Journal article
The Challenge of Very Early Systemic Sclerosis: A Combination of Mild and Early Disease?
- Blaja E E. Blaja, MD, S. Jordan, PhD, C.M. Mihai, MD, PhD, R. Dobrota, MD, PhD, M.O. Becker, MD, B. Maurer, MD, O. Distler, Professor of Rheumatology, MD, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland; M. Matucci-Cerinic, Division of Rheumatology, University of Florence, Florence, Italy. B. Maurer had grant/research support from AbbVie, Protagen, Novartis; congress support from Pfizer, Roche, Actelion, and MSD; and a consultancy relationship with Novartis. In addition, BM has a patent mir-29 for the treatment of systemic sclerosis (SSc) registered. The work of BM is supported by the Prof. Max Cloetta Foundation. O. Distler had a consultancy relationship and/or has received research funding from Actelion, Acceleron Pharma, Amgen, AnaMar, Baecon Discovery, Blade Therapeutics, Bayer, Boehringer Ingelheim, Catenion, CSL Behring, Curzion Pharmaceuticals, Ergonex, Glenmark Pharmaceuticals GSK, Inventiva, Italfarmaco, iQvia, Lilly, medac, Medscape, Mitsubishi Tanabe Pharma, MSD, Novartis, Pfizer, Roche, Target Bio Science, and UCB in the area of potential treatments of scleroderma and its complications. In addition, Prof. Distler has a patent mir-29 for the treatment of SSc issued (US8247389, EP2331143). Address correspondence to Dr. O. Distler, University Hospital Zurich, Department of Rheumatology, Gloriastr. 25, CH-8091 Zurich, Switzerland. Email: Oliver.Distler@usz.ch. Accepted for publication March 6, 2020.
- Jordan S E. Blaja, MD, S. Jordan, PhD, C.M. Mihai, MD, PhD, R. Dobrota, MD, PhD, M.O. Becker, MD, B. Maurer, MD, O. Distler, Professor of Rheumatology, MD, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland; M. Matucci-Cerinic, Division of Rheumatology, University of Florence, Florence, Italy. B. Maurer had grant/research support from AbbVie, Protagen, Novartis; congress support from Pfizer, Roche, Actelion, and MSD; and a consultancy relationship with Novartis. In addition, BM has a patent mir-29 for the treatment of systemic sclerosis (SSc) registered. The work of BM is supported by the Prof. Max Cloetta Foundation. O. Distler had a consultancy relationship and/or has received research funding from Actelion, Acceleron Pharma, Amgen, AnaMar, Baecon Discovery, Blade Therapeutics, Bayer, Boehringer Ingelheim, Catenion, CSL Behring, Curzion Pharmaceuticals, Ergonex, Glenmark Pharmaceuticals GSK, Inventiva, Italfarmaco, iQvia, Lilly, medac, Medscape, Mitsubishi Tanabe Pharma, MSD, Novartis, Pfizer, Roche, Target Bio Science, and UCB in the area of potential treatments of scleroderma and its complications. In addition, Prof. Distler has a patent mir-29 for the treatment of SSc issued (US8247389, EP2331143). Address correspondence to Dr. O. Distler, University Hospital Zurich, Department of Rheumatology, Gloriastr. 25, CH-8091 Zurich, Switzerland. Email: Oliver.Distler@usz.ch. Accepted for publication March 6, 2020.
