Clinical features of dermatomyositis patients with anti-TIF1 antibodies: A case based comprehensive review.

Background And Objectives: Dermatomyositis is chronic autoimmune disease primarily affecting skin and muscles. Antibodies are key players of pathogenesis and are in strong correlation with distinct clinical phenotypes. We present a case and a comprehensive review of the literature on dermatomyositis patients with Anti TIF1 antibodies.

Methods: PubMed and Web of Science databases were reviewed. 166 articles were identified; 95 of them were evaluated; 79 of them included to the study. 45 of the included articles were case reports 9 were case series and 25 were research articles. In total 1065 patients were identified but number of patients with available information for different clinical features varied.

Results: 69.6% of the patients with Anti TIF1-γ were female. Prevalence of malignancy was 42.6% among patients with Anti TIF1-γ. Muscle weakness (83%), Gottron sign (82.2%), heliotrope rash (73.7%), nailfold capillary changes (67.7%), dysphagia (38.4%), and joint involvement (31.1%) were the most common clinical features seen in patients with Anti TIF1-γ. Interstitial lung disease (ILD) was reported among 8.7% of patients with Anti TIF1-γ. Advanced age, male gender, dysphagia, and V-neck rash were significant risk factors for malignancy, whereas juvenile age, ILD, TIF1-β antibodies and joint involvement were associated with a decreased risk for malignancy. Advanced age, malignancy, dysphagia, and muscle involvement were associated with an increased risk for mortality.

Conclusions: Patients with advanced age, male gender, dysphagia, and V-neck rash require strict cancer screening. Patients with advanced age, malignancy, dysphagia, and muscle involvement have poor prognosis and should receive aggressive treatment.