Bortezomib-containing regiment in treating glomerulopathy with fibronectin deposits combined with monoclonal gammopathy of undetermined significance: a case report and literature review

Abstract

Background: Glomerulopathy with fibronectin deposits (GFND) is a newly recognized rare glomerular disease. As its onset can be stably inherited in affected families without sex differences and fibronectin 1 (FN1) mutations can be detected in 40% of patients' families, GFND is considered to be an autosomal dominant genetic disease. The main clinical manifestations are proteinuria, progressive renal failure, edema, hypertension, hematuria, and type 4 renal tubular acidosis. The diagnosis was confirmed by renal biopsy, and there was no specific treatment. Monoclonal gammopathy refers to the existence of monoclonal immunoglobulin (MIg) produced by monoclonal plasma cells in serum. When MIg damages the kidney by direct deposition or indirect mechanisms, it is defined as monoclonal gammopathy of renal significance (MGRS). The principle of treatment is to inhibit plasma cells from producing MIg.Case Description: We report the efficacy of a case of GFND combined with monoclonal gammopathy of undetermined significance (MGUS) treated with a bortezomib-containing regimen. A 44-year-old female patient was admitted to the hospital for "edema of both lower extremities for 1 month and aggravation for 5 days". In May 2018, after exertion, the patient developed edema of both lower extremities, accompanied by foamy urine with no obvious deepening of urine color or decreased output, no gross hematuria, and gradual aggravation with fatigue. Conclusion:After treatment, the edema of patient subsided, urinary protein decreased significantly, and serum albumin increased near to normal. It is achieving a very good therapeutic effect and long-term event-free survival.The treatment is safety and there are no obvious toxic side effects. It provides a new idea for the treatment of GFND.