Inflammatory Myopathies - Myopathies, Inflammatory

Idiopathic inflammatory myopathies (IIM) are a group of chronic autoimmune disorders characterized by inflammation and degeneration of skeletal muscles. The original Bohan and Peter criteria classify inflammatory myopathies into dermatomyositis (DM), polymyositis (PM), and inclusion body myositis (IBM). Newer subtypes include necrotizing autoimmune myositis and overlap myositis (Dalakas, 2015).

  • Diagnosis
  • Algorithms
  • Screening
  • Monitoring
  • Background
  • Pediatrics
  • Lab Tests
  • References
  • Related Topics

Indications for Testing

  • Progressive muscle weakness (usually proximal) 
  • Symmetrical or asymmetrical proximal muscle weakness after more common etiologies have been ruled out

Criteria for Diagnosis

  • See Clinical Background section for inflammatory myopathies and definitions

Laboratory Testing

  • Myositis antibody testing
    • Myositis-specific antibodies (MSA) may be useful in confirming a diagnosis of PM, DM or necrotizing autoimmune myopathies
    • Myositis-associated antibodies (MAA) are present in patients with IM and/or other CTD or overlap syndromes


  • Muscle biopsy – gold standard for diagnosis
    • Usually performed on proximal leg muscles but should not be performed in end-stage muscles
      • MRI may be helpful in choosing muscle
    • Open biopsy preferred – larger sample
    • Findings on muscle biopsy are usually diagnostic for specific subtypes

Other Testing

  • EMG – changes consistent with myopathy, including increased spontaneous and insertional activity with fibrillation potential, complex repetitive discharges, positive repetitive discharges, positive sharp waves, early recruitment and small polyphasic motor unit potentials
    • Abnormal in 70-90% of patients
    • Not specific for IIM
    • Amyopathic DM may have subtle myopathy on EMG

Imaging Studies

  • Ultrasound – muscle edema with alteration of normal architecture
    • May visualize subcutaneous calcifications
  • CT – fatty infiltration suggests chronic disease
  • MRI – very sensitive for detection of muscle edema; often used to guide biopsy site

Differential Diagnosis

  • All adult patients with dermatomyositis (DM) should be evaluated for malignancy due to increased risk of malignancy
  • American Academy of Dermatology recommends reevaluation for malignancy every 6-12 months for first 2 years following diagnosis
  • Creatinine kinase myoglobin, and lactate dehydrogenase (LD) levels are most useful in monitoring therapeutic response
  • Necrotizing autoimmune myopathy
    • Decline in anti-HMGCR antibodies associated with treatment response 


  • Incidence – 4-10/million (overall, all subtypes)
  • Age
    • DM – bimodal peaks
      • Childhood
      • 50-70 years
    • PM – age of onset typically >20 years
    • IBM – >50 years
    • Autoimmune necrotizing myositis – primarily adults, often older
  • Sex
    • DM and PM – M<F; 1:2
    • IBM – M>F; 2:1
  • Ethnicity
    • DM – unknown
    • PM – some studies suggest higher prevalence in African Americans compared to general U.S. population
    • IBM – higher prevalence in Caucasians


  • DM – microangiopathy affecting skin and muscle with deposition of complement-causing lysis of endomysial capillaries and muscle ischemia
  • PM and IBM – T-cells invade muscle fibers, leading to necrosis
  • Autoimmune necrotizing myositis – scattered necrotic myofiber with myophagocytosis, absence or paucity of T cells

Clinical Presentation

Clinical Background


  • Incidence – 2-3/million (rare)
  • Age
    • Dermatomyositis (DM) – more common in children
      • Mean onset is 7 years (25% present at <4 years)
    • Polymyositis (PM) – rare in children
    • Juvenile myositis (JM) – children 2-18 years
  • Sex – M<F, 1:2.3
  • Ethnicity
    • JDM – Caucasians
    • JPM – African Americans


  • Juvenile dermatomyositis (JDM)
  • Juvenile polymyositis (JPM)
  • Juvenile connective tissue disease myositis (JCTM)

Clinical Presentation


Indications for Testing

  • Symmetrical and proximal muscle weakness

Laboratory Testing

  • Initial screening tests – see adult laboratory testing
  • Muscle biopsy – less frequent in children so antibodies are important

Differential Diagnosis

Tests generally appear in the order most useful for common clinical situations. Click on number for test-specific information in the ARUP Laboratory Test Directory.

