CJ, Kolb M, Maher TM, Nusser R, Poletti V, Richeldi L, Vancheri C, Wilsher ML, Antoniou KM, Behr J, Bendstrup E, Brown KK, Corte TJ, Cottin V, Crestani B, Flaherty KR, Glaspole IN, Grutters J, Inoue Y, Kondoh Y, Kreuter M, Johannson KA, Ley B, Martinez FJ, Molina-Molina M, Morais A, Nunes H, Raghu G, Selman M, Spagnolo P, Taniguchi H, Tomassetti S, Valeyre D, Wijsenbeek M, Wuyts WA, Wells AU.

Am J Respir Crit Care Med. 2019 Jun 26. doi: 10.1164/rccm.201903-0493OC. [Epub ahead of print]


Digital Lung Auscultation: Will Early Diagnosis of Fibrotic Interstitial Lung Disease Become a Reality?

Richeldi L, Cottin V, Würtemberger G, Kreuter M, Calvello M, Sgalla G.

Am J Respir Crit Care Med. 2019 Jul 15;200(2):261-263. doi: 10.1164/rccm.201902-0306LE. No abstract available.


Lung Diseases in Inflammatory Myopathies.

Barba T, Mainbourg S, Nasser M, Lega JC, Cottin V.

Semin Respir Crit Care Med. 2019 Apr;40(2):255-270. doi: 10.1055/s-0039-1685187. Epub 2019 May 28.


The Ariane-IPF ERS Clinical Research Collaboration: seeking collaboration through launch of a federation of European registries on idiopathic pulmonary fibrosis.

Cottin V, Annesi-Maesano I, Günther A, Galvin L, Kreuter M, Powell P, Prasse A, Reynolds G, Richeldi L, Spagnolo P, Valenzuela C, Wijsenbeek M, Wuyts WA, Crestani B; Ariane-IPF Clinical Research Collaboration consortium.

Eur Respir J. 2019 May 23;53(5). pii: 1900539. doi: 10.1183/13993003.00539-2019. Print 2019 May. No abstract available.


Non-severe eosinophilic granulomatosis with polyangiitis: long-term outcomes after remission-induction trial.

Puéchal X, Pagnoux C, Baron G, Lifermann F, Geffray L, Quémeneur T, Saraux JL, Wislez M, Cottin V, Ruivard M, Limal N, Aouba A, Bonnotte B, Néel A, Agard C, Cohen P, Terrier B, Le Jeunne C, Mouthon L, Ravaud P, Guillevin L; French Vasculitis Study Group investigators .

Rheumatology (Oxford). 2019 May 5. pii: kez139. doi: 10.1093/rheumatology/kez139. [Epub ahead of print]


Association of Angiotensin Modulators With the Course of Idiopathic Pulmonary Fibrosis.

Kreuter M, Lederer DJ, Molina-Molina M, Noth I, Valenzuela C, Frankenstein L, Weycker D, Atwood M, Kirchgaessler KU, Cottin V.

Chest. 2019 Apr 29. pii: S0012-3692(19)30882-7. doi: 10.1016/j.chest.2019.04.015. [Epub ahead of print]


A step-wise approach for establishing a multidisciplinary team for the management of tuberous sclerosis complex: a Delphi consensus report.

Auvin S, Bissler JJ, Cottin V, Fujimoto A, Hofbauer GFL, Jansen AC, Jóźwiak S, Kerecuk L, Kingswood JC, Moavero R, Torra R, Villanueva V.

Orphanet J Rare Dis. 2019 Apr 30;14(1):91. doi: 10.1186/s13023-019-1072-y.


Exome sequencing and pathogenicity-network analysis of five French families implicate mTOR signalling and autophagy in familial sarcoidosis.

Calender A, Lim CX, Weichhart T, Buisson A, Besnard V, Rollat-Farnier PA, Bardel C, Roy P, Cottin V, Devouassoux G, Finat A, Pinson S, Lebecque S, Nunes H, Israel-Biet D, Bentaher A, Valeyre D, Pacheco Y; in the frame of GSF (Group Sarcoidosis France).

Eur Respir J. 2019 Aug 1;54(2). pii: 1900430. doi: 10.1183/13993003.00430-2019. Print 2019 Aug. No abstract available.


Identifying chronic thromboembolic pulmonary hypertension through the French national hospital discharge database.

