Clinical Characteristics of Anti-TIF-1 gamma Antibody-Positive Dermatomyositis Associated with Malignancy

We retrospectively analyzed the clinical and laboratory data of patients diagnosed with anti-transcriptional intermediary factor 1 (TIF-1 gamma) antibody-positive polymyositis (PM)/dermatomyositis (DM) to clarify the characteristics of this disease. We identified 14 patients with TIF-1 gamma antibody-positive DM (TIF-1 gamma DM), 47 with anti-aminoacyl-tRNA synthetase antibody (ARS)-positive PM/DM, and 24 with anti-melanoma differentiation-associated gene 5 antibody (MDA-5)-positive PM/DM treated at the Kurume University Hospital between 2002 and 2020. Patients with TIF-1 gamma DM were significantly older than the other two groups. Nine patients with TIF-1 gamma DM were female, thirteen patients had DM, and one had clinically amyopathic DM. Primary malignant lesions were lung (3), uterus (2), colon (2), breast (2), ovary (1), lymphoma (1), and unknown (2). Cutaneous manifestation and dysphagia were the most common symptoms in TIF-1 gamma DM. Erythema (9/14), the V-neck sign (8/14), heliotrope (9/14), and nailfold telangiectasia (14/14) were significantly more common in TIF-1 gamma DM. Furthermore, no patients with TIF-1 gamma DM had interstitial lung abnormality on high-resolution CT. In patients with TIF-1 gamma DM, the frequency of dysphagia and unusual erythema, particularly that which spreads from the trunk, and nailfold telangiectasia, were characteristic findings. In most patients with TIF-1 gamma DM, it is necessary to administer other immunosuppressive drugs along with glucocorticoids.