Journal article
Systemic sclerosis associated interstitial lung disease - individualized immunosuppressive therapy and course of lung function: results of the EUSTAR group.
- Adler S Department of Rheumatology, Immunology and Allergology, University Hospital and University of Bern, Freiburgstrasse 4, 3010, Bern, Switzerland. sabine.adler@insel.ch.
- Huscher D German Rheumatism Research Center, A Leibniz Institute, Berlin, Germany.
- Siegert E Department of Rheumatology and Clinical Immunology, Charité University Hospital, Berlin, Germany.
- Allanore Y Department of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France.
- Czirják L Department of Rheumatology and Immunology, University of Pecs, Pecs, Hungary.
- DelGaldo F University of Leeds, Leeds, UK.
- Denton CP UCL Division of Medicine, Centre for Rheumatology, Royal Free Hospital, London, UK.
- Distler O Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland.
- Frerix M Department of Rheumatology and Clinical Immunology, Osteology and Physical Therapy, Justus-Liebig-University Giessen, Kerckhoff Klinik, Bad Nauheim, Germany.
- Matucci-Cerinic M Department Experimental and Clinical Medicine, Division of Rheumatology AOUC, University of Florence, Florence, Italy.
- Mueller-Ladner U Department of Rheumatology and Clinical Immunology, Osteology and Physical Therapy, Justus-Liebig-University Giessen, Kerckhoff Klinik, Bad Nauheim, Germany.
- Tarner IH Department of Rheumatology and Clinical Immunology, Osteology and Physical Therapy, Justus-Liebig-University Giessen, Kerckhoff Klinik, Bad Nauheim, Germany.
- Valentini G Department of Rheumatology, Second University of Naples, Naples, Italy.
- Walker UA Department of Rheumatology, University of Basel, Basel, Switzerland.
- Villiger PM Department of Rheumatology, Immunology and Allergology, University Hospital and University of Bern, Freiburgstrasse 4, 3010, Bern, Switzerland.
- Riemekasten G Department of Rheumatology, University Medical Center Schleswig-Holstein, Kiel, Germany.
- 2018-02-01
Published in:
- Arthritis research & therapy. - 2018
Follow up
Immunosuppressants
Interstitial lung disease
Lung function
Systemic sclerosis
Adult
Aged
Cyclophosphamide
Drug Therapy, Combination
Female
Humans
Immunosuppressive Agents
Lung
Lung Diseases, Interstitial
Male
Methotrexate
Middle Aged
Mycophenolic Acid
Precision Medicine
Respiratory Function Tests
Scleroderma, Systemic
English
BACKGROUND
Interstitial lung disease in systemic sclerosis (SSc-ILD) is a major cause of SSc-related death. Imunosuppressive treatment (IS) is used in patients with SSc for various organ manifestations mainly to ameliorate progression of SSc-ILD. Data on everyday IS prescription patterns and clinical courses of lung function during and after therapy are scarce.
METHODS
We analysed patients fulfilling American College of Rheumatology (ACR)/European League against Rheumatism (EULAR) 2013 criteria for SSc-ILD and at least one report of IS. Types of IS, pulmonary function tests (PFT) and PFT courses during IS treatment were evaluated.
RESULTS
EUSTAR contains 3778/11,496 patients with SSc-ILD (33%), with IS in 2681/3,778 (71%). Glucocorticoid (GC) monotherapy was prescribed in 30.6% patients with GC combinations plus cyclophosphamide (CYC) (11.9%), azathioprine (AZA) (9.2%), methotrexate (MTX) (8.7%), or mycophenolate mofetil (MMF) (7.3%). Intensive IS (MMF + GC, CYC or CYC + GC) was started in patients with the worst PFTs and ground glass opacifications on imaging. Patients without IS showed slightly less worsening in forced vital capacity (FVC) when starting with FVC 50-75% or >75%. GC showed negative trends when starting with FVC <50%. Regarding diffusing capacity for carbon monoxide (DLCO), negative DLCO trends were found in patients with MMF.
CONCLUSIONS
IS is broadly prescribed in SSc-ILD. Clusters of clinical and functional characteristics guide individualised treatment. Data favour distinguished decision-making, pointing to either watchful waiting and close monitoring in the early stages or start of immunosuppressive treatment in moderately impaired lung function. Advantages of specific IS are difficult to depict due to confounding by indication. Data do not support liberal use of GC in SSc-ILD.
Interstitial lung disease in systemic sclerosis (SSc-ILD) is a major cause of SSc-related death. Imunosuppressive treatment (IS) is used in patients with SSc for various organ manifestations mainly to ameliorate progression of SSc-ILD. Data on everyday IS prescription patterns and clinical courses of lung function during and after therapy are scarce.
METHODS
We analysed patients fulfilling American College of Rheumatology (ACR)/European League against Rheumatism (EULAR) 2013 criteria for SSc-ILD and at least one report of IS. Types of IS, pulmonary function tests (PFT) and PFT courses during IS treatment were evaluated.
RESULTS
EUSTAR contains 3778/11,496 patients with SSc-ILD (33%), with IS in 2681/3,778 (71%). Glucocorticoid (GC) monotherapy was prescribed in 30.6% patients with GC combinations plus cyclophosphamide (CYC) (11.9%), azathioprine (AZA) (9.2%), methotrexate (MTX) (8.7%), or mycophenolate mofetil (MMF) (7.3%). Intensive IS (MMF + GC, CYC or CYC + GC) was started in patients with the worst PFTs and ground glass opacifications on imaging. Patients without IS showed slightly less worsening in forced vital capacity (FVC) when starting with FVC 50-75% or >75%. GC showed negative trends when starting with FVC <50%. Regarding diffusing capacity for carbon monoxide (DLCO), negative DLCO trends were found in patients with MMF.
CONCLUSIONS
IS is broadly prescribed in SSc-ILD. Clusters of clinical and functional characteristics guide individualised treatment. Data favour distinguished decision-making, pointing to either watchful waiting and close monitoring in the early stages or start of immunosuppressive treatment in moderately impaired lung function. Advantages of specific IS are difficult to depict due to confounding by indication. Data do not support liberal use of GC in SSc-ILD.
- Language
-
- English
- Open access status
- gold
- Identifiers
-
- DOI 10.1186/s13075-018-1517-z
- PMID 29382380
- Persistent URL
- https://folia.unifr.ch/global/documents/233313
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