Jan H Schirmer1, Marvin N Wright2, Reinhard Vonthein3, Kristine Herrmann4, Bernhard Nölle5, Marcus Both6, Frank O Henes7, Andreas Arlt8, Wolfgang L Gross9, Susanne Schinke4, Eva Reinhold-Keller4, Frank Moosig4, Julia U Holle4. 1. Department of Rheumatology, University Medical Center Schleswig-Holstein and Klinikum Bad Bramstedt, Bad Bramstedt, j.schirmer@klinikumbb.de. 2. Department of Medical Biometry and Statistics, University of Lübeck, University Medical Center Schleswig-Holstein, Campus Lübeck. 3. Department of Medical Biometry and Statistics, University of Lübeck, University Medical Center Schleswig-Holstein, Campus Lübeck, Center for Clinical Trials Lübeck, University of Lübeck, Lübeck. 4. Department of Rheumatology, University Medical Center Schleswig-Holstein and Klinikum Bad Bramstedt, Bad Bramstedt. 5. Department of Ophthalmology. 6. Department of Radiology and Neuroradiology, University Medical Center Schleswig-Holstein, Campus Kiel, Kiel. 7. Clinic and Policlinic for Diagnostic and Interventional Radiology, University Medical Center Hamburg-Eppendorf, Hamburg. 8. Department of Medical and Neurological Rehabilitation, Klinikum Bad Bramstedt, Bad Bramstedt and. 9. University Medical Center Schleswig-Holstein, Campus Lübeck, Lübeck, Germany.
Abstract
OBJECTIVE: To evaluate the clinical presentation and long-term outcome of a vasculitis centre cohort of patients with microscopic polyangiitis (MPA) with respect to organ manifestations, treatment, chronic damage and mortality. METHODS: We performed a retrospective chart review at our vasculitis referral centre. MPA patients admitted between 1991 and 2013 classified by a modified European Medicines Agency algorithm were diagnosed and treated according to a standardized interdisciplinary approach. RESULTS: Comprehensive data from standardized interdisciplinary workups was available for 144 patients (median follow-up 72 months). The overall standardized mortality ratio was 1.40 (95% CI 0.91, 2.07; P = 0.13). We observed a higher mortality [hazard ratio (HR) 4.04 (95% CI 1.21, 13.45), P = 0.02] in 17 patients with MPA-associated fibrosing interstitial lung disease (ILD) and 56 patients with peripheral nervous system involvement [HR 5.26 (95% CI 1.10, 25.14), P = 0.04] at disease onset. One hundred and fifteen patients (79.9%) responded to the initial treatment. Sixty-one (42.3%) achieved complete remission and 54 (37.5%) achieved partial remission. Twenty (13.9%) showed a refractory disease course. CONCLUSION: MPA patients at our tertiary rheumatology referral centre seemed to have a less severe phenotype resulting in a less severe disease course and better outcome than reported in other cohorts. Fibrosing ILD was significantly associated with mortality in this cohort.
OBJECTIVE: To evaluate the clinical presentation and long-term outcome of a vasculitis centre cohort of patients with microscopic polyangiitis (MPA) with respect to organ manifestations, treatment, chronic damage and mortality. METHODS: We performed a retrospective chart review at our vasculitis referral centre. MPA patients admitted between 1991 and 2013 classified by a modified European Medicines Agency algorithm were diagnosed and treated according to a standardized interdisciplinary approach. RESULTS: Comprehensive data from standardized interdisciplinary workups was available for 144 patients (median follow-up 72 months). The overall standardized mortality ratio was 1.40 (95% CI 0.91, 2.07; P = 0.13). We observed a higher mortality [hazard ratio (HR) 4.04 (95% CI 1.21, 13.45), P = 0.02] in 17 patients with MPA-associated fibrosing interstitial lung disease (ILD) and 56 patients with peripheral nervous system involvement [HR 5.26 (95% CI 1.10, 25.14), P = 0.04] at disease onset. One hundred and fifteen patients (79.9%) responded to the initial treatment. Sixty-one (42.3%) achieved complete remission and 54 (37.5%) achieved partial remission. Twenty (13.9%) showed a refractory disease course. CONCLUSION: MPA patients at our tertiary rheumatology referral centre seemed to have a less severe phenotype resulting in a less severe disease course and better outcome than reported in other cohorts. Fibrosing ILD was significantly associated with mortality in this cohort.
Authors: Marco A Alba; Luis Felipe Flores-Suárez; Ashley G Henderson; Hong Xiao; Peiqi Hu; Patrick H Nachman; Ronald J Falk; J Charles Jennette Journal: Autoimmun Rev Date: 2017-05-04 Impact factor: 9.754
Authors: Gabrielle Y Liu; Iazsmin Bauer Ventura; Natalia Achtar-Zadeh; Brett M Elicker; Kirk D Jones; Paul J Wolters; Harold R Collard; Ayodeji Adegunsoye; Mary E Strek; Brett Ley Journal: Chest Date: 2019-06-07 Impact factor: 9.410
Authors: Jan Henrik Schirmer; Peer M Aries; Kirsten de Groot; Bernhard Hellmich; Julia U Holle; Christian Kneitz; Ina Kötter; Peter Lamprecht; Ulf Müller-Ladner; Eva Reinhold-Keller; Christof Specker; Michael Zänker; Frank Moosig Journal: Z Rheumatol Date: 2017-11 Impact factor: 1.372
Authors: Peter Lamprecht; Anja Kerstein; Sebastian Klapa; Susanne Schinke; Christian M Karsten; Xinhua Yu; Marc Ehlers; Jörg T Epplen; Konstanze Holl-Ulrich; Thorsten Wiech; Kathrin Kalies; Tanja Lange; Martin Laudien; Tamas Laskay; Timo Gemoll; Udo Schumacher; Sebastian Ullrich; Hauke Busch; Saleh Ibrahim; Nicole Fischer; Katrin Hasselbacher; Ralph Pries; Frank Petersen; Gesche Weppner; Rudolf Manz; Jens Y Humrich; Relana Nieberding; Gabriela Riemekasten; Antje Müller Journal: Front Immunol Date: 2018-04-09 Impact factor: 7.561