Difference between revisions of "Dermatomyositis"

(Disposition)
(Clinical Features)
 
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==Background==
 
==Background==
*Inflammatory myopathy
+
*Inflammatory myopathy with skin manifestations
 
*Affects proximal muscles > distal
 
*Affects proximal muscles > distal
 
*Female:Male; 2:1
 
*Female:Male; 2:1
 
*Peak incidence 40-50s
 
*Peak incidence 40-50s
 +
*Juvenille dermatomyositis: 5-10 years of age
  
 
==Clinical Features==
 
==Clinical Features==
'''Muscular'''
+
[[File:Heliotrope.png|thumb|Heliotrope rash]]
#Proximal greater than distal weakness
+
[[File:Dermatomyositis.png|thumb|Various dermatologic manifestations of dermatomyositis. Gottron's papules are seen in image A. Shawl sign is seen in image D.]]
*Deltoid and hip flexors
+
===Muscular===
*difficulty standing from a chair
+
*Symmetric [[weakness]]
#Onset over months
+
*Proximal greater than distal weakness
#Mild myalgias
+
**Deltoid and hip flexors
 +
**difficulty standing from a chair
 +
*Onset over months
 +
*Mild [[myalgia]]s
 +
*[[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
#Gottron's papules (rash on MCP, PIP, and DIP joints)
+
*Periorbital edema
#Shawl sign (rash above shoulders)
+
*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
  
'''Other'''
+
===Respiratory===
#Cardiac (cardiomyopathy)
+
*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>
#High association with malignancy
+
**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>
#Interstial lung disease
+
*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==
 
==Differential Diagnosis==
*[[Polymyositis]]
+
{{Myalgia DDX}}
*[[SLE]]
+
 
*[[Guillain-Barre Syndrome]]
+
{{Weakness DDX}}
*Drug Induced Myopathies (Statins)
+
 
*[[Hypothyroidism]]
+
==Evaluation==
*Limb Girdle Muscular Dystrophy
+
*Evaluate for emergent complications (e.g. [[respiratory distress]], [[cardiomyopathy]], [[arrhythmia]])
*[[Rhabdomyolysis]]
+
*BMP
*[[Myasthenia Gravis]]
+
*[[ECG]], [[troponin]] to screen for  myocardial involvement
*[[Pyomyositis]]
+
*CK, [[Urinalysis]] for myoglobin
*Viral myositis
+
*Signs of systemic inflammation<ref>Koler RA and Montemarano A. Dermatomyositis. Am Fam Physician. 2001 Nov 1;64(9):1565-1573.</ref>
*[[Botulism]]
+
**ESR elevated in 50% of patients, does not correlate with disease activity
 +
**Elevated CRP
  
==Diagnosis==
+
===Non-ED Evaluation===
*CK
+
*ANA, Anti-Jo-1
*ANA
 
*Chem for creatinine
 
*Troponin, can involve myocardium
 
*EKG
 
 
*Bedside PFTs (FEV1 and FVC)
 
*Bedside PFTs (FEV1 and FVC)
*UA for myoglobin
+
*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
  
 
==Management==
 
==Management==
 
===Mild disease===
 
===Mild disease===
*Prednisone 1mg/kg/day (up to 80mg/day)
+
*[[Prednisone]] 1mg/kg/day (up to 80mg/day)
 
**Taper after 6-8 weeks
 
**Taper after 6-8 weeks
  
 
===Severe disease===
 
===Severe disease===
*Respiratory muscle symptoms of CHF
+
*Interstitial fibrosis or weakness of respiratory muscles
**Methylprednisolone 1000mg/day x 3 days
+
**[[Methylprednisolone]] 1000mg/day x 3 days
 +
*[[Methotrexate]] or [[azathioprine]] for cases refractory to steroids
 +
 
 +
===Outpatient===
 +
*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
 
  
 
==See Also==
 
==See Also==
 
*[[Weakness]]
 
*[[Weakness]]
*Polymyositis
+
*[[Collagen vascular disease]]
*SLE
 
*Rheumatoid arthritis
 
  
 
==References==
 
==References==
 
+
<references/>
[[Category: Rheum]][[Category: Derm]]
+
[[Category:Rheumatology]][[Category: Dermatology]]

Latest revision as of 18:30, 13 February 2021

Background

  • Inflammatory myopathy with skin manifestations
  • Affects proximal muscles > distal
  • Female:Male; 2:1
  • Peak incidence 40-50s
  • Juvenille dermatomyositis: 5-10 years of age

Clinical Features

Heliotrope rash
Various dermatologic manifestations of dermatomyositis. Gottron's papules are seen in image A. Shawl sign is seen in image D.

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

Myalgia

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

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.