Original article
Distinctive cutaneous and systemic features associated with antitranscriptional intermediary factor-1γ antibodies in adults with dermatomyositis

https://doi.org/10.1016/j.jaad.2014.12.009Get rights and content

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.

Section snippets

Patients

Patients with DM (age >18 years) were seen in the outpatient clinics at the Stanford University Department of Dermatology between July 2004 and April 2013. Patients were followed up for multiple visits (on average every 3-6 months) with a median time of follow-up of 361 days. The Stanford Institutional Review Board approved the collection of plasma and clinical information. Patients were considered to have DM if they met “probable” or “definite” criteria for DM based on Bohan and Peter11, 12

Patient characteristics and autoantibody frequencies

Major demographic and systemic features of the cohort are shown in Table I. The cohort was mostly (72%) female with a median age of 48.4 years (range 4.6-86.9 years) at age of diagnosis and had an average of 5.3 ± 5.1 years of follow-up. A total of 28 (21%) patients were clinically amyopathic, 22 (16%) had ILD, and 28 (21%) had a cancer-associated DM.

Of 134 patients, 111 (83%) had at least 1 circulating autoantibody against 1 of the tested antigens. Plasma from 12 (9%) patients reacted with 2

Discussion

We have applied a sensitive and specific serologic assay to characterize the frequency and clinical significance of anti-TIF-1γ antibodies in a large US cohort of adult patients with DM. Anti-TIF-1γ was the most common autoantibody in our cohort, seen in 41% of patients. Previously reported frequencies for anti-TIF-1γ in adult DM range from 7% to 24%,6, 8, 10, 18 and our higher prevalence could be a result of the increased sensitivity of our assay and the patient population. It is possible that

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    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.

    Conflicts of interest: None declared.

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