Anti-Synthetase Syndrome (PL-7 Positive) Presenting with Necrotizing Inflammatory Myositis and Right-Sided Pneumonia: A Diagnostic Challenge

Anti-Synthetase Syndrome (ASS) is an autoimmune disorder characterized by autoantibodies against aminoacyl- tRNA synthetases, including the rarer anti-PL-7 antibody. This variant is often associated with severe multi-system involvement, including necrotizing myopathy, interstitial lung disease (ILD), and non-erosive arthritis. The clinical manifestations can overlap with other systemic conditions, posing challenges in diagnosis and management. The case report describes about a 57-year-old woman with diabetes, hypertension, and morbid obesity was bedridden due to growing limb weakening and edema for a month. Her diagnosis included necrotizing inflammatory myositis, pneumonia, quadriparesis, hypokalemia, and anemia, along with Anti-Synthetase Syndrome (PL-7 positive). Imaging showed thoracic compressive myelopathy and myositis. Clinical improvement was achieved by the use of IVIG, corticosteroids, antibiotics, and supportive care.

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Publication Details

Published
2025-06-02
DOI
https://doi.org/10.38124/ijisrt/25may865
Primary Topic
Inflammatory Myopathies and Dermatomyositis
Type
article
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article

Anti-Synthetase Syndrome (PL-7 Positive) Presenting with Necrotizing Inflammatory Myositis and Right-Sided Pneumonia: A Diagnostic Challenge

T. Rama Rao, Vankodoth Sireesha, Gadila Sushma, Boddu Shirisha et al.
Inflammatory Myopathies and Dermatomyositis
article

Anti-Synthetase Syndrome (PL-7 Positive) Presenting with Necrotizing Inflammatory Myositis and Right-Sided Pneumonia: A Diagnostic Challenge

T. Rama Rao, Vankodoth Sireesha, Gadila Sushma, Boddu Shirisha, Mekala Sai Charitha
article en

Abstract

Anti-Synthetase Syndrome (ASS) is an autoimmune disorder characterized by autoantibodies against aminoacyl- tRNA synthetases, including the rarer anti-PL-7 antibody. This variant is often associated with severe multi-system involvement, including necrotizing myopathy, interstitial lung disease (ILD), and non-erosive arthritis. The clinical manifestations can overlap with other systemic conditions, posing challenges in diagnosis and management. The case report describes about a 57-year-old woman with diabetes, hypertension, and morbid obesity was bedridden due to growing limb weakening and edema for a month. Her diagnosis included necrotizing inflammatory myositis, pneumonia, quadriparesis, hypokalemia, and anemia, along with Anti-Synthetase Syndrome (PL-7 positive). Imaging showed thoracic compressive myelopathy and myositis. Clinical improvement was achieved by the use of IVIG, corticosteroids, antibiotics, and supportive care.

Openalex Percentile: Top 82%
Inflammatory Myopathies and Dermatomyositis
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