ABSTRACT
AIM: To assess the risk of severe adverse events (AEs) within 6 months after treatment with biological agents in patients with rheumatic diseases (RD). SUBJECTS AND METHODS: The 6-month open-label trial included 107 patients with rheumatoid arthritis, antineutrophil cytoplasmic antibody-associated vasculitides, systemic lupus erythematosus, and other RDs who received genetically engineered biological agents (GEBAs), primarily rituximab (n = 66) and infliximab (n = 31). RESULTS: The majority of patients were noted to have improvements, including complete and partial remission in 62 (57.9%) and 42 (39.3%), respectively. There were mild or moderate AEs in 22 (20.6%) of the 107 patients, severe AEs in 6 (5.6%): grade IV neutropenia in 2 patients (after the use of rituximab), severe infusion reactions in 2 (after the administration of infliximab and rituximab), and systemic infections in 2 (fatal nocardial sepsis after rituximab treatment and unspecified sepsis after infliximab treatment). CONCLUSION: The rate of serious AEs, mainly infusion AEs and infections during treatment with infliximab, rituximab, and other GEBAs proved to be relatively low in patients with different RDs. At the same time, the use of biological agents could lower RD activity in the presence of severe visceral injuries refractory to conventional immunosuppressive therapy.
Subject(s)
Antibodies, Monoclonal, Murine-Derived/adverse effects , Antibodies, Monoclonal/adverse effects , Immunologic Factors/adverse effects , Rheumatic Diseases/drug therapy , Adult , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/drug therapy , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/physiopathology , Antibodies, Monoclonal/therapeutic use , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Arthritis, Rheumatoid/drug therapy , Arthritis, Rheumatoid/physiopathology , Female , Genetic Engineering , Humans , Immunologic Factors/therapeutic use , Infliximab , Lupus Erythematosus, Systemic/drug therapy , Lupus Erythematosus, Systemic/physiopathology , Male , Remission Induction/methods , Rheumatic Diseases/physiopathology , Rituximab , Severity of Illness Index , Treatment OutcomeABSTRACT
A case of a rare hereditary disease, Alagille-Watson syndrome, is reported. It most frequently affects liver and cardiovascular system which leads to biliary cirrhosis and cardiovascular catastrophe in young patients. Pathogenesis and clinical variants of the disease are described.
Subject(s)
Alagille Syndrome/pathology , Liver Cirrhosis, Biliary/etiology , Adult , Alagille Syndrome/complications , Alagille Syndrome/drug therapy , Female , Humans , Liver Cirrhosis, Biliary/drug therapy , Liver Cirrhosis, Biliary/pathology , Young AdultABSTRACT
Two clinical observations of the variant form of hepatic lesion: autoimmune hepatitis--primary biliary cirrhosis with systemic manifestations are presented in patients with long-standing Sjogren's syndrome, one at the stage of lever cirrhosis, the other at stage F2 of fibrosis. Difficulties encountered in diagnostics and the necessity of changing the entire spectrum of autoimmune markers characteristic of Sjogren's syndrome, autoimmune hepatitis and primary biliary cirrhosis are discussed. The possibility of different forms of hepatic lesions in autoimmune hepatitis--primary biliary cirrhosis is emphasized.
