Dermatomyositis: Difference between revisions
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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 | ||
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[[File:Heliotrope.png|thumb|Heliotrope rash]] | [[File:Heliotrope.png|thumb|Heliotrope rash]] | ||
===Muscular=== | ===Muscular=== | ||
*Symmetric weakness | |||
*Proximal greater than distal weakness | *Proximal greater than distal weakness | ||
**Deltoid and hip flexors | **Deltoid and hip flexors | ||
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*Cardiac (cardiomyopathy) | *Cardiac (cardiomyopathy) | ||
*High association with malignancy | *High association with malignancy | ||
* | *Interstitial lung disease | ||
*Esophageal inflammation | |||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
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*Bedside PFTs (FEV1 and FVC) | *Bedside PFTs (FEV1 and FVC) | ||
*[[Urinalysis]] for myoglobin | *[[Urinalysis]] for myoglobin | ||
*EMG or muscle biopsy showing myositis | |||
*Consider malignancies | *Consider malignancies | ||
**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> | *Signs of systemic inflammation<ref>Koler RA and Montemarano A. Dermatomyositis. Am Fam Physician. 2001 Nov 1;64(9):1565-1573.</ref> | ||
**Fever | **Fever | ||
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===Severe disease=== | ===Severe disease=== | ||
* | *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 | |||
**Causes include reentry pathways, fibrosis, altered automaticity, conduction system injury or treatment side effects | |||
==Disposition== | ==Disposition== | ||
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**Stomach | **Stomach | ||
**Colorectal | **Colorectal | ||
**Lymphoma | **Non-Hodgkin Lymphoma | ||
**Bladder | **Bladder | ||
Revision as of 17:32, 21 August 2017
Background
- Inflammatory myopathy with skin manifestations
- Affects proximal muscles > distal
- Female:Male; 2:1
- Peak incidence 40-50s
Clinical Features
Muscular
- Symmetric weakness
- Proximal greater than distal weakness
- Deltoid and hip flexors
- difficulty standing from a chair
- Onset over months
- Mild myalgias
Dermatologic
- Heliotrope rash on face scalp
- Gottron's papules (rash on MCP, PIP, and DIP joints)
- Shawl sign (rash above shoulders)
Other
- Cardiac (cardiomyopathy)
- High association with malignancy
- Interstitial lung disease
- Esophageal inflammation
Differential Diagnosis
- Drug Induced Myopathies (Statins)
Weakness
- Neuromuscular weakness
- Upper motor neuron:
- CVA
- Hemorrhagic stroke
- Multiple sclerosis
- Amyotrophic Lateral Sclerosis (ALS) (upper and lower motor neuron)
- Lower motor neuron:
- Spinal and bulbar muscular atrophy (Kennedy's syndrome)
- Spinal cord disease:
- Infection (Epidural abscess)
- Infarction/ischemia
- Trauma (Spinal Cord Syndromes)
- Inflammation (Transverse Myelitis)
- Degenerative (Spinal muscular atrophy)
- Tumor
- Peripheral nerve disease:
- Neuromuscular junction disease:
- Muscle disease:
- Rhabdomyolysis
- Dermatomyositis
- Polymyositis
- Alcoholic myopathy
- Upper motor neuron:
- Non-neuromuscular weakness
- Can't miss diagnoses:
- ACS
- Arrhythmia/Syncope
- Severe infection/Sepsis
- Hypoglycemia
- Periodic paralysis (electrolyte disturbance, K, Mg, Ca)
- Respiratory failure
- Emergent Diagnoses:
- Symptomatic Anemia
- Severe dehydration
- Hypothyroidism
- Polypharmacy
- Malignancy
- Aortic disease - occlusion, stenosis, dissection
- Other causes of weakness and paralysis
- Acute intermittent porphyria (ascending weakness)
- Can't miss diagnoses:
Evaluation
- CK
- ANA
- Anti-Jo-1
- Chem for creatinine
- Troponin, can involve myocardium
- ECG
- Bedside PFTs (FEV1 and FVC)
- Urinalysis for myoglobin
- EMG or muscle biopsy showing myositis
- Consider malignancies
- Associated with increased age, male gender and elevated creatinine kinase levels
- Signs of systemic inflammation[1]
- Fever
- ESR elevated in 50% of patients, does not correlate with disease activity
- Elevated CRP
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
- Cardiac Myositis
- Presents with known Dermatomyositis and mild rhythm disturbances, CHF or myocardial fibrosis
- Causes include reentry pathways, fibrosis, altered automaticity, conduction system injury or treatment side effects
Disposition
- Admit to monitored bed for CHF symptoms, respiratory muscle weakness
- Admit for dysphagia/aspiration risk
- Out patient follow up with muscle biopsy for others
- Very strongly associated with malignancy (~30%), especially:[4]
- Ovarian
- Lung
- Pancreatic
- Stomach
- Colorectal
- Non-Hodgkin Lymphoma
- Bladder
See Also
References
- ↑ Koler RA and Montemarano A. Dermatomyositis. Am Fam Physician. 2001 Nov 1;64(9):1565-1573.
- ↑ 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.
- ↑ 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
- ↑ 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.