Sharpe’s syndrome: from basic anti-inflammatory therapy to genetically engineered biological drugs

Abstract

This article presents a clinical case of a patient with a mixed connective tissue disease. The clinical picture in the debut was polymorphic, the patient had arthritis, skin lesions, general inflammation, myopathy, Raynaud’s syndrome, dysphagia, angio-neurotic edema. The immunological panel reflected the presence of antibodies characteristic of autoimmune diseases (antinuclear antibodies, antibodies to ribonucleoprotein, antimitochondrial antibodies, antibodies to cytoplasmic antigen SS-B). Glucocorticosteroids (GCS), immunosuppressive drugs (metatrexate, sulfasalazine, hydroxychloroquine, azathioprine) were used as starting therapy, but the disease progressed. Pulse therapy of GCS and plasmapheresis did not give a positive result. We used Rituximab, because the pathogenesis of the disease was characterized by the activation of B-cells. Currently, two infusions have been performed, positive dynamics has been noted. Thus, the therapy of mixed connective tissue disease creates many diagnostic and therapeutic problems in clinical practice.