Mortality in Idiopathic
Inflammatory
Myopathies(IIM)
Adane Petros
Internal Medicine 3rd
year Resident
April, 2021
A 44 years old right handed male Presented with
Erythematous to violaceous papules over the extensor
joints of the hand with no muscle complaint.
P/E was pertinent for facial heliotrope rash and gottron
papules. with No neurological deficit on examination.
Case
Case 1
Which of the following is true
A. This patient has low risk for ILD in comparison with a patient with similar
presentation and proximal muscle weakness
B. Presence of either anti –jo-1 or anti- MDA5 antibody increases the risk of ILD in a
DM patient
C. DM has lower risk of malignancy and increased risk of ILD compared to PM
patient
D. Rhabdomyolysis is the commonest cause of death in such patients
 Introduction and Epidemiology
 Clinical features and causes
 Management approach
Outline of Presentation
716 newly diagnosed IIM matched in 1:10 with normal population
Mortality 31% in IIM group vs 12% in general population in 10 years
Mortality 9% in IIM group vs 1% in general population in 1 year
Epidemiology
Mortality in idiopathic inflammatory myopathy: results from a Swedish nationwide population-based
cohort study
Gerd Cecilie Dobloug etal
Pathophysiology
 Main causes of death
 Respiratory disease
 Malignancy
 Disease of circulatory system
 Connective tissue diseases
 Infection
Pathophysiology
 Risk factors
 Delay in treatment for six months after symptom onset
 Greater weakness at presentation
 The presence of dysphagia
 Respiratory muscle weakness
 Interstitial lung disease
 Associated malignancy
 Cardiac involvement
Pathophysiology
 Type of DM and PM
 Anti synthetase syndrome
 Amyopathic dermatomyositis
 Anti-MDA-5 antibodies
 Overlap syndromes
Pathophysiology
 Risk for Malignancy
 older age at disease onset
 dysphagia
 evidence of capillary damage on muscle biopsy
 cutaneous necrosis
 cutaneous leukocytoclastic vasculitis
Prevention and Management
 Careful surveillance of newly diagnosed patients with IIM for
 Cancer,
 Lung involvement, and
 Heart involvement,
 As well as careful monitoring of heart and lung function during
the first year of follow-up
 Disease activity scoring
 Thank You
 UpToDate online
 International Myositis Assessment and Clinical studies
group network
Harrison’s principle of internal medicine,20th
ed
 Review articles
 Adams Principles of Neurology
 Bradley’s Neurology
References

Best b presentation on Myopathy Mortality Adane.pptx

  • 1.
    Mortality in Idiopathic Inflammatory Myopathies(IIM) AdanePetros Internal Medicine 3rd year Resident April, 2021
  • 2.
    A 44 yearsold right handed male Presented with Erythematous to violaceous papules over the extensor joints of the hand with no muscle complaint. P/E was pertinent for facial heliotrope rash and gottron papules. with No neurological deficit on examination. Case
  • 3.
  • 4.
    Which of thefollowing is true A. This patient has low risk for ILD in comparison with a patient with similar presentation and proximal muscle weakness B. Presence of either anti –jo-1 or anti- MDA5 antibody increases the risk of ILD in a DM patient C. DM has lower risk of malignancy and increased risk of ILD compared to PM patient D. Rhabdomyolysis is the commonest cause of death in such patients
  • 5.
     Introduction andEpidemiology  Clinical features and causes  Management approach Outline of Presentation
  • 6.
    716 newly diagnosedIIM matched in 1:10 with normal population Mortality 31% in IIM group vs 12% in general population in 10 years Mortality 9% in IIM group vs 1% in general population in 1 year Epidemiology Mortality in idiopathic inflammatory myopathy: results from a Swedish nationwide population-based cohort study Gerd Cecilie Dobloug etal
  • 7.
    Pathophysiology  Main causesof death  Respiratory disease  Malignancy  Disease of circulatory system  Connective tissue diseases  Infection
  • 8.
    Pathophysiology  Risk factors Delay in treatment for six months after symptom onset  Greater weakness at presentation  The presence of dysphagia  Respiratory muscle weakness  Interstitial lung disease  Associated malignancy  Cardiac involvement
  • 9.
    Pathophysiology  Type ofDM and PM  Anti synthetase syndrome  Amyopathic dermatomyositis  Anti-MDA-5 antibodies  Overlap syndromes
  • 10.
    Pathophysiology  Risk forMalignancy  older age at disease onset  dysphagia  evidence of capillary damage on muscle biopsy  cutaneous necrosis  cutaneous leukocytoclastic vasculitis
  • 11.
    Prevention and Management Careful surveillance of newly diagnosed patients with IIM for  Cancer,  Lung involvement, and  Heart involvement,  As well as careful monitoring of heart and lung function during the first year of follow-up  Disease activity scoring
  • 12.
  • 13.
     UpToDate online International Myositis Assessment and Clinical studies group network Harrison’s principle of internal medicine,20th ed  Review articles  Adams Principles of Neurology  Bradley’s Neurology References

Editor's Notes