Difference between revisions of "Dermatomyositis"

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[[File:Heliotrope.png|thumb|Heliotrope rash]]
 
[[File:Heliotrope.png|thumb|Heliotrope rash]]
 
===Muscular===
 
===Muscular===
*Symmetric weakness
+
*Symmetric [[weakness]]
 
*Proximal greater than distal weakness
 
*Proximal greater than distal weakness
 
**Deltoid and hip flexors
 
**Deltoid and hip flexors
Line 14: Line 14:
 
*Onset over months
 
*Onset over months
 
*Mild myalgias
 
*Mild myalgias
 +
*[[Dysphagia]] due to esophageal muscle dysfunction
 +
*[[GERD]] due to smooth muscle inflammation, [[gastric ulcer disease]]
 +
*Thoracic muscle weakness
  
 
===Dermatologic===
 
===Dermatologic===
*Heliotrope rash on face scalp
+
*Heliotrope [[rash]] on face scalp
 +
*Periorbital edema
 
*Gottron's papules (rash on MCP, PIP, and DIP joints)
 
*Gottron's papules (rash on MCP, PIP, and DIP joints)
 
*Shawl sign (rash above shoulders)
 
*Shawl sign (rash above shoulders)
 +
*[[Pruritus]]
 +
*Calcinosis
 +
**Nodules over joints can lead to debilitating contractures, especially in children
 +
 +
===Respiratory===
 +
*Respiratory manifestations associated with presence of anti-Jo1 antibody<ref>Vymetal J, Skacelova M, Smrzova A, Klicova A, Schubertova M, Horak P, Zadrazil J. Emergency situations in rheumatology with a focus on systemic autoimmune diseases. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub. 2016 Mar;160(1):20-9. doi: 10.5507/bp.2016.002. Epub 2016 Feb 10. Review. PubMed PMID: 26868300.</ref>
 +
**Increased risk of aspiration, which is a leading cause of death in patients with restricted mobility<ref>Fujisawa T, Hozumi H, Kono M, Enomoto N, Hashimoto D, Nakamura Y, Inui N, Yokomura K, Koshimizu N, Toyoshima M, Shirai T, Yasuda K, Hayakawa H, Suda T. Prognostic factors for myositis-associated interstitial lung disease. PLoS One [serial on the internet] 2014 Jun [cited 2014 Oct 20]; Available from: http://www.ncbi.nlm.nih.gov/ pmc/articles/PMC4048238/?report=classic</ref>
 +
*Poor prognosis
 +
*interstitial lung disease
 +
*Thoracic muscle weakness
 +
 +
===Cardiac===
 +
*Dilated [[cardiomyopathy
 +
*Cardiac myositis
 +
**Suspect in known dermatomyositis with mild rhythm disturbances, [[CHF]] or myocardial fibrosis
 +
**Causes include reentry pathways, fibrosis, altered automaticity, conduction system injury or treatment side effects
  
 
===Other===
 
===Other===
*Cardiac (cardiomyopathy)
+
*[[Fever]], malaise, [[fatigue]], weight loss
 
*High association with malignancy
 
*High association with malignancy
*Interstitial lung disease
 
*Esophageal inflammation
 
  
 
==Differential Diagnosis==
 
==Differential Diagnosis==
Line 32: Line 50:
  
 
==Evaluation==
 
==Evaluation==
*CK
+
*Evaluate for emergent complications (e.g. [[respiratory distress]], [[cardiomyopathy]], [[arrhythmia]])
*ANA
+
*BMP
*Anti-Jo-1
+
*[[ECG]], Troponin to screen for  myocardial involvement
*Chem for creatinine
+
*CK, [[Urinalysis]] for myoglobin
*Troponin, can involve myocardium
+
*Signs of systemic inflammation<ref>Koler RA and Montemarano A. Dermatomyositis. Am Fam Physician. 2001 Nov 1;64(9):1565-1573.</ref>
*[[ECG]]
+
**ESR elevated in 50% of patients, does not correlate with disease activity
 +
**Elevated CRP
 +
 
