MDA-5 antibody syndrome 4.67/5 (3)

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Anti MDA5 Myositis Syndrome

MDA5 related dermatomyositis. Parronchi P, Clin Mol Allergy (2015). CC BY 4.0

Definition

A form of Dermatomyositis, but the muscle inflammation is little pronounced ("amyopathic dermatomyositis"). On the other hand, pulmonary symptoms are often severe. Everybody has antibody MDA5 although ANA is usually negative / non-existent.

Symptoms

Blood tests

Diagnosis

  • Typical lung abnormalities (Ground glass opacities)
  • MDA5 antibody
  • Absence of other explanations

Incorrect diagnosis (similar conditions, differential diagnoses)

Treatment

  • Corticosteroids in high doses
  • Mycophenolate (CellCept) 2-3 g / day
  • Tacrolimus 2-3,5 mg / day and serum levels 4-7μg / L. Tacrolimus is combined with mycophenolate (2 g / day) as needed
  • Sendoxan intravenous (pulse) is an alternative

Prognosis

  • Survival is individual and dependent on rapid treatment
  • Survival 60-70%
  • Deaths most often within 6-12 months from diagnosis

References


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