- Mihai CM E. Blaja, MD, S. Jordan, PhD, C.M. Mihai, MD, PhD, R. Dobrota, MD, PhD, M.O. Becker, MD, B. Maurer, MD, O. Distler, Professor of Rheumatology, MD, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland; M. Matucci-Cerinic, Division of Rheumatology, University of Florence, Florence, Italy. B. Maurer had grant/research support from AbbVie, Protagen, Novartis; congress support from Pfizer, Roche, Actelion, and MSD; and a consultancy relationship with Novartis. In addition, BM has a patent mir-29 for the treatment of systemic sclerosis (SSc) registered. The work of BM is supported by the Prof. Max Cloetta Foundation. O. Distler had a consultancy relationship and/or has received research funding from Actelion, Acceleron Pharma, Amgen, AnaMar, Baecon Discovery, Blade Therapeutics, Bayer, Boehringer Ingelheim, Catenion, CSL Behring, Curzion Pharmaceuticals, Ergonex, Glenmark Pharmaceuticals GSK, Inventiva, Italfarmaco, iQvia, Lilly, medac, Medscape, Mitsubishi Tanabe Pharma, MSD, Novartis, Pfizer, Roche, Target Bio Science, and UCB in the area of potential treatments of scleroderma and its complications. In addition, Prof. Distler has a patent mir-29 for the treatment of SSc issued (US8247389, EP2331143). Address correspondence to Dr. O. Distler, University Hospital Zurich, Department of Rheumatology, Gloriastr. 25, CH-8091 Zurich, Switzerland. Email: Oliver.Distler@usz.ch. Accepted for publication March 6, 2020.
- Dobrota R E. Blaja, MD, S. Jordan, PhD, C.M. Mihai, MD, PhD, R. Dobrota, MD, PhD, M.O. Becker, MD, B. Maurer, MD, O. Distler, Professor of Rheumatology, MD, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland; M. Matucci-Cerinic, Division of Rheumatology, University of Florence, Florence, Italy. B. Maurer had grant/research support from AbbVie, Protagen, Novartis; congress support from Pfizer, Roche, Actelion, and MSD; and a consultancy relationship with Novartis. In addition, BM has a patent mir-29 for the treatment of systemic sclerosis (SSc) registered. The work of BM is supported by the Prof. Max Cloetta Foundation. O. Distler had a consultancy relationship and/or has received research funding from Actelion, Acceleron Pharma, Amgen, AnaMar, Baecon Discovery, Blade Therapeutics, Bayer, Boehringer Ingelheim, Catenion, CSL Behring, Curzion Pharmaceuticals, Ergonex, Glenmark Pharmaceuticals GSK, Inventiva, Italfarmaco, iQvia, Lilly, medac, Medscape, Mitsubishi Tanabe Pharma, MSD, Novartis, Pfizer, Roche, Target Bio Science, and UCB in the area of potential treatments of scleroderma and its complications. In addition, Prof. Distler has a patent mir-29 for the treatment of SSc issued (US8247389, EP2331143). Address correspondence to Dr. O. Distler, University Hospital Zurich, Department of Rheumatology, Gloriastr. 25, CH-8091 Zurich, Switzerland. Email: Oliver.Distler@usz.ch. Accepted for publication March 6, 2020.
- Becker MO E. Blaja, MD, S. Jordan, PhD, C.M. Mihai, MD, PhD, R. Dobrota, MD, PhD, M.O. Becker, MD, B. Maurer, MD, O. Distler, Professor of Rheumatology, MD, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland; M. Matucci-Cerinic, Division of Rheumatology, University of Florence, Florence, Italy. B. Maurer had grant/research support from AbbVie, Protagen, Novartis; congress support from Pfizer, Roche, Actelion, and MSD; and a consultancy relationship with Novartis. In addition, BM has a patent mir-29 for the treatment of systemic sclerosis (SSc) registered. The work of BM is supported by the Prof. Max Cloetta Foundation. O. Distler had a consultancy relationship and/or has received research funding from Actelion, Acceleron Pharma, Amgen, AnaMar, Baecon Discovery, Blade Therapeutics, Bayer, Boehringer Ingelheim, Catenion, CSL Behring, Curzion Pharmaceuticals, Ergonex, Glenmark Pharmaceuticals GSK, Inventiva, Italfarmaco, iQvia, Lilly, medac, Medscape, Mitsubishi Tanabe Pharma, MSD, Novartis, Pfizer, Roche, Target Bio Science, and UCB in the area of potential treatments of scleroderma and its complications. In addition, Prof. Distler has a patent mir-29 for the treatment of SSc issued (US8247389, EP2331143). Address correspondence to Dr. O. Distler, University Hospital Zurich, Department of Rheumatology, Gloriastr. 25, CH-8091 Zurich, Switzerland. Email: Oliver.Distler@usz.ch. Accepted for publication March 6, 2020.