Cottin V, Avot D, Lévy-Bachelot L, Baxter CA, Ramey DR, Catella L, Bénard S, Sitbon O, Teal S.

PLoS One. 2019 Apr 18;14(4):e0214649. doi: 10.1371/journal.pone.0214649. eCollection 2019.


Study protocol: exploring the efficacy of cyclophosphamide added to corticosteroids for treating acute exacerbation of idiopathic pulmonary fibrosis; a randomized double-blind, placebo-controlled, multi-center phase III trial (EXAFIP).

Naccache JM, Montil M, Cadranel J, Cachanado M, Cottin V, Crestani B, Valeyre D, Wallaert B, Simon T, Nunes H.

BMC Pulm Med. 2019 Apr 11;19(1):75. doi: 10.1186/s12890-019-0830-x.


A 2-Year Observational Study in Patients Suffering from Idiopathic Pulmonary Fibrosis and Treated with Pirfenidone: A French Ancillary Study of PASSPORT.

Jouneau S, Gamez AS, Traclet J, Nunes H, Marchand-Adam S, Kessler R, Israël-Biet D, Borie R, Strombom I, Scalori A, Crestani B, Valeyre D, Cottin V.

Respiration. 2019;98(1):19-28. doi: 10.1159/000496735. Epub 2019 Apr 9.


Cancer incidence in patients with pre-capillary pulmonary hypertension.

Bravos E, Cottin V, Dauphin C, Bouvaist H, Traclet J, Trésorier R, Margelidon-Cozzolino V, Bezzeghoud S, Ahmad K, Accassat S, De Magalhaes E, Mornex JF, Pison C, Bertoletti L.

J Heart Lung Transplant. 2019 Jul;38(7):778-780. doi: 10.1016/j.healun.2019.03.007. Epub 2019 Mar 15. No abstract available.


A Rare Case of an Abdominal Aneurysm in a Patient with Lymphangioleiomyomatosis: A Case Report.

Chérrez-Ojeda I, Espinoza-Plaza J, Cottin V, Chérrez S.

Perm J. 2019;23. pii: 18-170. doi: 10.7812/TPP/18-170.


Orbital mass in ANCA-associated vasculitides: data on clinical, biological, radiological and histological presentation, therapeutic management, and outcome from 59 patients.

Durel CA, Hot A, Trefond L, Aumaitre O, Pugnet G, Samson M, Abad S, Belot A, Blanchard-Delaunay C, Cohen P, Cohen-Aubard F, Cottin V, Crestani B, Moreuil C, Durupt S, Garzaro M, Girszyn N, Godeau B, Hachulla E, Jamilloux Y, Jego P, Killian M, Lazaro E, Le Gallou T, Liozon E, Martin T, Papo T, Perlat A, Pillet P, Guillevin L, Terrier B; French Vasculitis Study Group (FVSG).

Rheumatology (Oxford). 2019 Mar 18. pii: kez071. doi: 10.1093/rheumatology/kez071. [Epub ahead of print]


Molecular endpoints for establishing target engagement by novel idiopathic pulmonary fibrosis therapies.

Wolters PJ, Cottin V.

Eur Respir J. 2019 Mar 18;53(3). pii: 1900283. doi: 10.1183/13993003.00283-2019. Print 2019 Mar. No abstract available.


Pulmonary fibrosis: "idiopathic" is not "cryptogenic".

Cottin V.

Eur Respir J. 2019 Mar 18;53(3). pii: 1802314. doi: 10.1183/13993003.02314-2018. Print 2019 Mar. No abstract available.


Epilepsy in tuberous sclerosis complex: Findings from the TOSCA Study.

Nabbout R, Belousova E, Benedik MP, Carter T, Cottin V, Curatolo P, Dahlin M, D Amato L, d'Augères GB, de Vries PJ, Ferreira JC, Feucht M, Fladrowski C, Hertzberg C, Jozwiak S, Lawson JA, Macaya A, Marques R, O'Callaghan F, Qin J, Sander V, Sauter M, Shah S, Takahashi Y, Touraine R, Youroukos S, Zonnenberg B, Jansen A, Kingswood JC; TOSCA Consortium and TOSCA Investigators.

Epilepsia Open. 2018 Dec 21;4(1):73-84. doi: 10.1002/epi4.12286. eCollection 2019 Mar.


