Online first
Case report
Published online: 2024-05-27

open access

Page views 27
Article views/downloads 17
Get Citation

Connect on Social Media

Connect on Social Media

Myositis with creatinine phosphokinase activity over 13,500 U/L in the course of granulomatosis with polyangiitis

Mikołaj Kamiński1, Paweł Hrycaj1


This study presents a detailed account of an exceptional case of severe myositis, characterized by an initial creatine phosphokinase (CPK) level of 13,575 U/L, in a 66-year-old female patient with a seven-year history of granulomatosis with polyangiitis (GPA). The patient’s condition was marked by significant deterioration in overall health, characterized by muscle weakness in the upper and lower extremities that resulted in her becoming bedridden. Treatment with methylprednisolone was initiated, leading to a significant decrease in CPK levels, yet the patient's health saw only minor improvement. The patient did not have autoantibodies usually associated with idiopathic inflammatory myositis. The patient ultimately succumbed to complications arising from pneumonia and sepsis a month after admission. This case provides valuable insights into the diagnostic challenges and treatment complexities of severe myositis in patients with GPA.

Article available in PDF format

View PDF Download PDF file


  1. Sattui SE, Lally L. Localized Granulomatous with Polyangiitis (GPA): Varied Clinical Presentations and Update on Treatment. Curr Allergy Asthma Rep. 2020; 20(10): 56.
  2. Conticini E, d'Alessandro M, Al Khayyat SG, et al. Inflammatory muscle involvement in systemic vasculitis: A systematic review. Autoimmun Rev. 2022; 21(3): 103029.
  3. Kim MiY, Bae SY, Lee M, et al. A case of ANCA-associated vasculitis presenting with calf claudication. Rheumatol Int. 2012; 32(9): 2909–2912.
  4. Lacou M, Leroy M, Le Lan N, et al. Muscle biopsy in anti-neutrophil cytoplasmic antibody-associated vasculitis: diagnostic yield depends on anti-neutrophil cytoplasmic antibody type, sex and neutrophil count. Rheumatology (Oxford). 2021; 60(2): 699–707.
  5. Lee YM, Chung SW. Granulomatosis with Polyangiitis Restricted to the Back Muscle: The First Case Report. Medicina (Kaunas). 2020; 56(9).
  6. Kimbrough BA, Naddaf E, Warrington KJ, et al. Granulomatosis with polyangiitis presenting as obstructive uropathy and vasculitic myopathy. Rheumatology (Oxford). 2023; 62(4): e136–e137.
  7. Dutcher JS, Bui A, Ibe TA, et al. ANCA-associated vasculitis and severe proximal muscle weakness. Proc (Bayl Univ Med Cent). 2021; 34(3): 384–386.
  8. Hervier B, Durant C, Masseau A, et al. Use of muscle biopsies for diagnosis of systemic vasculitides. J Rheumatol. 2011; 38(3): 470–474.
  9. Figueiredo R, Pires Duro I, Marinho A, et al. Granulomatosis with Polyangiitis in Adolescence: Two Distinct Presentations. Case Rep Rheumatol. 2021; 2021: 6642910.
  10. Bijlsma JWJ, Hachulla E. Hrsg. EULAR textbook on rheumatic diseases. 3rd ed. BMJ Publishing Group, London 2018.