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Clinical presentation and long-term outcome of 144 patients with microscopic polyangiitis in a monocentric German cohort

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Clinical presentation and long-term outcome of 144 patients with microscopic polyangiitis in a monocentric German cohort. / Schirmer, Jan H; Wright, Marvin N; Vonthein, Reinhard; Herrmann, Kristine; Nölle, Bernhard; Both, Marcus; Henes, Frank O; Arlt, Andreas; Gross, Wolfgang L; Schinke, Susanne; Reinhold-Keller, Eva; Moosig, Frank; Holle, Julia U.

In: RHEUMATOLOGY, Vol. 55, No. 1, 01.2016, p. 71-9.

Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review

Harvard

Schirmer, JH, Wright, MN, Vonthein, R, Herrmann, K, Nölle, B, Both, M, Henes, FO, Arlt, A, Gross, WL, Schinke, S, Reinhold-Keller, E, Moosig, F & Holle, JU 2016, 'Clinical presentation and long-term outcome of 144 patients with microscopic polyangiitis in a monocentric German cohort', RHEUMATOLOGY, vol. 55, no. 1, pp. 71-9. https://doi.org/10.1093/rheumatology/kev286

APA

Schirmer, J. H., Wright, M. N., Vonthein, R., Herrmann, K., Nölle, B., Both, M., Henes, F. O., Arlt, A., Gross, W. L., Schinke, S., Reinhold-Keller, E., Moosig, F., & Holle, J. U. (2016). Clinical presentation and long-term outcome of 144 patients with microscopic polyangiitis in a monocentric German cohort. RHEUMATOLOGY, 55(1), 71-9. https://doi.org/10.1093/rheumatology/kev286

Vancouver

Schirmer JH, Wright MN, Vonthein R, Herrmann K, Nölle B, Both M et al. Clinical presentation and long-term outcome of 144 patients with microscopic polyangiitis in a monocentric German cohort. RHEUMATOLOGY. 2016 Jan;55(1):71-9. https://doi.org/10.1093/rheumatology/kev286

Bibtex

@article{eaf668f0a31344d1ba626f7910e68216,
title = "Clinical presentation and long-term outcome of 144 patients with microscopic polyangiitis in a monocentric German cohort",
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.",
keywords = "Adolescent, Adult, Aged, Aged, 80 and over, Disease Progression, Female, Follow-Up Studies, Germany, Glucocorticoids, Humans, Immunosuppressive Agents, Incidence, Male, Microscopic Polyangiitis, Middle Aged, Remission Induction, Retrospective Studies, Survival Rate, Time Factors, Young Adult, Journal Article",
author = "Schirmer, {Jan H} and Wright, {Marvin N} and Reinhard Vonthein and Kristine Herrmann and Bernhard N{\"o}lle and Marcus Both and Henes, {Frank O} and Andreas Arlt and Gross, {Wolfgang L} and Susanne Schinke and Eva Reinhold-Keller and Frank Moosig and Holle, {Julia U}",
note = "{\textcopyright} The Author 2015. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.",
year = "2016",
month = jan,
doi = "10.1093/rheumatology/kev286",
language = "English",
volume = "55",
pages = "71--9",
journal = "RHEUMATOLOGY",
issn = "1462-0324",
publisher = "Oxford University Press",
number = "1",

}

RIS

TY - JOUR

T1 - Clinical presentation and long-term outcome of 144 patients with microscopic polyangiitis in a monocentric German cohort

AU - Schirmer, Jan H

AU - Wright, Marvin N

AU - Vonthein, Reinhard

AU - Herrmann, Kristine

AU - Nölle, Bernhard

AU - Both, Marcus

AU - Henes, Frank O

AU - Arlt, Andreas

AU - Gross, Wolfgang L

AU - Schinke, Susanne

AU - Reinhold-Keller, Eva

AU - Moosig, Frank

AU - Holle, Julia U

N1 - © The Author 2015. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

PY - 2016/1

Y1 - 2016/1

N2 - 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.

AB - 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.

KW - Adolescent

KW - Adult

KW - Aged

KW - Aged, 80 and over

KW - Disease Progression

KW - Female

KW - Follow-Up Studies

KW - Germany

KW - Glucocorticoids

KW - Humans

KW - Immunosuppressive Agents

KW - Incidence

KW - Male

KW - Microscopic Polyangiitis

KW - Middle Aged

KW - Remission Induction

KW - Retrospective Studies

KW - Survival Rate

KW - Time Factors

KW - Young Adult

KW - Journal Article

U2 - 10.1093/rheumatology/kev286

DO - 10.1093/rheumatology/kev286

M3 - SCORING: Journal article

C2 - 26297628

VL - 55

SP - 71

EP - 79

JO - RHEUMATOLOGY

JF - RHEUMATOLOGY

SN - 1462-0324

IS - 1

ER -