Rituximab Treatment in a Patient with Kimura Disease and Membranous Nephropathy: Case Report

Abstract

Kimura disease (KD) is a chronic, inflammatory disorder with slowly developing subcutaneous tumor-like swellings, often occurring in the head and neck region. KD is diagnosed based on histology, elevated levels of immunoglobulin type E, and increased peripheral eosinophil granulocytes. KD may coexist with glomerular renal diseases, and this case report is based on a patient with KD-associated membranous nephropathy. Patients with membranous nephropathy without KD have demonstrated responsiveness to treatment with monoclonal anti-CD20 antibodies. This case report is the first to investigate the effect of rituximab treatment in a patient with KD-associated membranous nephropathy. A 30-year-old Italian man living in Denmark was diagnosed with Kimura’s disease based on subcutaneous nodules with eosinophil angiolymphoid hyperplasia. The patient was admitted to the hospital due to nephrotic syndrome. Serology showed eosinophil granulocytosis and negative PLA2-receptor antibody. Renal biopsy showed membranous nephropathy, and the patient was treated with systemic methylprednisolone followed by cyclosporin and then cyclophosphamide with only partial remission. Ultimately, treatment with intravenous rituximab was initiated, which resulted in overall remission and no nephrotic relapses at 30 months of follow-up. Thus, intravenous rituximab effectively decreased proteinuria and prevented nephrotic relapses in a patient with treatment-refractory membranous nephropathy due to KD.

Anti‐immunoglobulin E provides an additional therapy to conventional steroids for Kimura's disease

“…Although systemic steroids are widely used (prednisolone 20-50 mg/day) and the majority of patients exhibit initial responses, reoccurrence is frequent after its cessation (about 45%). 2,7,8 Moreover, common and various side effects usually cause benefit debating of steroids in KD.…”

“…Without B cells that could program to plasma cells, IgE production will be substantially decreased. 8,11 Regarding related mechanisms, we reviewed 13 KD cases that were treated with the abovementioned biologics (Table 1). [5][6][7][9][10][11][12][13][14][15] From these reports, all patients obtained disease remission and none had recurrence during follow-up.…”

“…[7][8][9]11,14,15 Two KD cases (case 12 and 13) that underwent rituximab therapy also had skin lymphoma and kidney disease, indicating that rituximab might be an option for KD accompanied by severe systemic comorbidities. 8,11 In addition to renal involvement, other complications from the reviewed cases include allergic diseases, ulcerative colitis, aortitis syndrome, endocarditis, Buerger's disease-like vasculitis, and Raynaud's phenomenon. 5,[7][8][9]11,14,15 Case 7 had eczema and responded well to dupilumab, suggesting dupilumab may be an alternative option for KD with allergic conditions.…”

Challenging diagnosis of Kimura disease in a child with nephrotic syndrome: A case report

“…Kimura disease (KD) is a rare, inflammatory, idiopathic condition characterized by painless, slow‐growing subcutaneous masses frequently in the head and neck region, 1,2 that occurs more commonly in young males with a peak incidence in their second to fourth decades of life 3 …”

Challenging diagnosis of Kimura disease in a child with nephrotic syndrome: A case report

“…Kimura disease (KD) is a rare, inflammatory, idiopathic condition characterized by painless, slow-growing subcutaneous masses frequently in the head and neck region (1,2), that occurs more commonly in young males with a peak incidence in their second to fourth decades of life (3).…”