Anti-transcription Intermediary Factor-1 Gamma Antibody-positive Dermatomyositis with Interstitial Lung Disease and Multisystem Involvement: A Case Report

ABSTRACT: Dermatomyositis (DM) is a type of inflammatory myopathy of unknown origin that is marked by weakness in the proximal muscles, skin manifestations, and involvement of other systems. The existence of anti-transcription intermediary factor-1 gamma (anti-TIF1 γ) antibodies identifies a specific subgroup linked to cancer, interstitial lung disease (ILD), and unusual clinical presentations. We present a rare and complex case of DM positive for anti-TIF1 γ antibodies, which manifested as inflammatory myopathy, ILD, neuropathy, symptoms of sicca syndrome, and gastrointestinal involvement in an elderly woman with various other health issues. A 69-year-old female patient with progressive weakness in her proximal muscles, joint pain, difficulty swallowing, dry eyes and mouth, a persistent cough, and weight loss. Laboratory tests showed anemia, raised inflammatory markers, a positive antinuclear antibody test, (RF) Rheumatoid Factor, (Anti-CCP) Anti-Cyclic Citrullinated Peptide, and a strongly positive anti-TIF1 γ antibody result. A magnetic resonance imaging of her bilateral thighs indicated signs of inflammatory myositis, while a high-resolution computed tomography (CT) scan of the chest revealed an ILD pattern that was non-(UIP) Usual Interstitial Pneumonia, suggesting the presence of follicular bronchiolitis. Nerve conduction tests indicated sensory-motor axonal neuropathy. A positron emission tomography-CT scan revealed no signs of metabolically active cancer. The patient was diagnosed with anti-TIF1 γ-positive DM featuring ILD and multiple system involvement and began immunosuppressive treatment. This case underscores the diagnostic challenges associated with anti-TIF1 γ DM, particularly in the absence of cancer, and highlights the necessity of a multidisciplinary care approach. Early recognition of antibody-specific manifestations is crucial for proper assessment, outcome prediction, and treatment.

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Journal
Annals of African Medicine
Published
2026-07-23
DOI
https://doi.org/10.4103/aam.aam_547_26
Primary Topic
Inflammatory Myopathies and Dermatomyositis
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article
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article

Anti-transcription Intermediary Factor-1 Gamma Antibody-positive Dermatomyositis with Interstitial Lung Disease and Multisystem Involvement: A Case Report

Nilesh Jagdale, Md. Amjad, Sachin Kamble
Annals of African Medicine
Inflammatory Myopathies and Dermatomyositis
article

Anti-transcription Intermediary Factor-1 Gamma Antibody-positive Dermatomyositis with Interstitial Lung Disease and Multisystem Involvement: A Case Report

Nilesh Jagdale, Md. Amjad, Sachin Kamble
article en

Abstract

ABSTRACT: Dermatomyositis (DM) is a type of inflammatory myopathy of unknown origin that is marked by weakness in the proximal muscles, skin manifestations, and involvement of other systems. The existence of anti-transcription intermediary factor-1 gamma (anti-TIF1 γ) antibodies identifies a specific subgroup linked to cancer, interstitial lung disease (ILD), and unusual clinical presentations. We present a rare and complex case of DM positive for anti-TIF1 γ antibodies, which manifested as inflammatory myopathy, ILD, neuropathy, symptoms of sicca syndrome, and gastrointestinal involvement in an elderly woman with various other health issues. A 69-year-old female patient with progressive weakness in her proximal muscles, joint pain, difficulty swallowing, dry eyes and mouth, a persistent cough, and weight loss. Laboratory tests showed anemia, raised inflammatory markers, a positive antinuclear antibody test, (RF) Rheumatoid Factor, (Anti-CCP) Anti-Cyclic Citrullinated Peptide, and a strongly positive anti-TIF1 γ antibody result. A magnetic resonance imaging of her bilateral thighs indicated signs of inflammatory myositis, while a high-resolution computed tomography (CT) scan of the chest revealed an ILD pattern that was non-(UIP) Usual Interstitial Pneumonia, suggesting the presence of follicular bronchiolitis. Nerve conduction tests indicated sensory-motor axonal neuropathy. A positron emission tomography-CT scan revealed no signs of metabolically active cancer. The patient was diagnosed with anti-TIF1 γ-positive DM featuring ILD and multiple system involvement and began immunosuppressive treatment. This case underscores the diagnostic challenges associated with anti-TIF1 γ DM, particularly in the absence of cancer, and highlights the necessity of a multidisciplinary care approach. Early recognition of antibody-specific manifestations is crucial for proper assessment, outcome prediction, and treatment.

Annals of African Medicine
Dr. D. Y. Patil Medical College, Hospital and Research Centre (IN), Dr. D.Y. Patil Vidyapeeth, Pune (IN)
Good health and well-being
Openalex Percentile: Top 9%
Inflammatory Myopathies and Dermatomyositis
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