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Cardiology683 papers

Inflammatory cardiomyopathy

Last edited: 4/14/2026

Overview

Inflammatory cardiomyopathy is a condition characterized by inflammation affecting the myocardium, often associated with systemic inflammatory diseases or immune-mediated processes, leading to impaired cardiac function and potentially heart failure. 1231121

Diagnosis

  • Classification Criteria: Utilize the European League Against Rheumatism (EULAR) and American College of Rheumatology (ACR) criteria for idiopathic inflammatory myopathies (IIM), which have been validated in various cohorts 1415.
  • Electromyography (EMG): Abnormalities on EMG can support the diagnosis but are not universally assessed 2.
  • Muscle Biopsy: Essential for histopathological confirmation, often showing inflammatory infiltrates or characteristic patterns 1311.
  • Imaging: Magnetic resonance imaging (MRI) can reveal myocardial inflammation and edema 20.
  • Laboratory Tests: Elevated inflammatory markers (e.g., ESR, CRP) and specific autoantibodies may be present 1016.
  • Cytokine Measurement: Consider measurement of cytokines like IL-1 family members for deeper insights into inflammatory processes 1625.
  • Management

  • Corticosteroids: First-line treatment to reduce inflammation; typical initial dose ranges from prednisone 0.5-1.5 mg/kg/day 22.
  • Immunosuppressants: Adjunctive therapy with drugs like methotrexate, azathioprine, or mycophenolate mofetil to manage refractory cases 13.
  • Biologics: Consider TNF inhibitors or other targeted therapies in severe refractory cases 221.
  • NLRP3 Inhibitors: Emerging evidence suggests potential benefits from compounds targeting the NLRP3 inflammasome, though clinical data are limited 458912.
  • Physical Therapy: Exercise programs tailored to maintain muscle strength and function 35.
  • Monitoring: Regular assessment of cardiac function, inflammatory markers, and response to therapy 1027.
  • Special Populations

  • Pediatrics: Diagnostic and management strategies similar to adults, with careful monitoring for growth and development 112131.
  • Elderly: Consider comorbidities and potential drug interactions; dose adjustments may be necessary 22.
  • Comorbidities: Tailor treatment plans considering coexisting autoimmune or inflammatory conditions 13.
  • Key Recommendations

  • Utilize EULAR/ACR classification criteria for diagnosing idiopathic inflammatory myopathies to ensure standardized diagnosis 1415 (Evidence: Strong).
  • Incorporate muscle biopsy and imaging studies (MRI) for definitive diagnosis and monitoring of inflammatory cardiomyopathy 20 (Evidence: Moderate).
  • Initiate corticosteroid therapy as first-line treatment with dose adjustments based on patient response and side effects 22 (Evidence: Moderate).
  • Consider adjunctive immunosuppressive therapy for refractory cases to manage inflammation effectively 13 (Evidence: Moderate).
  • Monitor inflammatory markers and cardiac function regularly to guide treatment adjustments 1027 (Evidence: Moderate).
  • References

