SHM Converge Scientific Abstract ePoster Gallery-Anti-Transcriptional Intermediary Factor (Tif) 1 Gamma Antibody-Positive Dermatomyositis and Malignancy Risk

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Anti-transcriptional intermediary factor 1 gamma (anti-TIF-1-γ) antibody-positive dermatomyositis is strongly linked to an increased risk of malignancy and should prompt thorough cancer screening and continued surveillance if the initial workup is negative.<br /><br />This case describes a 44-year-old woman with a history of systemic lupus erythematosus, rheumatoid arthritis overlap syndrome, fibromyalgia, and psychogenic nonepileptic seizures who developed progressive dysphagia over one year, followed by proximal muscle weakness and a rash on the face and chest. Exam showed 4/5 strength in proximal upper and lower extremities, heliotrope rash, V-sign rash, and periungual erythema. Laboratory testing revealed elevated AST, ALT, CK, and aldolase, with a high ANA titer and strongly positive anti-TIF-1-γ antibody. EMG demonstrated proximal irritable myopathy, supporting the diagnosis of dermatomyositis.<br /><br />Because anti-TIF-1-γ antibodies are associated with malignancy, she underwent CT imaging of the chest, abdomen, and pelvis, which did not show cancer. She was already current on routine cancer screening. Rheumatology recommended additional outpatient transvaginal ultrasound to assess for ovarian cancer. Treatment included intravenous immunoglobulin and high-dose methylprednisolone followed by a steroid taper, with improvement in weakness, dysphagia, and rash.<br /><br />The discussion highlights that anti-TIF-1-γ is a myositis-specific antibody found in about 22% of dermatomyositis patients and is associated with a markedly increased malignancy risk, especially for lung, stomach, breast, and ovarian cancers. Recommended screening for anti-TIF-1-γ-positive female patients includes CT of the neck, chest, abdomen, and pelvis, cervical cancer screening, mammography, CA-125, pelvic or transvaginal ultrasound, and fecal occult blood testing.<br /><br />Overall, the case emphasizes the need for vigilant cancer evaluation in dermatomyositis patients with anti-TIF-1-γ positivity.

Asset Subtitle

Karen A. Clarke

Meta Tag

Author List Karen A. Clarke, Kelly Kaysen Ammons, Veda Koneru

Category Clinical Vignettes

Concept Dermatomyositis

Concept Anti-TIF-1γ Antibody

Concept Malignancy

Concept Proximal Muscle Weakness

Concept Heliotrope Rash

Concept Anti-TIF-1? Antibody

Distinguished Non-Finalist

Presenter Organization Emory University

Presenting Author Karen A. Clarke

Track Adult

Keywords

anti-TIF-1-gamma antibody

dermatomyositis

malignancy risk

cancer screening

proximal muscle weakness

dysphagia

heliotrope rash

myositis-specific antibody

intravenous immunoglobulin

transvaginal ultrasound

Dermatomyositis

Anti-TIF-1γ Antibody

Malignancy

Proximal Muscle Weakness

Heliotrope Rash