G Leuschner1, C Neurohr2. 1. Medizinische Klinik und Poliklinik V, Campus Großhadern, Klinikum der Universität München, Marchioninistr. 15, 81377, München, Deutschland. Gabriela.leuschner@med.uni-muenchen.de. 2. Abteilung für Pneumologie und Beatmungsmedizin, Robert-Bosch-Krankenhaus, Klinik Schillerhöhe, Solitudestr. 18, 70839, Gerlingen, Deutschland.
Abstract
BACKGROUND: Pulmonary involvement in patients with underlying autoimmune diseases poses a major diagnostic and therapeutic challenge to the treating physician. Due to the associated increased mortality risk, early diagnosis is crucial. OBJECTIVE: The incidence and mortality rate of connective tissue disease-related interstitial lung diseases (CTD-ILD) and pulmonary hypertension (PH) were evaluated in patients with rheumatic disease including clinical aspects, diagnostic procedure, prognosis and treatment recommendations. MATERIAL AND METHODS: An analysis of remarkable publications was carried out and guidelines are presented. RESULTS: The CTD-ILD and PH are frequent comorbidities with significantly increased mortality risk, especially in patients with systemic sclerosis (SSc). In primary fibrotic and non-inflammatory CTD-ILD, as occurs especially in patients with rheumatoid arthritis, immunosuppressive therapy is only partially effective. Currently, in some eligible patients only lung transplantation remains as a definitive therapy. CONCLUSION: The diagnostics and treatment of CTD-ILD and PH in patients with an underlying autoimmune disease requires an interdisciplinary approach. The effectiveness of antifibrotic treatment needs to be evaluated in the future.
BACKGROUND: Pulmonary involvement in patients with underlying autoimmune diseases poses a major diagnostic and therapeutic challenge to the treating physician. Due to the associated increased mortality risk, early diagnosis is crucial. OBJECTIVE: The incidence and mortality rate of connective tissue disease-related interstitial lung diseases (CTD-ILD) and pulmonary hypertension (PH) were evaluated in patients with rheumatic disease including clinical aspects, diagnostic procedure, prognosis and treatment recommendations. MATERIAL AND METHODS: An analysis of remarkable publications was carried out and guidelines are presented. RESULTS: The CTD-ILD and PH are frequent comorbidities with significantly increased mortality risk, especially in patients with systemic sclerosis (SSc). In primary fibrotic and non-inflammatory CTD-ILD, as occurs especially in patients with rheumatoid arthritis, immunosuppressive therapy is only partially effective. Currently, in some eligible patients only lung transplantation remains as a definitive therapy. CONCLUSION: The diagnostics and treatment of CTD-ILD and PH in patients with an underlying autoimmune disease requires an interdisciplinary approach. The effectiveness of antifibrotic treatment needs to be evaluated in the future.
Authors: Aryeh Fischer; Kevin K Brown; Roland M Du Bois; Stephen K Frankel; Gregory P Cosgrove; Evans R Fernandez-Perez; Tristan J Huie; Mahalakshmi Krishnamoorthy; Richard T Meehan; Amy L Olson; Joshua J Solomon; Jeffrey J Swigris Journal: J Rheumatol Date: 2013-03-01 Impact factor: 4.666
Authors: Talmadge E King; Williamson Z Bradford; Socorro Castro-Bernardini; Elizabeth A Fagan; Ian Glaspole; Marilyn K Glassberg; Eduard Gorina; Peter M Hopkins; David Kardatzke; Lisa Lancaster; David J Lederer; Steven D Nathan; Carlos A Pereira; Steven A Sahn; Robert Sussman; Jeffrey J Swigris; Paul W Noble Journal: N Engl J Med Date: 2014-05-18 Impact factor: 91.245
Authors: Joshua J Solomon; Jonathan H Chung; Gregory P Cosgrove; M Kristen Demoruelle; Evans R Fernandez-Perez; Aryeh Fischer; Stephen K Frankel; Stephen B Hobbs; Tristan J Huie; Jill Ketzer; Amar Mannina; Amy L Olson; Gloria Russell; Yutaka Tsuchiya; Zulma X Yunt; Pearlanne T Zelarney; Kevin K Brown; Jeffrey J Swigris Journal: Eur Respir J Date: 2015-11-19 Impact factor: 16.671
Authors: Florian M P Meier; Klaus W Frommer; Robert Dinser; Ulrich A Walker; Laszlo Czirjak; Christopher P Denton; Yannick Allanore; Oliver Distler; Gabriela Riemekasten; Gabriele Valentini; Ulf Müller-Ladner Journal: Ann Rheum Dis Date: 2012-05-21 Impact factor: 19.103
Authors: Jürgen Behr; Petra Neuser; Antje Prasse; Michael Kreuter; Klaus Rabe; Carmen Schade-Brittinger; Jasmin Wagner; Andreas Günther Journal: BMC Pulm Med Date: 2017-09-06 Impact factor: 3.317
Authors: J Gerry Coghlan; Christopher P Denton; Ekkehard Grünig; Diana Bonderman; Oliver Distler; Dinesh Khanna; Ulf Müller-Ladner; Janet E Pope; Madelon C Vonk; Martin Doelberg; Harbajan Chadha-Boreham; Harald Heinzl; Daniel M Rosenberg; Vallerie V McLaughlin; James R Seibold Journal: Ann Rheum Dis Date: 2013-05-18 Impact factor: 19.103
Authors: Donald P Tashkin; Michael D Roth; Philip J Clements; Daniel E Furst; Dinesh Khanna; Eric C Kleerup; Jonathan Goldin; Edgar Arriola; Elizabeth R Volkmann; Suzanne Kafaja; Richard Silver; Virginia Steen; Charlie Strange; Robert Wise; Fredrick Wigley; Maureen Mayes; David J Riley; Sabiha Hussain; Shervin Assassi; Vivien M Hsu; Bela Patel; Kristine Phillips; Fernando Martinez; Jeffrey Golden; M Kari Connolly; John Varga; Jane Dematte; Monique E Hinchcliff; Aryeh Fischer; Jeffrey Swigris; Richard Meehan; Arthur Theodore; Robert Simms; Suncica Volkov; Dean E Schraufnagel; Mary Beth Scholand; Tracy Frech; Jerry A Molitor; Kristin Highland; Charles A Read; Marvin J Fritzler; Grace Hyun J Kim; Chi-Hong Tseng; Robert M Elashoff Journal: Lancet Respir Med Date: 2016-07-25 Impact factor: 30.700