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


Authors : Vankodoth Sireesha; Gadila Sushma; Mekala Sai Charitha; Boddu Shirisha; Dr. T. Rama Rao

Volume/Issue : Volume 10 - 2025, Issue 5 - May


Google Scholar : https://tinyurl.com/4a4jf43d

DOI : https://doi.org/10.38124/ijisrt/25may865

Note : A published paper may take 4-5 working days from the publication date to appear in PlumX Metrics, Semantic Scholar, and ResearchGate.


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.

Keywords : Amino Acyl-tRNA Synthetases, Interstitial Lung Disease, Myositis, Anti PL-7.

References :

  1. Liu J, Nie N, Zhang R, Wang D, Lin Y, Chang H. Anti-synthetase syndrome with anti-PL-7 antibody positive in a child: a case report and literature review. Front Immunol. 2025 Mar 3;16:1525432. doi: 10.3389/fimmu.2025.1525432. PMID: 40098963; PMCID: PMC11911354.
  2. Esposito AC, Gige TC, Miot HA. Syndrome in question: antisynthetase syndrome (anti-PL-7). An Bras Dermatol. 2016 Sep-Oct;91(5):683–5. doi: 10.1590/abd1806-4841.20164449. PMID: 27828653; PMCID: PMC5087238.
  3. Labirua-Iturburu A, Selva-O'Callaghan A, Vincze M, et al. Anti-PL-7 (anti-threonyl-tRNA synthetase) antisynthetase syndrome: clinical manifestations in a series of patients from a European multicenter study (EUMYONET) and review of the literature. Medicine (Baltimore). 2012 Jul;91(4):206–11. doi: 10.1097/MD.0b013e318260977c. PMID: 22732951.
  4. Gonzalez-Gay MA, Garcia-Porrua C, Sanchez-Andrade A, et al. Anti-PL-7-positive antisynthetase syndrome: a study of 20 patients. Rheumatology (Oxford). 2013 Mar;52(3): 513–516. doi: 10.1093/rheumatology/kes327. PMID: 23243183.
  5. Matsushima M, Shimizu Y, Takahashi I, Sato K, Hirotani M, Kano T, Yabe I, Sasaki H. A case of anti-PL7 antibody positive myositis and a clinical and pathological review of the anti-synthetase syndrome. Rinsho Shinkeigaku. 2015;55(11):810–5. doi: 10.5692/clinicalneurol.cn-000701. PMID: 26458569.

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.

Keywords : Amino Acyl-tRNA Synthetases, Interstitial Lung Disease, Myositis, Anti PL-7.

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