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Anti-MDA5-positive dermatomyositis and remission in a single referral centre population
E. Tiniakou1, C.A. Mecoli2, W. Kelly3, J. Albayda4, J.J. Paik5, B.L. Adler6, C.T. Lin7, A.L. Mammen8, S.K. Danoff9, L. Casciola-Rosen10, L. Christopher-Stine11
- Division of Rheumatology, Johns Hopkins School of Medicine, Baltimore, MD, USA.
- Division of Rheumatology, Johns Hopkins School of Medicine, Baltimore, MD, USA. cmecoli1@jhmi.edu
- Division of Rheumatology, Johns Hopkins School of Medicine, Baltimore, MD, USA.
- Division of Rheumatology, Johns Hopkins School of Medicine, Baltimore, MD, USA.
- Division of Rheumatology, Johns Hopkins School of Medicine, Baltimore, MD, USA.
- Division of Rheumatology, Johns Hopkins School of Medicine, Baltimore, MD, USA.
- Department of Radiology, Johns Hopkins School of Medicine, Baltimore, MD, USA.
- Muscle Disease Unit, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda; and Department of Neurology, Johns Hopkins School of Medicine, Baltimore, MD, USA.
- Division of Pulmonary and Critical Care Medicine, Johns Hopkins School of Medicine, Baltimore, MD, USA.
- Division of Rheumatology, Johns Hopkins School of Medicine, Baltimore, MD, USA.
- Division of Rheumatology, Johns Hopkins School of Medicine, Baltimore, and Department of Neurology, Johns Hopkins School of Medicine, Baltimore, MD, USA. lchrist4@jhmi.edu
CER16289
2023 Vol.41, N°2
PI 0309, PF 0315
Full Papers
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PMID: 36826791 [PubMed]
Received: 17/10/2022
Accepted : 23/01/2023
In Press: 15/02/2023
Published: 01/03/2023
Abstract
OBJECTIVES:
To describe a single-centre North American adult cohort of anti-MDA5-positive dermatomyositis patients, with emphasis on drug-free long-term remission.
METHODS:
We conducted an observational retrospective cohort study of anti-MDA5-positive DM patients. All consented patients seen in the Johns Hopkins Myositis Centre from 2003-2020 with suspected muscle disease were routinely screened for myositis-specific autoantibodies. All sera were screened for anti-MDA5 autoantibodies by line blot; positives were verified by enzyme-linked immunoassay. Patients whose sera were anti-MDA5 positive by both assays (n=52) were followed longitudinally. If clinical status was unavailable, structured telephone interviews were conducted. Clinical remission was defined as being off all immunosuppression >1 year while remaining asymptomatic.
RESULTS:
38/52 (73%) of the patients were women with a median age at disease-onset of 47 (IQR 40-54). Twenty-five of the patients (48%) were White, 16 (30%) were Black and 3 (6%) were Asian. Most patients (42/52, 80%) had interstitial lung disease, defined by inflammatory or fibrotic changes on high resolution computed tomography (HRCT). 18/52 (35%) of patients required pulse-dose methylprednisolone, 4/52 (8%) experienced spontaneous pneumothorax/pneumomediastinum, 6/52 (12%) required intubation, and 5/52 (10%) died. Over longitudinal follow-up (median 3.5 years), 9 (18%) patients achieved clinical remission. The median time from symptom onset to clinical remission was 4 years, and the median duration of sustained remission was 3.5 years (range 1.4-7.8). No demographic or disease characteristics were significantly associated with remission.
CONCLUSIONS:
In this single centre, tertiary referral population of anti-MDA5-positive dermatomyositis, ~20% of patients experienced long-term drug-free remission after a median disease duration of 4 years. No clinical or biologic factors were associated with clinical remission.
