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.
journal_name
J Am Acad Dermatoljournal_title
Journal of the American Academy of Dermatologyauthors
Fiorentino DF,Kuo K,Chung L,Zaba L,Li S,Casciola-Rosen Ldoi
10.1016/j.jaad.2014.12.009subject
Has Abstractpub_date
2015-03-01 00:00:00pages
449-55issue
3eissn
0190-9622issn
1097-6787pii
S0190-9622(14)02269-5journal_volume
72pub_type
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