 +
===Non-ED Evaluation===
 +
*ANA, Anti-Jo-1
 
*Bedside PFTs (FEV1 and FVC)
 
*Bedside PFTs (FEV1 and FVC)
*[[Urinalysis]] for myoglobin
 
 
*EMG or muscle biopsy showing myositis
 
*EMG or muscle biopsy showing myositis
*Consider malignancies
+
*Malignancy screening/evaluation
 +
**Increased risk of malignancy (~30%), especially:<ref>Hill CL et al. Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study. Lancet. 2001 Jan 13;357(9250):96-100.</ref> ovarian, lung, pancreatic, stomach, colorectal, bladder, and non-hodgkin lymphoma
 
**Associated with increased age, male gender and elevated creatinine kinase levels
 
**Associated with increased age, male gender and elevated creatinine kinase levels
*Signs of systemic inflammation<ref>Koler RA and Montemarano A. Dermatomyositis. Am Fam Physician. 2001 Nov 1;64(9):1565-1573.</ref>
 
**Fever
 
**ESR elevated in 50% of patients, does not correlate with disease activity
 
**Elevated CRP
 
  
 
==Management==
 
==Management==
Line 55: Line 73:
 
===Severe disease===
 
===Severe disease===
 
*Interstitial Fibrosis or weakness of respiratory muscles
 
*Interstitial Fibrosis or weakness of respiratory muscles
**Presents with breathlessness, respiratory insufficiency
 
**Associated with presence of anti-Jo1 antibody<ref>Vymetal J, Skacelova M, Smrzova A, Klicova A, Schubertova M, Horak P, Zadrazil J. Emergency situations in rheumatology with a focus on systemic autoimmune diseases. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub. 2016 Mar;160(1):20-9. doi: 10.5507/bp.2016.002. Epub 2016 Feb 10. Review. PubMed PMID: 26868300.</ref>
 
**Increased risk of aspiration, which is a leading cause of death in patients with restricted mobility<ref>Fujisawa T, Hozumi H, Kono M, Enomoto N, Hashimoto D, Nakamura Y, Inui N, Yokomura K, Koshimizu N, Toyoshima M, Shirai T, Yasuda K, Hayakawa H, Suda T. Prognostic factors for myositis-associated interstitial lung disease. PLoS One [serial on the internet] 2014 Jun [cited 2014 Oct 20]; Available from: http://www.ncbi.nlm.nih.gov/ pmc/articles/PMC4048238/?report=classic</ref>
 
**Poor prognosis
 
 
**Methylprednisolone 1000mg/day x 3 days
 
**Methylprednisolone 1000mg/day x 3 days
*Cardiac Myositis
+
 
**Presents with known Dermatomyositis and mild rhythm disturbances, CHF or myocardial fibrosis
+
===Outpatient===
**Causes include reentry pathways, fibrosis, altered automaticity, conduction system injury or treatment side effects
+
*Sunscreen!
 +
*[[Hydroxychloroquine]]
 +
*Other immunosuppressive meds (e.g. methotrexate, rituximab, azathioprine]]
 +
*[[Calcium channel blockers]] for calcinosis
  
 
==Disposition==
 
==Disposition==
*Admit to monitored bed for [[CHF]] symptoms, respiratory muscle weakness
+
*Admit for decompensated respiratory, cardiac, or other major disease
*Admit for dysphagia/aspiration risk
 
*Out patient follow up with muscle biopsy for others
 
*Very strongly associated with malignancy (~30%), especially:<ref>Hill CL et al. Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study. Lancet. 2001 Jan 13;357(9250):96-100.</ref>
 
**Ovarian
 
**Lung
 
**Pancreatic
 
**Stomach
 
**Colorectal
 
**Non-Hodgkin Lymphoma
 
**Bladder
 
  
 