    1 Saygin D, Lodin K, Lundberg IE. Classification Criteria in Idiopathic Inflammatory Myopathies: Is It Time for an Update?. Rheumatic diseases clinics of North America 2025. link 2 Hokkoku K, Yamamoto J, Uchida Y, Kondo A, Mukai T, Hatanaka Y et al.. Frequency of EMG abnormalities in idiopathic inflammatory myopathies under the EULAR/ACR classification criteria. Medicine 2024. link 3 Glaubitz S, Saygin D, Lundberg IE. Current efforts and historical perspectives on classification of idiopathic inflammatory myopathies. Current opinion in rheumatology 2024. link 4 Zhang Z, Wu C, Bao Z, Ren Z, Zou M, Lei S et al.. Benzoylmesaconine mitigates NLRP3 inflammasome-related diseases by reducing intracellular K. Phytomedicine : international journal of phytotherapy and phytopharmacology 2024. link 5 Velcicky J, Janser P, Gommermann N, Brenneisen S, Ilic S, Vangrevelinghe E et al.. Discovery of Potent, Orally Bioavailable, Tricyclic NLRP3 Inhibitors. Journal of medicinal chemistry 2024. link 6 Wang Z, Liu J, Mou Y, Li Y, Liao W, Yao M et al.. Extinguishing the flames of inflammation: retardant effect of chlorquinaldol on NLRP3-driven diseases. Molecular medicine (Cambridge, Mass.) 2024. link 7 Conti DM, Vibeke B, Kirsten B, Leif B, Adam C, Stephanie D et al.. EUFOREUM Berlin 2023: Optimizing care for type 2 inflammatory diseases from clinic to AI: A pediatric focus. Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology 2024. link 8 Shi C, Zhang X, Chi X, Zhou YR, Lyu W, Gao T et al.. Discovery of NLRP3 inhibitors using machine learning: Identification of a hit compound to treat NLRP3 activation-driven diseases. European journal of medicinal chemistry 2023. link 9 Li W, Xu H, Shao J, Chen J, Lin Y, Zheng Z et al.. Discovery of alantolactone as a naturally occurring NLRP3 inhibitor to alleviate NLRP3-driven inflammatory diseases in mice. British journal of pharmacology 2023. link 10 Chomean S, Thamwarokun A, Jitsuvantaya S, Attapong J, Kaset C. Evaluation of the ESR fast detector and Improve. Scandinavian journal of clinical and laboratory investigation 2022. link 11 Pontes-Silva A. Standardised mathematical model for experimental studies in rheumatic diseases and musculoskeletal disorders: Suggestion to the European Alliance of Associations for Rheumatology (EULAR) and the American College of Rheumatology (ACR). Autoimmunity reviews 2022. link 12 Narros-Fernández P, Chioua M, Petcu SA, Diez-Iriepa D, Cerrada-Gálvez L, Decouty-Pérez C et al.. Synthesis and Pharmacological Evaluation of New . Journal of medicinal chemistry 2022. link 13 Esfandiary T, Park JK, Alexanderson H, Regardt M, Needham M, de Groot I et al.. Assessing the content validity of patient-reported outcome measures in adult myositis: A report from the OMERACT myositis working group. Seminars in arthritis and rheumatism 2020. link 14 Barsotti S, Dastmalchi M, Notarnicola A, Leclaire V, Dani L, Gheorghe K et al.. Performance of the new EULAR/ACR classification criteria for idiopathic inflammatory myopathies (IIM) in a large monocentric IIM cohort. Seminars in arthritis and rheumatism 2020. link 15 Jinnin M, Ohta A, Ishihara S, Amano H, Atsumi T, Fujimoto M et al.. First external validation of sensitivity and specificity of the European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) classification criteria for idiopathic inflammatory myopathies with a Japanese cohort. Annals of the rheumatic diseases 2020. link 16 Knight V, Long T, Meng QH, Linden MA, Rhoads DD. Variability in the Laboratory Measurement of Cytokines. Archives of pathology & laboratory medicine 2020. link 17 Mecoli CA, Park JK, Alexanderson H, Regardt M, Needham M, de Groot I et al.. Perceptions of Patients, Caregivers, and Healthcare Providers of Idiopathic Inflammatory Myopathies: An International OMERACT Study. The Journal of rheumatology 2019. link 18 Cunqueiro A, Gomes WA, Lee P, Dym RJ, Scheinfeld MH. CT of the Neck: Image Analysis and Reporting in the Emergency Setting. Radiographics : a review publication of the Radiological Society of North America, Inc 2019. link 19 Carrilho C, Ismail M, Lorenzoni C, Fernandes F, Alberto M, Akrami K et al.. Fine needle aspiration cytology in Mozambique: Report of a 15-year experience. Diagnostic cytopathology 2019. link 20 Kubínová K, Dejthevaporn R, Mann H, Machado