Nuclear Antibody (ANA) by IFA, IgG 0050639
Method: Semi-Quantitative Indirect Fluorescent Antibody


A negative ANA by IFA test does not rule out the presence of connective tissue disease 

Anti-Nuclear Antibodies (ANA), IgG by ELISA with Reflex to ANA, IgG by IFA 0050080
Method: Qualitative Enzyme-Linked Immunosorbent Assay/Semi-Quantitative Indirect Fluorescent Antibody


ANA ELISA assays have been reported to have lower sensitivities than ANA IFA for systemic autoimmune rheumatic diseases

Anti-Nuclear Antibody (ANA), IgG by IFA with Reflex by IFA Pattern 2008467
Method: Semi-Quantitative Indirect Fluorescent Antibody/Qualitative Enzyme-Linked Immunosorbent Assay/Semi-Quantitative Enzyme-Linked Immunosorbent Assay/Semi-Quantitative Multiplex Bead Assay/Qualitative Immunoblot


Only cytoplasmic, nuclear mitotic apparatus (NuMA), and/or nuclear dot pattern will be reported if observed; titers are not performed

Dual or mixed patterns will not be reflexed; additional testing for dual or mixed patterns should be determined by the ordering physician

A negative ANA by IFA test does not rule out the presence of connective tissue disease 

Polymyositis and Dermatomyositis Panel 2013992
Method: Qualitative Immunoprecipitation/Semi-Quantitative Multiplex Bead Assay/Qualitative Immunoblot


Results by themselves are not diagnostic; strong clinical correlation is recommended

Negative results do not rule out a diagnosis of inflammatory myopathy or overlap syndrome

Polymyositis Panel 2013990
Method: Qualitative Immunoprecipitation/Semi-Quantitative Multiplex Bead Assay

Dermatomyositis Panel 2013991
Method: Qualitative Immunoprecipitation/Qualitative Immunoblot

Interstitial Lung Disease Panel 2013993
Method: Qualitative Immunoprecipitation/Semi-Quantitative Multiplex Bead Assay/Qualitative Immunoblot/Semi-Quantitative Enzyme-Linked Immunosorbent Assay/Quantitative Immunoturbidimetry

Myositis Extended Panel 2013961
Method: Qualitative Immunoprecipitation/Semi-Quantitative Multiplex Bead Assay/Qualitative Immunoblot


Results by themselves are not diagnostic; strong clinical correlation is recommended

Negative results do not rule out a diagnosis of inflammatory myopathy or overlap syndromes 

3-Hydroxy-3-Methylglutaryl Coenzyme A Reductase (HMGCR) Antibody, IgG 2013101
Method: Semi-Quantitative Enzyme-Linked Immunosorbent Assay


Diagnostic relevance in a minor subset of patients with inflammatory myopathy

Results should be used in conjunction with clinical findings, muscle biopsy and other relevant laboratory tests for disease evaluation

Negative results do not rule out IM, necrotizing autoimmune myopathy, or statin-associated myopathy



Choosing Wisely. An initiative of the ABIM Foundation. [Accessed: Nov 2017]

General References

Ancuta C, Ancuta E, Chirieac R. Aminoacyl-tRNA Synthetases in Idiopathic Inflammatory Myopathies: An Update on Immunopathogenic Significance, Clinical and Therapeutic Implications. In: Idiopathic Inflammatory Myopathies - Recent Developments, Gran, JT, editor. InTech. [Accessed: Oct 2017]

Carstens P, Schmidt J. Diagnosis, pathogenesis and treatment of myositis: recent advances. Clin Exp Immunol. 2014; 175(3): 349-58. PubMed

Casciola-Rosen L, Mammen AL. Myositis autoantibodies. Curr Opin Rheumatol. 2012; 24(6): 602-8. PubMed

Catalán M, Selva-O'Callaghan A, Grau JM. Diagnosis and classification of sporadic inclusion body myositis (sIBM). Autoimmun Rev. 2014; 13(4-5): 363-6. PubMed

Chatterjee S, Prayson R, Farver C. Antisynthetase syndrome: not just an inflammatory myopathy. Cleve Clin J Med. 2013; 80(10): 655-66. PubMed

Dalakas MC. Inflammatory muscle diseases. N Engl J Med. 2015; 372(18): 1734-47. PubMed

Dimachkie MM, Barohn RJ, Amato AA. Idiopathic inflammatory myopathies. Neurol Clin. 2014; 32(3): 595-628, vii. PubMed

Ernste FC, Reed AM. Idiopathic inflammatory myopathies: current trends in pathogenesis, clinical features, and up-to-date treatment recommendations. Mayo Clin Proc. 2013; 88(1): 83-105. PubMed