==See Also==
 
==See Also==
 
*[[Weakness]]
 
*[[Weakness]]
 +
*[[Collagen vascular disease]]
  
 
==References==
 
==References==
 
<references/>
 
<references/>
 
[[Category:Rheumatology]][[Category: Dermatology]]
 
[[Category:Rheumatology]][[Category: Dermatology]]

Revision as of 13:16, 22 August 2017

Background

  • Inflammatory myopathy with skin manifestations
  • Affects proximal muscles > distal
  • Female:Male; 2:1
  • Peak incidence 40-50s

Clinical Features

Heliotrope rash

Muscular

  • Symmetric weakness
  • Proximal greater than distal weakness
    • Deltoid and hip flexors
    • difficulty standing from a chair
  • Onset over months
  • Mild myalgias
  • Dysphagia due to esophageal muscle dysfunction
  • GERD due to smooth muscle inflammation, gastric ulcer disease
  • Thoracic muscle weakness

Dermatologic

  • Heliotrope rash on face scalp
  • Periorbital edema
  • Gottron's papules (rash on MCP, PIP, and DIP joints)
  • Shawl sign (rash above shoulders)
  • Pruritus
  • Calcinosis
    • Nodules over joints can lead to debilitating contractures, especially in children

Respiratory

  • Respiratory manifestations associated with presence of anti-Jo1 antibody[1]
    • Increased risk of aspiration, which is a leading cause of death in patients with restricted mobility[2]
  • Poor prognosis
  • interstitial lung disease
  • Thoracic muscle weakness

Cardiac

  • Dilated [[cardiomyopathy
  • Cardiac myositis
    • Suspect in known dermatomyositis with mild rhythm disturbances, CHF or myocardial fibrosis
    • Causes include reentry pathways, fibrosis, altered automaticity, conduction system injury or treatment side effects

Other

  • Fever, malaise, fatigue, weight loss
  • High association with malignancy

Differential Diagnosis

  • Drug Induced Myopathies (Statins)

Weakness

Evaluation

Non-ED Evaluation

  • ANA, Anti-Jo-1
  • Bedside PFTs (FEV1 and FVC)
  • EMG or muscle biopsy showing myositis
  • Malignancy screening/evaluation
    • Increased risk of malignancy (~30%), especially:[4] ovarian, lung, pancreatic, stomach, colorectal, bladder, and non-hodgkin lymphoma
    • Associated with increased age, male gender and elevated creatinine kinase levels

Management

Mild disease

  • Prednisone 1mg/kg/day (up to 80mg/day)
    • Taper after 6-8 weeks

Severe disease

  • Interstitial Fibrosis or weakness of respiratory muscles
    • Methylprednisolone 1000mg/day x 3 days

Outpatient

Disposition

  • Admit for decompensated respiratory, cardiac, or other major disease

See Also

References

  1. Vymetal J, Skacelova M, Smrzova A, Klicova A, Schubertova M, Horak P, Zadrazil J. Emergency situations in rheumatology with a focus on systemic autoimmune diseases. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub. 2016 Mar;160(1):20-9. doi: 10.5507/bp.2016.002. Epub 2016 Feb 10. Review. PubMed PMID: 26868300.
  2. Fujisawa T, Hozumi H, Kono M, Enomoto N, Hashimoto D, Nakamura Y, Inui N, Yokomura K, Koshimizu N, Toyoshima M, Shirai T, Yasuda K, Hayakawa H, Suda T. Prognostic factors for myositis-associated interstitial lung disease. PLoS One [serial on the internet] 2014 Jun [cited 2014 Oct 20]; Available from: http://www.ncbi.nlm.nih.gov/ pmc/articles/PMC4048238/?report=classic
  3. Koler RA and Montemarano A. Dermatomyositis. Am Fam Physician. 2001 Nov 1;64(9):1565-1573.
  4. Hill CL et al. Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study. Lancet. 2001 Jan 13;357(9250):96-100.