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Título

Inclusion Body Myositis associated with Sjogren’s Syndrome: A new myopathy description?

RESUMO

Case Report: A 47-year-old man presented with polyarthralgia associated with knee and ankle joints swelling 4 years prior to evaluation. A year later, he noticed progressive weakness of upper and lower limbs, with difficulty raising the arms and climbing stairs. He also referred trouble in writing, reaching out for things and picking up objects. Neurological examination revealed myopathic gait, atrophy of the deltoids and pectorals, facial weakness, proximal and distal muscle weakness in the upper and lower limbs. Deep tendon reflexes were decreased in the upper limbs and normal in the lower limbs. Cranial nerve, sensitivity, cerebellum and cognition tests were normal. The creatine kinase (CK) was 3,077 U/L and serum aldolase level was 32 (normal, < 7,6 U/L). Erythrocyte sedimentation rate was elevated. Anti-cN1A and Anti-SSA/Ro antibodies were positive. Complement levels and serum levels of thyroid hormones were within normal ranges. Negative autoantibodies included: antinuclear antibodies, anti-SSB/La, anti-Sm, anti-DNAh, anti-SRP, anti-RNP, anti-SCL-70, anti-RNA polymerase III. Lower limb MRI showed muscle edema with fatty partial replacement and atrophy. Electromyography demonstrated a pattern of proximal and distal weakness in both upper and down extremities associated with spontaneous activity. Repetitive stimulation test was normal. Muscle biopsy was consistent with Inclusion Body Myositis (IBM). An association between IBM and Sjogren's Syndrome (SS) was considered and the patient was started on oral prednisone (1mg/kg daily). After two weeks with corticosteroid, he showed partial clinical improvement thus we indicated methylprednisolone pulse therapy (1g IV/daily for 5 days). At 1-month follow-up, the patient showed improvement in muscle strength, specially of proximal upper extremities. Discussion: Inclusion Body Myositis and its histopathological features were first described in 1967. An association between IBM and SS was proposed fifteen years later, in 1982. The pathogenesis of this association is unclear. Recent studies suggest for disease concurrence a shared association with T-cell granular lymphocyte leukemia. Even though sporadic IBM is considered treatment-refractory, studies have reported response to immunotherapy when this combination is present, likewise our case. Final comments: Faced with the diagnosis of IBM we suggest that SS should be investigated, as patients with this association may benefit from immunosuppressive therapy.