Ernste FC, Reed AM. Recent advances in juvenile idiopathic inflammatory myopathies. Curr Opin Rheumatol. 2014; 26(6): 671-8. PubMed

Feldman BM, Rider LG, Reed AM, Pachman LM. Juvenile dermatomyositis and other idiopathic inflammatory myopathies of childhood. Lancet. 2008; 371(9631): 2201-12. PubMed

Gunawardena H, Betteridge ZE, McHugh NJ. Myositis-specific autoantibodies: their clinical and pathogenic significance in disease expression. Rheumatology (Oxford). 2009; 48(6): 607-12. PubMed

Hamann PD, Cooper RG, McHugh NJ, Chinoy H. Statin-induced necrotizing myositis - a discrete autoimmune entity within the "statin-induced myopathy spectrum". Autoimmun Rev. 2013; 12(12): 1177-81. PubMed

Harris BT, Mohila CA. Essential muscle pathology for the rheumatologist. Rheum Dis Clin North Am. 2011; 37(2): 289-308, vii. PubMed

Huber AM. Idiopathic inflammatory myopathies in childhood: current concepts. Pediatr Clin North Am. 2012; 59(2): 365-80. PubMed

Khan S, Christopher-Stine L. Polymyositis, dermatomyositis, and autoimmune necrotizing myopathy: clinical features. Rheum Dis Clin North Am. 2011; 37(2): 143-58, v. PubMed

Lazarou IN, Guerne P. Classification, diagnosis, and management of idiopathic inflammatory myopathies. J Rheumatol. 2013; 40(5): 550-64. PubMed

Lloyd TE, Christopher-Stine L, Pinal-Fernandez I, Tiniakou E, Petri M, Baer A, Danoff SK, Pak K, Casciola-Rosen LA, Mammen AL. Cytosolic 5'-Nucleotidase 1A As a Target of Circulating Autoantibodies in Autoimmune Diseases. Arthritis Care Res (Hoboken). 2016; 68(1): 66-71. PubMed

Lynch MC, Cohen JA. A primer on electrophysiologic studies in myopathy. Rheum Dis Clin North Am. 2011; 37(2): 253-68, vii. PubMed

Mahler M, Miller FW, Fritzler MJ. Idiopathic inflammatory myopathies and the anti-synthetase syndrome: a comprehensive review. Autoimmun Rev. 2014; 13(4-5): 367-71. PubMed

Mammen AL, Chung T, Christopher-Stine L, Rosen P, Rosen A, Doering KR, Casciola-Rosen LA. Autoantibodies against 3-hydroxy-3-methylglutaryl-coenzyme A reductase in patients with statin-associated autoimmune myopathy. Arthritis Rheum. 2011; 63(3): 713-21. PubMed

Mammen AL, Gaudet D, Brisson D, Christopher-Stine L, Lloyd TE, Leffell MS, Zachary AA. Increased frequency of DRB1*11:01 in anti-hydroxymethylglutaryl-coenzyme A reductase-associated autoimmune myopathy. Arthritis Care Res (Hoboken). 2012; 64(8): 1233-7. PubMed

Mammen AL. Statin-Associated Autoimmune Myopathy. N Engl J Med. 2016; 374(7): 664-9. PubMed

Nasr R, Reed AM, Peterson EJ. Update: biomarkers for idiopathic inflammatory myopathies. Curr Opin Rheumatol. 2012; 24(6): 609-15. PubMed

Solomon J, Swigris JJ, Brown KK. Myositis-related interstitial lung disease and antisynthetase syndrome. J Bras Pneumol. 2011; 37(1): 100-9. PubMed

Solorzano GE, Phillips LH. Inclusion body myositis: diagnosis, pathogenesis, and treatment options. Rheum Dis Clin North Am. 2011; 37(2): 173-83, v. PubMed

van der Kooi AJ, de Visser M. Idiopathic inflammatory myopathies. Handb Clin Neurol. 2014; 119: 495-512. PubMed

References from the ARUP Institute for Clinical and Experimental Pathology®

Jaskowski TD, Schroder C, Martins TB, Mouritsen L, Hill HR. Comparison of three commercially available enzyme immunoassays for the screening of autoantibodies to extractable nuclear antigens. J Clin Lab Anal. 1995; 9(3): 166-72. PubMed

Tebo AE. Commentary. Clin Chem. 2015; 61(9): 1136-7. PubMed

Medical Reviewers

Last Update: November 2017