Pulmonary alveolar proteinosis.

Trapnell BC, Nakata K, Bonella F, Campo I, Griese M, Hamilton J, Wang T, Morgan C, Cottin V, McCarthy C.

Nat Rev Dis Primers. 2019 Mar 7;5(1):16. doi: 10.1038/s41572-019-0066-3. Review.


Fibrosing interstitial lung diseases: knowns and unknowns.

Cottin V, Wollin L, Fischer A, Quaresma M, Stowasser S, Harari S.

Eur Respir Rev. 2019 Feb 27;28(151). pii: 180100. doi: 10.1183/16000617.0100-2018. Print 2019 Mar 31. Review.


Lung function outcomes in the INPULSIS® trials of nintedanib in idiopathic pulmonary fibrosis.

Brown KK, Flaherty KR, Cottin V, Raghu G, Inoue Y, Azuma A, Huggins JT, Richeldi L, Stowasser S, Stansen W, Schlenker-Herceg R, Maher TM, Wells AU.

Respir Med. 2019 Jan;146:42-48. doi: 10.1016/j.rmed.2018.11.012. Epub 2018 Nov 19.


Interstitial lung disease associated with systemic sclerosis (SSc-ILD).

Cottin V, Brown KK.

Respir Res. 2019 Jan 18;20(1):13. doi: 10.1186/s12931-019-0980-7. Review.


Therapeutic effects of nintedanib are not influenced by emphysema in the INPULSIS trials.

Cottin V, Azuma A, Raghu G, Stansen W, Stowasser S, Schlenker-Herceg R, Kolb M.

Eur Respir J. 2019 Apr 18;53(4). pii: 1801655. doi: 10.1183/13993003.01655-2018. Print 2019 Apr. No abstract available.


Proceedings of the American College of Rheumatology/Association of Physicians of Great Britain and Ireland Connective Tissue Disease-Associated Interstitial Lung Disease Summit: A Multidisciplinary Approach to Address Challenges and Opportunities.

Fischer A, Strek ME, Cottin V, Dellaripa PF, Bernstein EJ, Brown KK, Danoff SK, Distler O, Hirani N, Jones KD, Khanna D, Lee JS, Lynch DA, Maher TM, Millar AB, Raghu G, Silver RM, Steen VD, Volkmann ER, Mullan RH, O'Dwyer DN, Donnelly SC.

QJM. 2019 Feb 1;112(2):81-93. doi: 10.1093/qjmed/hcy272. No abstract available.


Proceedings of the American College of Rheumatology/Association of Physicians of Great Britain and Ireland Connective Tissue Disease-Associated Interstitial Lung Disease Summit: A Multidisciplinary Approach to Address Challenges and Opportunities.

Fischer A, Strek ME, Cottin V, Dellaripa PF, Bernstein EJ, Brown KK, Danoff SK, Distler O, Hirani N, Jones KD, Khanna D, Lee JS, Lynch DA, Maher TM, Millar AB, Raghu G, Silver RM, Steen VD, Volkmann ER, Mullan RH, O'Dwyer DN, Donnelly SC.

Arthritis Rheumatol. 2019 Feb;71(2):182-195. doi: 10.1002/art.40769. Epub 2019 Jan 3. No abstract available.


Widening the landscape of heritable pulmonary hypertension mutations in paediatric and adult cases.

Eyries M, Montani D, Nadaud S, Girerd B, Levy M, Bourdin A, Trésorier R, Chaouat A, Cottin V, Sanfiorenzo C, Prevot G, Reynaud-Gaubert M, Dromer C, Houeijeh A, Nguyen K, Coulet F, Bonnet D, Humbert M, Soubrier F.

Eur Respir J. 2019 Mar 14;53(3). pii: 1801371. doi: 10.1183/13993003.01371-2018. Print 2019 Mar.


Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases.

Cottin V, Hirani NA, Hotchkin DL, Nambiar AM, Ogura T, Otaola M, Skowasch D, Park JS, Poonyagariyagorn HK, Wuyts W, Wells AU.

Eur Respir Rev. 2018 Dec 21;27(150). pii: 180076. doi: 10.1183/16000617.0076-2018. Print 2018 Dec 31. Review.


Treatment of idiopathic inflammatory myositis associated interstitial lung disease: A systematic review and meta-analysis.

