Distinctive cutaneous and systemic features associated with antitranscriptional intermediary factor-1? antibodies in adults with dermatomyositis - 15/02/15
Abstract |
Background |
Antibodies against transcriptional intermediary factor (TIF)-1γ are associated with malignancy in dermatomyositis (DM). Identification of clinical findings associated with anti-TIF-1γ antibodies in DM is a high priority for both patient diagnosis and risk assessment.
Objective |
We sought to define the clinical phenotype of patients with anti-TIF-1γ DM.
Methods |
Using a novel, sensitive, and specific assay for anti-TIF-1γ antibodies, we retrospectively tested plasma from 134 adult patients with DM and examined associations between anti-TIF-1γ antibodies and particular clinical and laboratory features.
Results |
In all, 55 (41%) patients had autoantibodies to TIF-1γ. Anti-TIF-1γ positive patients were less likely to have systemic features including interstitial lung disease, Raynaud phenomenon, and arthritis/arthralgia. Patients with TIF-1γ autoantibodies had more extensive skin involvement, and some patients manifested characteristic findings including palmar hyperkeratotic papules, psoriasis-like lesions and a novel finding of hypopigmented and telangiectatic (“red on white”) patches.
Limitations |
This was a retrospective study from a single tertiary referral center.
Conclusion |
TIF-1γ is the most commonly targeted DM-specific autoantigen in adults in a large US cohort. Although these patients tend to have less systemic involvement, their skin disease is often extensive and characteristic. Recognition of cutaneous findings in anti-TIF-1γ positive patients may allow more accurate and timely diagnosis and effective treatment of patients with DM.
Le texte complet de cet article est disponible en PDF.Key words : autoantibodies, Cutaneous Dermatomyositis Assessment and Severity Index, dermatomyositis, malignancy, phenotype, transcriptional intermediary factor-1γ
Abbreviations used : CDASI, DM, ILD, NXP2, TIF, UV
Plan
Drs Fiorentino and Rosen are supported by National Institutes of Health (NIH) AR062615-01A1. Dr Rosen is supported by NIH RO1 AR-44684 and the Donald and Dorothy Stabler Foundation. The Johns Hopkins Rheumatic Diseases Research Core Center, where the assays were performed, is supported by NIH P30-AR-053503. |
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Conflicts of interest: None declared. |
Vol 72 - N° 3
P. 449-455 - mars 2015 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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