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Publisher: Peytchinski Publishing Ltd.
ISSN: 1312-773X (Online)
Issue: 2026, vol. 32, issue3
Subject Area: Medicine
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DOI: 10.5272/jimab.2026323.6964
Published online: 13 July 2026
Case report
J of IMAB. 2026 Jul-Sep;32(3):6964-6970
POLYMYOSITIS ASSOCIATED WITH PRIMARY BILIARY CIRRHOSIS AND OVARIAN CARCINOMA – CLINICAL CASE PRESENTATION AND LITERATURE REVIEW
Margarita Pironeva
, Kosta Kostov,
Clinic of Neurology, Medical Institute – Ministry of Interior, Sofia, Bulgaria.
ABSTRACT:
Polymyositis is an idiopathic inflammatory myopathy classified under a heterogeneous group of autoimmune diseases predominantly presenting with symmetrical proximal muscle weakness, weakness of neck muscles with the manifestation of a "dropped head" symptom, dysphagia, dysphonia; elevated serum levels of creatine kinase (CK), sometimes in conjunction with elevated liver enzymes; characteristic EMG findings and muscle biopsy. The presence of myositis-specific antibodies in more than half of the patients facilitates the definition of distinct clinical subtypes and aids in accurate diagnosis, with an associated higher risk of involvement of other organs and systems such as the lungs, heart, liver, etc., most commonly presenting as interstitial pulmonary fibrosis, cardiomyopathy, and significantly less frequently primary biliary cirrhosis. A twofold increase in malignancies has been established compared to the general population, or the manifestation of myositis can be presented as a paraneoplastic syndrome. The first-line treatment method is the administration of intravenous corticosteroids in moderate or high doses combined with cytostatic therapy, with rare necessity for intravenous immunoglobulin administration, while plasmapheresis is considered an inapplicable method. We report a case of polymyositis with characteristic clinical and serological findings, with severe respiratory failure, developing twice in the absence of interstitial pulmonary fibrosis, primarily secondary myocardial damage during disease activity, and the presence of asymptomatic ovarian cancer and primary biliary cirrhosis discovered during the course of the disease, demonstrating the best therapeutic effectiveness from plasmapheresis. Accurate diagnosis relies on clinical, electrophysiological, and immunological findings, posing diagnostic challenges and questions that require the collaborative efforts of a multidisciplinary team.
Keywords: polymyositis, myositis-specific antibodies, primary biliary cirrhosis, ovarian carcinoma, plasmapheresis,
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Please cite this article as: Pironeva M, Kostov K. Polymyositis Associated with Primary Biliary Cirrhosis and Ovarian Carcinoma – clinical case presentation and literature review. J of IMAB. 2026 Jul-Sep;32(3):6964-6970. [Crossref - 10.5272/jimab.2026323.6964]
Correspondence to: Margarita Pironeva, Neurology clinic, Medical Institute - Ministry of Interior, Sofia; 79, Skobelev Blvd., 1606 Sofia, Bulgaria. E-mail: mpironeva@gmail.com
REFERENCES:
1. Lundberg IE, Tjärnlund A, Bottai M, Werth VP, Pilkington C, Visser M, et al. 2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. Ann Rheum Dis. 2017 Dec;76(12):1955-1964. [PubMed]
2. Lundberg IE, Fujimoto M, Vencovsky J, Aggarwal R, Holmqvist M, Christopher-Stine L, et al. Idiopathic inflammatory myopathies. Nat Rev Dis Primers. 2021 Dec 2;7(1):86. [PubMed]
3. Postolova A, Chen JK, Chung L. Corticosteroids in Myositis and Scleroderma. RheumDisClin North Am. 2016 Feb;42(1):103-18, ix. [PubMed]
4. Dalakas MC. Inflammatory muscle diseases. N Eng JMed. 2015;372:1734–47. [PubMed]
5. Mammen AL. Autoimmune myopathies: autoantibodies, phenotypes and pathogenesis. Nat Rev Neurol. 2011 Jun 8;7(6):343-54. [PubMed]
6. Basuita M, Fidler LM. Myositis Antibodies and Interstitial Lung Disease. J Appl Lab Med. 2022 Jan 5;7(1):240-258. [PubMed]
7. Sahu R, Rathaur BP, Chaudhari TS, Shukla R, Malhotra KP. Laryngeal carcinoma presenting as polymyositis: A paraneoplastic syndrome. Ann Indian Acad Neurol. 2016 Jan-Mar;19(1):150–1. [PubMed]
8. Graus F, Delattre JY, Antoine JC, Dalmau J, Giometto B, Grisold W, et al. Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry. 2004 Aug;75(8):1135-40. [PubMed]
9. Ashton C, Paramalingam S, Stevenson B, Brusch A, Needham M. Idiopathic inflammatory myopathies: a review. Intern Med J. 2021 Jun;51(6):845-852. [PubMed]
10. Saito H, Takahashi A, Abe K, Okai K, Katsushima F, Monoe K, et al. Autoantibodies by line immunoassay in patients with primary biliary cirrhosis. Fukushima J Med Sci. 2012; 58(2):107-16. [PubMed]
11. Trivella J, John BV, Levy C. Primary biliary cholangitis: Epidemiology, prognosis, and treatment. Hepatol Commun. 2023 Jun 2; 7(6):e0179. [PubMed]
12.Younossi ZM, Bernstein D, Shiffman ML, Kwo P, Kim WR, Kowdley KV, et al. Diagnosis and Management of Primary Biliary Cholangitis. Am J Gastroenterol. 2019 Jan;114(1): 48-63. [PubMed]
13. Nagai A, Nagai T, Yaguchi H, Fujii S, Uwatoko H, Shirai S, et al. Clinical features of anti-mitochondrial M2 antibody-positive myositis: case series of 17 patients. JNeurol Sci. 2022 Nov;442:120391. [Crossref]
14. Pamfil C, Candrea E, Berki E, Popov H, Radu P, Rednic S. Primary biliarycirrhosis-autoimmune hepatitis overlap syndrome associated with dermatomyositis, autoimmune thyroiditis and antiphospholipid syndrome. J Gastrointestin Liver Dis. 2015 Mar;24(1):101-4. [PubMed]
15. Ledenko T, Sorić Hosman IS, Ćorić M, Gagro A. Case Report: Simultaneously Developed Amyopathic Dermatomyositis and Autoimmune Sclerosing Cholangitis - a Coincidenceor a Shared Immunopathogenesis. FrontImmunol. 2022 Feb 23;13:825799. [PubMed]
16.Zahr ZA, Baer AN. Malignancy in myositis. Curr Rheumatol Rep. 2011 Jun;13(3):208-15. [PubMed]
17. Ernste FC, Reed AM. Idiopathic inflammatory myopathies: current trends in pathogenesis, clinical features, and up-to-date treatment recommendations. Mayo Clin Proc. 2013 Jan;88(1):83-105. [PubMed]
18. Bartt RE, Topel JL. Chapter 50 - Autoimmune and Inflammatory Disorders. In: Textbook of Clinical Neurology. (Third Edition). Edit. Goetz CG. 2007; 1155-1184. [Crossref]
Received: 24 September 2025
Published online: 13 July 2026