Barba T, Fort R, Cottin V, Provencher S, Durieu I, Jardel S, Hot A, Reynaud Q, Lega JC.

Autoimmun Rev. 2019 Feb;18(2):113-122. doi: 10.1016/j.autrev.2018.07.013. Epub 2018 Dec 17.


Targeting interleukin-13 in idiopathic pulmonary fibrosis: from promising path to dead end.

Wijsenbeek MS, Kool M, Cottin V.

Eur Respir J. 2018 Dec 13;52(6). pii: 1802111. doi: 10.1183/13993003.02111-2018. Print 2018 Dec. No abstract available.


Air travel and incidence of pneumothorax in lymphangioleiomyomatosis.

Gonano C, Pasquier J, Daccord C, Johnson SR, Harari S, Leclerc V, Falconer L, Miano E, Cordier JF, Cottin V, Lazor R.

Orphanet J Rare Dis. 2018 Dec 13;13(1):222. doi: 10.1186/s13023-018-0964-6.


Regulator of telomere length 1 (RTEL1) mutations are associated with heterogeneous pulmonary and extra-pulmonary phenotypes.

Borie R, Bouvry D, Cottin V, Gauvain C, Cazes A, Debray MP, Cadranel J, Dieude P, Degot T, Dominique S, Gamez AS, Jaillet M, Juge PA, Londono-Vallejo A, Mailleux A, Mal H, Boileau C, Menard C, Nunes H, Prevot G, Quetant S, Revy P, Traclet J, Wemeau-Stervinou L, Wislez M, Kannengiesser C, Crestani B.

Eur Respir J. 2019 Feb 7;53(2). pii: 1800508. doi: 10.1183/13993003.00508-2018. Print 2019 Feb.


Impact of comorbidities and delay in diagnosis in elderly patients with pulmonary hypertension.

Ginoux M, Turquier S, Chebib N, Glerant JC, Traclet J, Philit F, Sénéchal A, Mornex JF, Cottin V.

ERJ Open Res. 2018 Nov 28;4(4). pii: 00100-2018. doi: 10.1183/23120541.00100-2018. eCollection 2018 Oct.


[Etiological diagnosis of pulmonary hypertension: A cause of difficult diagnosis].

Rigaud P, Traclet J, Cottin V.

Rev Mal Respir. 2019 Mar;36(3):350-354. doi: 10.1016/j.rmr.2018.11.004. Epub 2018 Nov 22. French.


Combined pulmonary fibrosis and emphysema in systemic sclerosis: A syndrome associated with heavy morbidity and mortality.

Champtiaux N, Cottin V, Chassagnon G, Chaigne B, Valeyre D, Nunes H, Hachulla E, Launay D, Crestani B, Cazalets C, Jego P, Bussone G, Bérezné A, Guillevin L, Revel MP, Cordier JF, Mouthon L; Groupe d'Etudes et de Recherche sur les Maladies « Orphelines » pulmonaires (GERM«O»P).

Semin Arthritis Rheum. 2019 Aug;49(1):98-104. doi: 10.1016/j.semarthrit.2018.10.011. Epub 2018 Oct 13.


Alveolar Hemorrhage in Vasculitis (Primary and Secondary).

Nasser M, Cottin V.

Semin Respir Crit Care Med. 2018 Aug;39(4):482-493. doi: 10.1055/s-0038-1668533. Epub 2018 Nov 7. Review.


12q13.12q13.13 microdeletion encompassing ACVRL1 and SCN8A genes: Clinical report of a new contiguous gene syndrome.

Poisson A, Lesca G, Chatron N, Favre E, Cottin V, Gamondes D, Sanlaville D, Edery P, Giraud S, Demily C, Dupuis-Girod S.

Eur J Med Genet. 2018 Oct 30. pii: S1769-7212(18)30557-3. doi: 10.1016/j.ejmg.2018.10.017. [Epub ahead of print]


Long-term safety of pirfenidone: results of the prospective, observational PASSPORT study.

Cottin V, Koschel D, Günther A, Albera C, Azuma A, Sköld CM, Tomassetti S, Hormel P, Stauffer JL, Strombom I, Kirchgaessler KU, Maher TM.

ERJ Open Res. 2018 Oct 19;4(4). pii: 00084-2018. doi: 10.1183/23120541.00084-2018. eCollection 2018 Oct.


