# Pleuroparenchymal fibroelastosis associated with anti-Ku antibody-positive polymyositis: A case report with prominent systemic muscle atrophy and review of five cases

**Authors:** Yusuke Sakaue, Ryosuke Hanaoka

PMC · DOI: 10.1016/j.rmcr.2026.102383 · Respiratory Medicine Case Reports · 2026-02-06

## Full-text entities

- **Genes:** CMPK1 (cytidine/uridine monophosphate kinase 1) [NCBI Gene 51727] {aka CK, CMK, CMPK, UMK, UMP-CMPK, UMPK}, MUC1 (mucin 1, cell surface associated) [NCBI Gene 4582] {aka ADMCKD, ADMCKD1, ADTKD2, CA 15-3, CD227, Ca15-3}, ALB (albumin) [NCBI Gene 213] {aka FDAHT, HSA, PRO0883, PRO0903, PRO1341}, LTBP4 (latent transforming growth factor beta binding protein 4) [NCBI Gene 8425] {aka ARCL1C, LTBP-4, LTBP4L, LTBP4S}, TRIM21 (tripartite motif containing 21) [NCBI Gene 6737] {aka RNF81, RO52, Ro/SSA, SSA, SSA1, TRIM21/Ro52}, MB (myoglobin) [NCBI Gene 4151] {aka MYOSB, PVALB}, SFTPD (surfactant protein D) [NCBI Gene 6441] {aka COLEC7, PSP-D, SFTP4, SP-D}
- **Diseases:** Aspergillus infection (MESH:D001228), pulmonary restriction (MESH:D002313), chronic (MESH:D002908), CTD-ILD (MESH:D017563), muscle loss (MESH:D009135), Raynaud's phenomenon (MESH:D011928), systemic (MESH:D015619), emaciation (MESH:D004614), UIP (MESH:D054990), air (MESH:D004618), COVID-19 (MESH:D000086382), Atrial fibrillation (MESH:D001281), pneumothorax (MESH:D011030), polymyositis (MESH:D017285), weight loss (MESH:D015431), PPFE (MESH:D004695), hypoproteinemia (MESH:D007019), arthritis (MESH:D001168), death (MESH:D003643), connective tissue disease (MESH:D003240), malnutrition (MESH:D044342), traction bronchiectasis (MESH:D001987), metabolic abnormalities (MESH:D008659), lupus (MESH:D008180), scleroderma (MESH:D012595), hypoxia (MESH:D000860), dermatomyositis (MESH:D003882), fasciculation (MESH:D005207), air trapping (MESH:C536657), hypercapnic (MESH:D012131), pneumomediastinum (MESH:D008478), autoimmune diseases (MESH:D001327), hypersensitivity pneumonitis (MESH:D000542), inflammatory infiltrates (MESH:D017254), cachexia (MESH:D002100), inflammatory myopathies (MESH:D009220), hypoalbuminemia (MESH:D034141), hypercapnia (MESH:D006935), pleural elastosis (MESH:D005148), pleural thickening (MESH:D010995), dyspnea (MESH:D004417), lung volumes (MESH:D008171), limb weakness (MESH:D018908), emphysema (MESH:D004646), anorexia (MESH:D000855), atrophy (MESH:D001284), lung injury (MESH:D055370), Chronic inflammation (MESH:D007249), intercostal muscle atrophy (MESH:D009133), fatty replacement (MESH:D008067), fibrosis (MESH:D005355), volume loss (MESH:D016388), muscle involvement (MESH:C566343), mitochondrial dysfunction (MESH:D028361), nontuberculous mycobacterial infection (MESH:D009165), mitochondrial myopathy (MESH:D017240), syndrome (MESH:D013577)
- **Chemicals:** eosin (MESH:D004801), nintedanib (MESH:C530716), carbon dioxide (MESH:D002245), mizoribine (MESH:C010052), pirfenidone (MESH:C093844), prednisolone (MESH:D011239), tacrolimus (MESH:D016559), cyclosporine (MESH:D016572), Hematoxylin (MESH:D006416), cyclophosphamide (MESH:D003520), edoxaban (MESH:C552171), lactate (MESH:D019344), azathioprine (MESH:D001379), tofacitinib (MESH:C479163), mycophenolate mofetil (MESH:D009173), pyruvate (MESH:D019289)
- **Species:** Homo sapiens (human, species) [taxon 9606]

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## References

12 references — full list in the complete paper: https://tomesphere.com/paper/PMC12927088/full.md

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Source: https://tomesphere.com/paper/PMC12927088