- Maurer B E. Blaja, MD, S. Jordan, PhD, C.M. Mihai, MD, PhD, R. Dobrota, MD, PhD, M.O. Becker, MD, B. Maurer, MD, O. Distler, Professor of Rheumatology, MD, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland; M. Matucci-Cerinic, Division of Rheumatology, University of Florence, Florence, Italy. B. Maurer had grant/research support from AbbVie, Protagen, Novartis; congress support from Pfizer, Roche, Actelion, and MSD; and a consultancy relationship with Novartis. In addition, BM has a patent mir-29 for the treatment of systemic sclerosis (SSc) registered. The work of BM is supported by the Prof. Max Cloetta Foundation. O. Distler had a consultancy relationship and/or has received research funding from Actelion, Acceleron Pharma, Amgen, AnaMar, Baecon Discovery, Blade Therapeutics, Bayer, Boehringer Ingelheim, Catenion, CSL Behring, Curzion Pharmaceuticals, Ergonex, Glenmark Pharmaceuticals GSK, Inventiva, Italfarmaco, iQvia, Lilly, medac, Medscape, Mitsubishi Tanabe Pharma, MSD, Novartis, Pfizer, Roche, Target Bio Science, and UCB in the area of potential treatments of scleroderma and its complications. In addition, Prof. Distler has a patent mir-29 for the treatment of SSc issued (US8247389, EP2331143). Address correspondence to Dr. O. Distler, University Hospital Zurich, Department of Rheumatology, Gloriastr. 25, CH-8091 Zurich, Switzerland. Email: Oliver.Distler@usz.ch. Accepted for publication March 6, 2020.
- Matucci-Cerinic M E. Blaja, MD, S. Jordan, PhD, C.M. Mihai, MD, PhD, R. Dobrota, MD, PhD, M.O. Becker, MD, B. Maurer, MD, O. Distler, Professor of Rheumatology, MD, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland; M. Matucci-Cerinic, Division of Rheumatology, University of Florence, Florence, Italy. B. Maurer had grant/research support from AbbVie, Protagen, Novartis; congress support from Pfizer, Roche, Actelion, and MSD; and a consultancy relationship with Novartis. In addition, BM has a patent mir-29 for the treatment of systemic sclerosis (SSc) registered. The work of BM is supported by the Prof. Max Cloetta Foundation. O. Distler had a consultancy relationship and/or has received research funding from Actelion, Acceleron Pharma, Amgen, AnaMar, Baecon Discovery, Blade Therapeutics, Bayer, Boehringer Ingelheim, Catenion, CSL Behring, Curzion Pharmaceuticals, Ergonex, Glenmark Pharmaceuticals GSK, Inventiva, Italfarmaco, iQvia, Lilly, medac, Medscape, Mitsubishi Tanabe Pharma, MSD, Novartis, Pfizer, Roche, Target Bio Science, and UCB in the area of potential treatments of scleroderma and its complications. In addition, Prof. Distler has a patent mir-29 for the treatment of SSc issued (US8247389, EP2331143). Address correspondence to Dr. O. Distler, University Hospital Zurich, Department of Rheumatology, Gloriastr. 25, CH-8091 Zurich, Switzerland. Email: Oliver.Distler@usz.ch. Accepted for publication March 6, 2020.
