Updated: 11/23/2018

Polymyositis / Dermatomyositis

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Snapshot
  • A 53-year-old woman with a past medical history of thyroid disease presents to her physician’s office for weakness. She reports that she has been feeling weak, has difficulty climbing the stairs, and combing her hair every morning. Today, she also has difficulty arising out of her chair. Physical exam reveals decreased strength, especially in the shoulders. She also has an impressive lilac periorbital rash and a sunburn on her cheeks. She is sent for further laboratory workup and counseled on the likely initiation of high-dose steroids.
Introduction
  • Clinical definition
    • dermatomyositis is an autoimmune myopathy characterized by
      • symmetric proximal muscle weakness and rash
        • the clinical variants include amyopathic dermatomyositis (dermatomyositis without myositis)
    • polymyositis an autoimmune myopathy characterized by
      • symmetric proximal muscle weakness and no rash
  • Epidemiology
    • incidence
      • rare
    • demographics
      • female > male
      • typically in adults 30-50 years of age
      • if in children, considered juvenile dermatomyositis
    • risk factors
      • drugs
      • physical exertion
  • Pathogenesis
    • dermatomyositis
      • an antibody-driven autoimmune disease characterized by perimysial inflammation and atrophy, resulting in muscle tissue damage
        • perimysium is closer to the skin and therefore has cutaneous manifestations – “dermato”
      • associated with CD4 and complement activation
    • polymyositis
      • T-cell driven autoimmune disease characterized by endomysial inflammation
        • endomysium is farther from the skin and therefore has no cutaneous manifestations
      • associated with CD8
  • Associated conditions
    • interstitial lung disease
    • malignancy which includes
      • breast or ovarian cancer
      • prostate cancer
      • lung cancer
    • other connective tissue diseases
  • Prognosis
    • dermatomyositis
      • ↑ risk of malignancy (typically develop within 5 years of diagnosis)
Presentation
  • Symptoms
    • progressive weakness in hips and shoulders
    • dysphagia
    • dysphonia
      • laryngeal muscle weakness
  • Physical exam
    • dermatomyositis
      • characteristic cutaneous findings
        • a photosensitive pink rash of the neck and trunk (shawl or V-sign, as it often appears as sunburn with V-neck t-shirt)
        • purple/lilac or red rash around eyes and on eyelids (heliotrope rash, can resemble malar rash)
        • raised violaceous, slightly scaly plaques, on bony prominences of the hands and elbows (Gottron papules)   
        • Mechanic hands: thickening of the skin on the palms and radial aspects of the digits
    • polymyositis and dermatomyositis
      • proximal muscle weakness in the shoulders and hips
        • difficulty combing hair
        • difficulty raising arms
        • difficulty rising from a chair
Studies
  • Labs
    • ↑ creatinine phosphokinase (CPK or CK)
    • ↑ creatine kinase-MB (CK-MB)
    • ↑ aldolase
    • ↑ lactate dehydrogenase (LDH)
    • autoantibodies
      • + antinuclear antibody (ANA)
      • + anti-histidyl tRNA synthetase antibodies (anti-Jo-1) (~20% of patients) 
      • + anti-signal recognition particle (SRP) (~5% of patients)
        • usually associated with polymyositis
      • + anti-Mi-2 (<10% of adults)
        • usually associated with dermatomyositis
  • Muscle biopsy
    • indication
      • diagnostic gold standard to differentiate between inflammatory and non-inflammatory myopathies
    • findings
      • degenerating muscle fibers and inflammatory infiltrates
      • dermatomyositis
        • perimysial inflammation
      • polymyositis
        • endomysial inflammation
  • Making the diagnosis
    • based on clinical presentation and laboratory studies
Differential
  • Systemic lupus erythematosus
  • Localized scleroderma
  • Infectious myopathy
  • Myasthenia gravis
Treatment
  • Management approach
    • both diseases are often treated initially with a course of steroids followed by long-term immunosuppression
  • Conservative
    • sun protection
      • indication
        • for all patients with dermatomyositis
  • Medical
    • systemic steroids (high-dose)
      • indications
        • for all patients with myositis as first-line therapy
        • 2-4 weeks with subsequent taper
    • antimalarial
      • indications
        • for patients with primarily skin manifestations of dermatomyositis
        • drugs
          • hydroxychloroquine
    • non-steroidal immunosuppression
      • indications
        • for refractory disease
        • for prevention of recurrence after steroid taper
        • drugs
          • methotrexate
          • azathioprine
Complications
  • Cardiomyopathy
  • Macrophage activation syndrome
 

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Questions (4)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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(M1.MK.65) A 42-year-old man presents to clinic complaining of increasing difficulty climbing stairs and standing up from sitting in his chair. On exam you perceive that his strength to be 5/5 distally, but only 3/5 in proximal muscle groups bilaterally. There is a distinctive rash on his upper eyelids and around his eyes. Examination of the fingers is most likely to reveal which of the following? Review Topic

QID: 102002
1

Dactylitis

9%

(3/34)

2

Enlargement of the PIP

9%

(3/34)

3

Ulnar deviation of the fingers

9%

(3/34)

4

Nail pitting with oil spots

15%

(5/34)

5

Violaceous papules over the MCP, PIP, and DIP

56%

(19/34)

M1

Select Answer to see Preferred Response

PREFERRED RESPONSE 5

(M1.MK.19) A 40-year-old male presents to your office complaining that he is too weak to climb stairs or brush his hair. He denies any headaches or change in vision. A muscle biopsy reveals CD8+ lymphocyte infiltration in the endomysium. Which of the following is the most likely diagnosis? Review Topic

QID: 100440
1

Systemic lupus erythematosus

1%

(1/143)

2

Scleroderma

1%

(2/143)

3

Polymyalgia rheumatica

10%

(14/143)

4

Polymyositis

80%

(114/143)

5

Dermatomyositis

7%

(10/143)

M1

Select Answer to see Preferred Response

PREFERRED RESPONSE 4
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