Peter Špalek, Jozef Lukáč, Iveta Mečiarová
Dermatomyositis (DM) is an autoimmune disease. Complement activation occurs early in the disease, leading to
the formation and deposition of membranolytic attack complex on the endomysial capillaries, followed by capillary
destruction, perivascular inflammation, and perivascular atrophy. Onset of proximal and symmetrical muscle weakness
and myalgias is typically subacute (over weeks), although it can develop abruptly (over few days). Involvement
of oropharyngeal, laryngeal or masticatory muscles occurs in cca 20 % DM patients. Characteristic skin rash usually
accompanies or precedes the onset of muscle weakness. A heliotrope rash is the classic purplish discoloration of the
eyelids, often associated with periorbital edema. A macular erythematosus rash can affect the face, neck, anterior chest
and upper back. Subcutaneous calcinosis and skin ulcerations often occur in juvenile DM but are uncommon in adult DM.
Multisystem involvement may occur in DM – interstitial lung disease, cardiac, joint, gastrointestinal manifestations
and necrotizing vasculitis. In up to 10 % of adult DM patients, there may be an underlying malignancy, necessitating
a thorough workup and careful monitoring, especially during the first 4 years from disease onset. Bohan´s and Peter´s
clinical, biochemical (hyperCKemia), EMG and histopathological criteria are practical and sensitive for diagnosis of DM.
Corticosteroids are the first-line treatment. We start the treatment with intravenous methylprednisolon (1 g/day for
3-5 days) and then switch to the oral regimen with prednisone > 1 mg/kg, as a single daily dose, taken in the morning.
In patients with severe disease we start with a combined immunosuppressive treatment (prednisone and azathioprine).
From the onset is IVIg the best choice in very rapidly progressive cases with severe weakness, myalgias and evolving
secondary rhabdomyolysis. The standard dose is 0,4 g/kg/day given for 5 days. IVIg has beneficial responses also in cases
of DM refractory to corticosteroids and immunosuppressive agents. We report a 52-year-old male patient who developed
severe acute dermatomyositis with respiratory and bulbar weakness, secondary rhabdomyolysis, myoglobinuria, renal
failure and multiple autoimmune disorders (arthritis, skin ulcerations, severe gastrointestinal inflammation, anemia,
subcutaneous calcinosis). Combined immunotherapy with i.v. methylprednisolone, prednisone, immunosuppressive
agents (azathioprine, methotrexate), i.v. cyclophosphamide and repeated courses of IVIg have been needed to achieve
an improvement in muscle strength, in activities of daily living, as well as the improvements in systemic manifestations.
Long-lasting maintenance treatment with cyclosporine (150 mg daily), prednisone (20 mg daily) and IVIg (30 g in 1
month´s intervals) is necessary because of repeated disease exacerbations.