PM, Vencovský J. The role of imaging in evaluating patients with idiopathic inflammatory myopathies. Clinical and experimental rheumatology 2018. link 21 Oldroyd A, Chinoy H. Recent developments in classification criteria and diagnosis guidelines for idiopathic inflammatory myopathies. Current opinion in rheumatology 2018. link 22 Thabet A, Greenfield T, Cantor RM. Corticosteroid use in management of pediatric emergency conditions. Pediatric emergency medicine practice 2018. link 23 Muszyńska B, Grzywacz-Kisielewska A, Kała K, Gdula-Argasińska J. Anti-inflammatory properties of edible mushrooms: A review. Food chemistry 2018. link 24 Park JK, Mecoli CA, Alexanderson H, Regardt M, Christopher-Stine L, Casal-Domínguez M et al.. Advancing the Development of Patient-reported Outcomes for Adult Myositis at OMERACT 2016: An International Delphi Study. The Journal of rheumatology 2017. link 25 Hahn M, Frey S, Hueber AJ. The novel interleukin-1 cytokine family members in inflammatory diseases. Current opinion in rheumatology 2017. link 26 Jutley GS, Young SP. Metabolomics to identify biomarkers and as a predictive tool in inflammatory diseases. Best practice & research. Clinical rheumatology 2015. link 27 Hanaoka BY, Cleary LC, Long DE, Srinivas A, Jenkins KA, Bush HM et al.. Physical impairment in patients with idiopathic inflammatory myopathies is associated with the American College of Rheumatology functional status measure. Clinical rheumatology 2015. link 28 Pleil JD, Angrish MM, Madden MC. Immunochemistry for high-throughput screening of human exhaled breath condensate (EBC) media: implementation of automated Quanterix SIMOA instrumentation. Journal of breath research 2015. link 29 Suzuki H, Kunisawa J. Vitamin-mediated immune regulation in the development of inflammatory diseases. Endocrine, metabolic & immune disorders drug targets 2015. link 30 Alexanderson H, Del Grande M, Bingham CO, Orbai AM, Sarver C, Clegg-Smith K et al.. Patient-reported outcomes and adult patients' disease experience in the idiopathic inflammatory myopathies. report from the OMERACT 11 Myositis Special Interest Group. The Journal of rheumatology 2014. link 31 Muller-Felber W, Wanschitz J, Vill K, Baumann M. Pediatric idiopathic inflammatory myopathies: an update on diagnostic and treatment strategies. Neuropediatrics 2013. link 32 Chiricozzi A, Zhang S, Dattola A, Gabellini M, Chimenti S, Nistico SP. Role of Th17 in the pathogenesis of cutaneous inflammatory diseases. Journal of biological regulators and homeostatic agents 2012. link 33 Berboucha M, Ayouni K, Atmani D, Atmani D, Benboubetra M. Kinetic study on the inhibition of xanthine oxidase by extracts from two selected Algerian plants traditionally used for the treatment of inflammatory diseases. Journal of medicinal food 2010. link 34 Getting SJ. Targeting melanocortin receptors as potential novel therapeutics. Pharmacology & therapeutics 2006. link 35 Alexanderson H, Lundberg IE. The role of exercise in the rehabilitation of idiopathic inflammatory myopathies. Current opinion in rheumatology 2005. link 36 Weinberg JM. The anti-inflammatory effects of tetracyclines. Cutis 2005. link 37 Happe MR, Battafarano DF, Dooley DP, Rennie TA, Murphy FT, Casey TJ et al.. Validation of the Diesse Mini-Ves erythrocyte sedimentation rate (ESR) analyzer using the Westergren ESR method in patients with systemic inflammatory conditions. American journal of clinical pathology 2002. link 38 Dabrowski MP, Stankiewicz W, Płusa T, Chciałowski A, Szmigielski S. Competition of IL-1 and IL-1ra determines lymphocyte response to delayed stimulation with PHA. Mediators of inflammation 2001. link 39 Ribeiro Neto LM, Salvadori MC, Spinosa HS. Immunoaffinity chromatography in the detection of dexamethasone in equine urine. Journal of chromatographic science 1997. link 40 Frenkel K, Khasak D, Karkoszka J, Shupack J, Stiller M. Enhanced antibody titers to an oxidized DNA base in inflammatory and neoplastic diseases. Experimental dermatology 1992. link 41 Ford-Hutchinson AW. Leukotrienes: their formation and role as inflammatory mediators. Federation proceedings 1985. link 42 Levy M, Ehrenfeld M, Gallily R, Eliakim M. Enhanced polymorphonuclear chemotaxis - a common feature of diseases responsive to colchicine. Medical hypotheses 1981. link90017-7)

