Dermatomyositis

Last Updated: 2021-10-18

Author(s): Anzengruber, Navarini

  • Wagner 1863
  • Unverricht 1887

Polymyositis

Rare antibody- or immune complex-mediated autoimmune disease, which belongs to the group of inflammatory myopathies.

  • Incidence (children): 2-7/1'000'000/year
  • Incidence (adults): 6-10/1'000'000/year
  • Peak of incidence (adults): ca. 40 LY and v. 55-60 yrs
  • Peak in incidence (children): 5-14 yrs
  • African-Americans > Caucasians
  • Women : men = up to 2 : 1
  • Clinical are distinguished (with frequencies):
    • Type I: adult polymyositis
    • Type II: typical adult dermatomyositis with skin/muscle involvement
    • Type III: typical adult dermatomyositis with malignant tumours
    • Type IV: Typical dermatomyositis of the adult of childhood (especially after infection of the respiratory tract)
    • Type V: Polymyositis with myoglobulinuria (acute myolysis)
    • Type VI: Polymyositis with Sjörgren's syndrome
    • Type VII: Polymyositis with other collagenoses
  • UV light may lead to exacerbation of dermatomyositis
  • Genetic predispositions:
    • Idiopathic myositis: HLA-B8, HLA DRB 03
    • Classic and juvenile dermatomyositis: HLA-A68, HLA-DR3
    • Drug-associated dermatomyositis: HLA-B 08, HLA-DR4 haplotypes
    • Amyopahtic dermatomyositis: no HLA associations
  • Infections:
    • Picornaviruses
    • Coxsackieviruses
    • AIDS
    • HTLV-1
  • Medications:
    • D-penicillamine
    • Procainamide
    • NSAR
    • Simvastatin
    • Antimalarials
    • Interferon α
    • Allopurinol
  • Neoplasia
  • General symptoms
  • Fatigue
  • Myalgias (muscle-cartilage-like)
  • Problems combing hair and other daily activities

 

  • Cutaneous symptoms:
    • Facial swelling with eyelid oedema
    • Pruriginous or mildly dolente, lilac erythema on the face and décolleté
    • "Weepy facial expression"
    • Heliotropic erythema of the eyelids
    • Cheeks: butterfly-shaped erythema
    • Shawl sign: perioral recess erythema
    • Atrophic, hypo- or hyperpigmentation
    • Gottron's sign
      • In 70% of patients
      • pathognomic
      • Striated erythema and papules on the finger extensor sides
    • Nailfold hyperkeratoses with telangiectasia and telangiectasia
      • Keining's sign = pain when trying to push back the nail fold
    • Rhagades of the fingertips
    • Scleroderma-like indurations
    • Raynaud's syndrome
    • Uncharacteristic psoriasiform, lichen planus-like exanthema
    • Diffuse effluvium
    • Poikiloderma
  • Musculature:
    • Muscle weakness, especially proximal limb sections (neck, shoulder girdle, legs)
    • Symmetric muscle weakness/ myalgias/ muscle atrophies/ contractures
    • Pharyngeal and laryngeal muscles may also be affected
    • Calcifications of the musculature are possible
    • Dermatomyositis sine myositis or amyopathic dermatomyositis are called dermatomyositides without muscle involvement
  • Bones and joints:
    • Osteoporosis
    • Arthralgias
    • Arthritides
    • Symmetric polyarthritis, also known as oligo- or monarthritis
  • Overlap syndromes are possible
  • Other (rare) organ involvement of
    • Heart
    • Intestine
    • Kidneys
  • Lab
    • All laboratory values listed may be normal despite the presence of dermatomyositis.
    • BSG ↑, CRP ↑.
    • Blood count: leukocytosis, lymphopenia, eosinophilia.
    • ↑ Serum creatinine phosphokinase, aldolase, CPK, GOT, GPT, LDH
    • Antinuclear antibody (ANA) positive in approx. 1/3 of cases.
    • Jo-1 in 20%
    • PM-Scl 5%
    • nRNP 5%
    • Anti-Mi 10-30%
    • PL-7 < 3%
    • PL-12 < 3%
    • OJ in < 3%
    • EJ in < 3%
    • Serum electrophoresis: α2- and γ-globulin.
    • Urine: creatinine and creatinineuria. Proteinuria in relapse, myoglobinuria.
  • Clinic
  • Laboratory:
    • blood sedimentation rate, C-reactive protein, liver and kidney values, creatinine kinase, aldolase, LDH, rheumatoid factor
    • Thyroid antibodies
    • ANAs and ENAs
  • U status/creatin excretion in 24-hour urine
  • Biopsy:
    • Skin biopsy
    • Muscle biopsy
  • Electromyogram: polyphasic potentials
  • Capillaroscopy
  • Tumour exclusion
  • To diagnose "polymyositis", at least 3 of the following criteria should be positive:
    • Muscle enzymes (creatinine kinase)
    • Electromyogram: polyphasic potentials
    • Muscle biopsy
  • Unpredictable
  • 5-year survival rate at 95% or the 10-year survival rate at 84%
  • Skeletal exercises
  • UV protection measures
  • Systemic glucocorticoids
    • Prednisolone p.o. 1.0-2.0 mg/kg bw/day if necessary pulse therapy with 1g daily for 3-5 days
  • Azathioprine p.o. 1x daily
    • Progression: reduction every 2-4 weeks by 5-10 mg to the lowest still effective dosage
    • CAVE: With concomitant administration of allopurinol, a reduction of the azathioprine dose to a ¼ dose is indicated
    Initially: 1-3 mg/kg bw
  • Methotrexate (MTX) s.c. 15 mg 1x weekly (CAVE: no i.m injections!)
    • Start with 10 mg 1x weekly
    • Increase by 2.5 mg weekly until 15 mg is reached
    • Folic acid p.o. 5 mg 1-0-0 the following day
  • Cyclophosphamide i.v. 100-150 mg daily
    • Shock therapy 0.5-1.0 g/m2 KO/month i.v.
  • Cyclosporine p.o. 3-5 mg/kg bw daily
    • Evidence: case reports only
    • Take independently of meals
  • Mycophenolate mofetil p.o. 1-1.5 g 2x daily
    • Evidence: case reports only
    • Take-up time approx. 3 months
  • Intravenous immunoglobulins i.v. 0.5-1.0 g/kg bw daily for 3-5 days every 3-4 weeks
    • Initial dose: 0.4-0.8 g/kg bw
    • In the course: 0.2 g/kg bw every 3 to 4 weeks
    • Determination of the IgG serum level always immediately before the next infusion!
    • An IgG valley level of at least 5 to 6 g/l should be reached before a new infusion
  • Chimeric anti-CD20 monoclonal antibody rituximab i.v. 275 mg/m² KOF (days 0, 7, 14, 21)
    • Premedication:
      • Analgesics 1000 mg paracetamol p.o.
      • Antihistamines
        • H1 blockers
        • Levocetirizine p.o. 5 mg 1x daily
        • Desloratadine p.o. 5 mg 1x daily
        • Fexofenadine p.o. 180 mg 1x daily
    • First infusion:
      • Initial infusion rate: 50 mg/h
      • After 1 hour, an increase of 50 mg/h every 30 minutes up to 400 mg/h can be considered
    • Additional infusions:
      • Initial infusion rate: 100 mg/h
      • After 1 hour, increase by 100 mg/h up to 400 mg/h
      • Rituximab must not be administered undiluted and not as a short infusion!
      • If respiratory symptoms or hypotension occur, 24-hour monitoring is indicated!
  • Plasmapheresis
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