The ineffectiveness of aggressive immunosuppressive therapy, biological therapy and plasmapheresis in the treatment of juvenile systemic lupus erythematosus with non-proliferative form of lupus nephritis
Journal Title: Postępy Nauk Medycznych - Year 2013, Vol 26, Issue 5
Abstract
The paper presents a case of a 20-year-old female patient, treated from the age of 13 due to familial systemic lupus erythematosus. From the beginning of its onset, the disease was characterized by high clinical and serological activity. The clinical picture was dominated by neuropathic symptoms with a progressive tendency: from microscopic hematuria (erythrocyturia), through nephritic syndrome with hypertension to chronic kidney disease. Renal biopsy performed during the first year of treatment revealed class II lupus nephritis, while a rebiopsy performed after four years of unsuccessful immunosuppressive therapy revealed class IIa nephritis with progression towards FSGS. Data from the literature indicate that proliferative forms of nephritis (WHO class III or IV) are most prevalent in young people. The patient had been receiving aggressive, repeatedly modified immunosuppressive treatment comprising the administration of cyclophosphamide, imuran, methylprednisolone, followed by mycophenolate mofetil, cyclosporin A and finally rituximab as well as plasmaphereses for the period of 7 years. Unfortunately, no reduction in clinical or serological activity was achieved. Additionally, symptoms of severe haematological dysfunction, pancreatitis as well as pericarditis occurred in the clinical course. After 7 years of aggressive therapy, the patient reached stage V chronic kidney disease and renal replacement therapy was initiated. This therapeutic failure probably resulted from the accumulation of several unfavourable factors including a positive family history, disease onset at puberty as well as atypical pathomorphological form of nephropathy.
Authors and Affiliations
Lidia Hyla-Klekot, Grażyna Kucharska, Karina Słonka, Katarzyna Karwicka
Keywords
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