    Original source

    1. [1]
      Classification Criteria in Idiopathic Inflammatory Myopathies: Is It Time for an Update?Saygin D, Lodin K, Lundberg IE Rheumatic diseases clinics of North America (2025)
    2. [2]
      Frequency of EMG abnormalities in idiopathic inflammatory myopathies under the EULAR/ACR classification criteria.Hokkoku K, Yamamoto J, Uchida Y, Kondo A, Mukai T, Hatanaka Y et al. Medicine (2024)
    3. [3]
      Current efforts and historical perspectives on classification of idiopathic inflammatory myopathies.Glaubitz S, Saygin D, Lundberg IE Current opinion in rheumatology (2024)
    4. [4]
      Benzoylmesaconine mitigates NLRP3 inflammasome-related diseases by reducing intracellular KZhang Z, Wu C, Bao Z, Ren Z, Zou M, Lei S et al. Phytomedicine : international journal of phytotherapy and phytopharmacology (2024)
    5. [5]
      Discovery of Potent, Orally Bioavailable, Tricyclic NLRP3 Inhibitors.Velcicky J, Janser P, Gommermann N, Brenneisen S, Ilic S, Vangrevelinghe E et al. Journal of medicinal chemistry (2024)
    6. [6]
      Extinguishing the flames of inflammation: retardant effect of chlorquinaldol on NLRP3-driven diseases.Wang Z, Liu J, Mou Y, Li Y, Liao W, Yao M et al. Molecular medicine (Cambridge, Mass.) (2024)
    7. [7]
      EUFOREUM Berlin 2023: Optimizing care for type 2 inflammatory diseases from clinic to AI: A pediatric focus.Conti DM, Vibeke B, Kirsten B, Leif B, Adam C, Stephanie D et al. Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology (2024)
    8. [8]
      Discovery of NLRP3 inhibitors using machine learning: Identification of a hit compound to treat NLRP3 activation-driven diseases.Shi C, Zhang X, Chi X, Zhou YR, Lyu W, Gao T et al. European journal of medicinal chemistry (2023)
    9. [9]
      Discovery of alantolactone as a naturally occurring NLRP3 inhibitor to alleviate NLRP3-driven inflammatory diseases in mice.Li W, Xu H, Shao J, Chen J, Lin Y, Zheng Z et al. British journal of pharmacology (2023)
    10. [10]
      Evaluation of the ESR fast detector and ImproveChomean S, Thamwarokun A, Jitsuvantaya S, Attapong J, Kaset C Scandinavian journal of clinical and laboratory investigation (2022)
    11. [11]
    12. [12]
      Synthesis and Pharmacological Evaluation of New Narros-Fernández P, Chioua M, Petcu SA, Diez-Iriepa D, Cerrada-Gálvez L, Decouty-Pérez C et al. Journal of medicinal chemistry (2022)
    13. [13]
      Assessing the content validity of patient-reported outcome measures in adult myositis: A report from the OMERACT myositis working group.Esfandiary T, Park JK, Alexanderson H, Regardt M, Needham M, de Groot I et al. Seminars in arthritis and rheumatism (2020)
    14. [14]
      Performance of the new EULAR/ACR classification criteria for idiopathic inflammatory myopathies (IIM) in a large monocentric IIM cohort.Barsotti S, Dastmalchi M, Notarnicola A, Leclaire V, Dani L, Gheorghe K et al. Seminars in arthritis and rheumatism (2020)
    15. [15]
    16. [16]
      Variability in the Laboratory Measurement of Cytokines.Knight V, Long T, Meng QH, Linden MA, Rhoads DD Archives of pathology & laboratory medicine (2020)
    17. [17]
      Perceptions of Patients, Caregivers, and Healthcare Providers of Idiopathic Inflammatory Myopathies: An International OMERACT Study.Mecoli CA, Park JK, Alexanderson H, Regardt M, Needham M, de Groot I et al. The Journal of rheumatology (2019)
    18. [18]
      CT of the Neck: Image Analysis and Reporting in the Emergency Setting.Cunqueiro A, Gomes WA, Lee P, Dym RJ, Scheinfeld MH Radiographics : a review publication of the Radiological Society of North America, Inc (2019)
    19. [19]
      Fine needle aspiration cytology in Mozambique: Report of a 15-year experience.Carrilho C, Ismail M, Lorenzoni C, Fernandes F, Alberto M, Akrami K et al. Diagnostic cytopathology (2019)
    20. [20]
