Počki (Mar 2018)
Clinical case of successful treatment of Goodpasture syndrome with rituximab
Abstract
A clinical case of the successful use of rituximab in a patient with severe course of Goodpasture syndrome is described. The patient was admitted to the hospitaal in extremely serious condition with the manifestations of respiratory and renal failure, which required constant respiratory support and hemodialysis. The diagnosis of Goodpasture syndrome was made on the ground of isolated lung and kidney damage as a type of rapidly progressing glomerulonephritis and hemorrhagic alveolitis, high titers of anti-glomerular basement membrane antibodies (twice), no increase in the level of other antibodies specific for systemic connective tissue diseases and systemic vasculitis. The patient underwent synchronous pulse therapy with sequential application of cascade plasma filtration, cyclophosphamide and methylprednisolone, without significant improvement. The rationale for the use of rituximab in Goodpasture syndrome was literature data, description of clinical cases, as well as a more than 2-fold increase in CD19+, a high level of anti-glomerular basement membrane antibodies, anemic syndrome and thrombocytopenia, as well as failure of previous immunosuppressive therapy. Therapy with rituximab was performed at a dose of 1000 mg twice with a break of 14 days. A significant improvement in the clinical condition of the patient was noted, as well as restoration of ability to spontaneously breathe, the absence of dyspnoea at rest and on exertion, the absence of hemoptysis, the normalization of platelet and hemoglobin indices, the improvement of lung picture on computed tomogram, and a repeated blood test for antibodies to the basal membranes of the glomerular apparatus indicated their absence. Rituximab therapy conducted in the first month after the diagnosis of Goodpasture syndrome contributed to a positive change in the course of the disease, almost complete reversal of lung damage, elimination of life-threatening cytopenia and, ultimately, saving of the patient’s life. As in a number of other clinical observations, treatment with rituximab was ineffective in restoring kidney function.
Keywords