MUC5B Promoter Variant and Rheumatoid Arthritis with Interstitial Lung Disease.

Juge PA, Lee JS, Ebstein E, Furukawa H, Dobrinskikh E, Gazal S, Kannengiesser C, Ottaviani S, Oka S, Tohma S, Tsuchiya N, Rojas-Serrano J, González-Pérez MI, Mejía M, Buendía-Roldán I, Falfán-Valencia R, Ambrocio-Ortiz E, Manali E, Papiris SA, Karageorgas T, Boumpas D, Antoniou K, van Moorsel CHM, van der Vis J, de Man YA, Grutters JC, Wang Y, Borie R, Wemeau-Stervinou L, Wallaert B, Flipo RM, Nunes H, Valeyre D, Saidenberg-Kermanac'h N, Boissier MC, Marchand-Adam S, Frazier A, Richette P, Allanore Y, Sibilia J, Dromer C, Richez C, Schaeverbeke T, Lioté H, Thabut G, Nathan N, Amselem S, Soubrier M, Cottin V, Clément A, Deane K, Walts AD, Fingerlin T, Fischer A, Ryu JH, Matteson EL, Niewold TB, Assayag D, Gross A, Wolters P, Schwarz MI, Holers M, Solomon JJ, Doyle T, Rosas IO, Blauwendraat C, Nalls MA, Debray MP, Boileau C, Crestani B, Schwartz DA, Dieudé P.

N Engl J Med. 2018 Dec 6;379(23):2209-2219. doi: 10.1056/NEJMoa1801562. Epub 2018 Oct 20.


Lung Biopsy in Interstitial Lung Diseases: There Is More than One Way to Cook an Egg.

Nasser M, Cottin V.

Respiration. 2018;96(5):403-405. doi: 10.1159/000493157. Epub 2018 Oct 9. No abstract available.


Dysregulation of the endothelin pathway in lymphangioleiomyomatosis with no direct effect on cell proliferation and migration.

Chebib N, Archer F, Bobet-Erny A, Leroux C, Cottin V.

Sci Rep. 2018 Oct 2;8(1):14698. doi: 10.1038/s41598-018-32795-3.


Pirfenidone in patients with unclassifiable progressive fibrosing interstitial lung disease: design of a double-blind, randomised, placebo-controlled phase II trial.

Maher TM, Corte TJ, Fischer A, Kreuter M, Lederer DJ, Molina-Molina M, Axmann J, Kirchgaessler KU, Cottin V.

BMJ Open Respir Res. 2018 Sep 4;5(1):e000289. doi: 10.1136/bmjresp-2018-000289. eCollection 2018.


Haemodynamics and serial risk assessment in systemic sclerosis associated pulmonary arterial hypertension.

Weatherald J, Boucly A, Launay D, Cottin V, Prévot G, Bourlier D, Dauphin C, Chaouat A, Savale L, Jaïs X, Jevnikar M, Traclet J, De Groote P, Simonneau G, Hachulla E, Mouthon L, Montani D, Humbert M, Sitbon O.

Eur Respir J. 2018 Oct 18;52(4). pii: 1800678. doi: 10.1183/13993003.00678-2018. Print 2018 Oct.


TSC-associated neuropsychiatric disorders (TAND): findings from the TOSCA natural history study.

de Vries PJ, Belousova E, Benedik MP, Carter T, Cottin V, Curatolo P, Dahlin M, D'Amato L, d'Augères GB, Ferreira JC, Feucht M, Fladrowski C, Hertzberg C, Jozwiak S, Kingswood JC, Lawson JA, Macaya A, Marques R, Nabbout R, O'Callaghan F, Qin J, Sander V, Sauter M, Shah S, Takahashi Y, Touraine R, Youroukos S, Zonnenberg B, Jansen AC; TOSCA Consortium and TOSCA Investigators.

Orphanet J Rare Dis. 2018 Sep 10;13(1):157. doi: 10.1186/s13023-018-0901-8.


Should Patients With Interstitial Lung Disease Be Seen by Experts?

Cottin V, Castillo D, Poletti V, Kreuter M, Corte TJ, Spagnolo P.

Chest. 2018 Sep;154(3):713-714. doi: 10.1016/j.chest.2018.05.044. No abstract available.


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