The association of
systemic lupus erythematosus (SLE) with
idiopathic polymyositis or
dermatomyositis is reported to occur in the range of 4-16%.
Myositis can occur before or after SLE, or sporadically both diseases can be present simultaneously. This case report concerns a 36-year-old female patient suffering from Raynaud's phenomenon,
polyarthralgia in the small joints of the hands, and skin changes compatible with Gotron's indications. Symmetric proximal
muscle weakness of the extremities,
fever of up to 40 degrees C, heliotrope rashes with erythematous changes in the face, upper arms, and posterior shoulders occurred subsequently. Laboratory analyses revealed increased
acute phase reactants, hypochromic anaemia,
lymphopenia, and increased levels of all muscle
enzymes. Immunoserology demonstrated positive ANA, anti-Sm, and
anticardiolipin antibodies (aCL), while anti dsDNA, anti Ro, anti La, and anti Jo-1
antibodies proved negative. Hypocomplementaemia and elevated levels of
immune complexes were also detected. Pathologic sediment and
proteinuria were revealed via urine analyses, while a kidney biopsy confirmed
lupus nephritis (type IVa according to the World Health Organisation classification). Biopsy of erythematous changes of the posterior shoulder demonstrated
leukocytoclastic vasculitis. Electromyography of the lower extremities established myopathic changes.
Inflammation of the muscles was confirmed via magnetic resonance imaging. The patient was categorised as having two separate coexistent diseases--SLE and
dermatomyositis. Both the classification criteria of the American College of Rheumatology for SLE and the diagnostic criteria for
dermatomyositis, proposed by Bohon and Peter, were fulfilled simultaneously. Treatment commenced with pulses of
methylprednisolone and continued with oral
therapy, including
Resochin. Pulses of intravenous
cyclophosphamide were also administered. After six weeks of
therapy, biohumoral remission of both diseases was achieved, while complete recovery from
muscle weakness was accomplished after four months.