- Distler O E. Blaja, MD, S. Jordan, PhD, C.M. Mihai, MD, PhD, R. Dobrota, MD, PhD, M.O. Becker, MD, B. Maurer, MD, O. Distler, Professor of Rheumatology, MD, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland; M. Matucci-Cerinic, Division of Rheumatology, University of Florence, Florence, Italy. B. Maurer had grant/research support from AbbVie, Protagen, Novartis; congress support from Pfizer, Roche, Actelion, and MSD; and a consultancy relationship with Novartis. In addition, BM has a patent mir-29 for the treatment of systemic sclerosis (SSc) registered. The work of BM is supported by the Prof. Max Cloetta Foundation. O. Distler had a consultancy relationship and/or has received research funding from Actelion, Acceleron Pharma, Amgen, AnaMar, Baecon Discovery, Blade Therapeutics, Bayer, Boehringer Ingelheim, Catenion, CSL Behring, Curzion Pharmaceuticals, Ergonex, Glenmark Pharmaceuticals GSK, Inventiva, Italfarmaco, iQvia, Lilly, medac, Medscape, Mitsubishi Tanabe Pharma, MSD, Novartis, Pfizer, Roche, Target Bio Science, and UCB in the area of potential treatments of scleroderma and its complications. In addition, Prof. Distler has a patent mir-29 for the treatment of SSc issued (US8247389, EP2331143). Address correspondence to Dr. O. Distler, University Hospital Zurich, Department of Rheumatology, Gloriastr. 25, CH-8091 Zurich, Switzerland. Email: Oliver.Distler@usz.ch. Accepted for publication March 6, 2020.
- 2020-03-17
Published in:
- The Journal of rheumatology. - 2020
English
OBJECTIVE
To address the hypothesis that very early patients with systemic sclerosis (SSc) are a heterogeneous group with mild or early disease, we analyzed the extent of heterogeneity in clinical, epidemiological, and immunological characteristics of these patients.
METHODS
We performed an analysis of very early SSc patients from the Zurich cohort, who fulfilled neither the 2013 American College of Rheumatology (ACR)/European League Against Rheumatism nor the 1980 ACR classification criteria, but had a clinical expert diagnosis of SSc with Raynaud phenomenon (RP) and additional features of SSc (puffy fingers, SSc-specific antibodies, SSc pattern on nailfold capillaroscopy, or any organ involvement characteristic for SSc). Disease duration was defined from first RP symptom.
RESULTS
One hundred and two patients fulfilled the inclusion criteria and were analyzed. Their clinical presentation was heterogeneous with the large majority presenting with RP, antinuclear antibodies, and nailfold capillaroscopy changes, but with varying presentations of other features such as SSc-specific antibodies and early signs of organ involvement. While 54.1% (52/96) of patients had a disease duration of < 5 years, as many as 29.1% (28/96) of patients had a disease duration of > 10 years, indicating long-standing mild disease. Patients with very early, potentially progressive disease did not differ from patients with long-standing mild disease in terms of their clinical features at first presentation.
CONCLUSION
This study showed that patients with very early SSc are a mixture with mild or early disease. This needs to be considered in clinical practice for risk stratification and for the study design of patients considered as early SSc.
To address the hypothesis that very early patients with systemic sclerosis (SSc) are a heterogeneous group with mild or early disease, we analyzed the extent of heterogeneity in clinical, epidemiological, and immunological characteristics of these patients.
METHODS
We performed an analysis of very early SSc patients from the Zurich cohort, who fulfilled neither the 2013 American College of Rheumatology (ACR)/European League Against Rheumatism nor the 1980 ACR classification criteria, but had a clinical expert diagnosis of SSc with Raynaud phenomenon (RP) and additional features of SSc (puffy fingers, SSc-specific antibodies, SSc pattern on nailfold capillaroscopy, or any organ involvement characteristic for SSc). Disease duration was defined from first RP symptom.
RESULTS
One hundred and two patients fulfilled the inclusion criteria and were analyzed. Their clinical presentation was heterogeneous with the large majority presenting with RP, antinuclear antibodies, and nailfold capillaroscopy changes, but with varying presentations of other features such as SSc-specific antibodies and early signs of organ involvement. While 54.1% (52/96) of patients had a disease duration of < 5 years, as many as 29.1% (28/96) of patients had a disease duration of > 10 years, indicating long-standing mild disease. Patients with very early, potentially progressive disease did not differ from patients with long-standing mild disease in terms of their clinical features at first presentation.
CONCLUSION
This study showed that patients with very early SSc are a mixture with mild or early disease. This needs to be considered in clinical practice for risk stratification and for the study design of patients considered as early SSc.
- Language
-
- English
- Open access status
- closed
- Identifiers
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- DOI 10.3899/jrheum.190976
- PMID 32173655
- Persistent URL
- https://folia.unifr.ch/global/documents/230226
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