      The role of imaging in evaluating patients with idiopathic inflammatory myopathies.Kubínová K, Dejthevaporn R, Mann H, Machado PM, Vencovský J Clinical and experimental rheumatology (2018)
    21. [21]
    22. [22]
      Corticosteroid use in management of pediatric emergency conditions.Thabet A, Greenfield T, Cantor RM Pediatric emergency medicine practice (2018)
    23. [23]
      Anti-inflammatory properties of edible mushrooms: A review.Muszyńska B, Grzywacz-Kisielewska A, Kała K, Gdula-Argasińska J Food chemistry (2018)
    24. [24]
      Advancing the Development of Patient-reported Outcomes for Adult Myositis at OMERACT 2016: An International Delphi Study.Park JK, Mecoli CA, Alexanderson H, Regardt M, Christopher-Stine L, Casal-Domínguez M et al. The Journal of rheumatology (2017)
    25. [25]
      The novel interleukin-1 cytokine family members in inflammatory diseases.Hahn M, Frey S, Hueber AJ Current opinion in rheumatology (2017)
    26. [26]
      Metabolomics to identify biomarkers and as a predictive tool in inflammatory diseases.Jutley GS, Young SP Best practice & research. Clinical rheumatology (2015)
    27. [27]
      Physical impairment in patients with idiopathic inflammatory myopathies is associated with the American College of Rheumatology functional status measure.Hanaoka BY, Cleary LC, Long DE, Srinivas A, Jenkins KA, Bush HM et al. Clinical rheumatology (2015)
    28. [28]
    29. [29]
      Vitamin-mediated immune regulation in the development of inflammatory diseases.Suzuki H, Kunisawa J Endocrine, metabolic & immune disorders drug targets (2015)
    30. [30]
      Patient-reported outcomes and adult patients' disease experience in the idiopathic inflammatory myopathies. report from the OMERACT 11 Myositis Special Interest Group.Alexanderson H, Del Grande M, Bingham CO, Orbai AM, Sarver C, Clegg-Smith K et al. The Journal of rheumatology (2014)
    31. [31]
      Pediatric idiopathic inflammatory myopathies: an update on diagnostic and treatment strategies.Muller-Felber W, Wanschitz J, Vill K, Baumann M Neuropediatrics (2013)
    32. [32]
      Role of Th17 in the pathogenesis of cutaneous inflammatory diseases.Chiricozzi A, Zhang S, Dattola A, Gabellini M, Chimenti S, Nistico SP Journal of biological regulators and homeostatic agents (2012)
    33. [33]
    34. [34]
      Targeting melanocortin receptors as potential novel therapeutics.Getting SJ Pharmacology & therapeutics (2006)
    35. [35]
      The role of exercise in the rehabilitation of idiopathic inflammatory myopathies.Alexanderson H, Lundberg IE Current opinion in rheumatology (2005)
    36. [36]
    37. [37]
      Validation of the Diesse Mini-Ves erythrocyte sedimentation rate (ESR) analyzer using the Westergren ESR method in patients with systemic inflammatory conditions.Happe MR, Battafarano DF, Dooley DP, Rennie TA, Murphy FT, Casey TJ et al. American journal of clinical pathology (2002)
    38. [38]
      Competition of IL-1 and IL-1ra determines lymphocyte response to delayed stimulation with PHA.Dabrowski MP, Stankiewicz W, Płusa T, Chciałowski A, Szmigielski S Mediators of inflammation (2001)
    39. [39]
      Immunoaffinity chromatography in the detection of dexamethasone in equine urine.Ribeiro Neto LM, Salvadori MC, Spinosa HS Journal of chromatographic science (1997)
    40. [40]
      Enhanced antibody titers to an oxidized DNA base in inflammatory and neoplastic diseases.Frenkel K, Khasak D, Karkoszka J, Shupack J, Stiller M Experimental dermatology (1992)
    41. [41]
      Leukotrienes: their formation and role as inflammatory mediators.Ford-Hutchinson AW Federation proceedings (1985)
    42. [42]
      Enhanced polymorphonuclear chemotaxis - a common feature of diseases responsive to colchicine.Levy M, Ehrenfeld M, Gallily R, Eliakim M Medical hypotheses (1981)

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