Henoch-Schönlein purpura nephritis associated with monoclonal gammopathy of renal significance: a case report

Abstract

Monoclonal gammopathy of renal significance (MGRS) can present with different morphologic features and lead to kidney failure. The Henoch-Schönlein purpura nephritis (HSPN) that cannot be relieved by treatment with glucocorticoid and immunosuppressive agents suggests the presence of monoclonal gammopathy in adult patients. The present study reports on a single case of HSPN associated with IgA-κMGRS. The patient who suffered from recurrent skin purpura for 6 months and nephrotic syndrome for 2 months was admitted to our hospital. Bone marrow biopsy showed monoclonal gammopathy of undetermined significance. Kidney biopsy indicated a Henoch-Schönlein purpura nephritis (HSPN, ISKDC classified as type III) with positive staining with κ-light chain in the glomeruli and renal tubular epithelial cells. Furthermore, skin biopsy showed leukocytoclastic vasculitis and negative staining for Congo red and light chain. Given both the renal and cutaneous involvement, the patient was considered to have HSPN associated with IgA-κMGRS. The patient experienced an exacerbation in his purpura-like lesions and clinical status after treatment with glucocorticoid and immunosuppressive agents. Consequently, the patient was put on a regimen that included dexamethasone (20 mg on the 1st, 4th, 8th, and 11th days of each month, iv ) and bortezomib (2.4 mg on the 1st, 4th, 8th, and 11th days of each month, iv ). Eight weeks after treatment, he had complete resolution of his cutaneous purpura and his biochemical parameters improved. The latent presence of MGRS in cases of HSPN should be considered in adult patients. Increased cognizance and correct treatment options could improve patient outcomes.

Effects of dexamethasone and gamma globulin combined with prednisone on the therapeutic effect and immune function of Henoch‐Schonlein purpura nephritis in children

“…In the initial stage, children with HSP often show extrarenal symptoms like skin purpura, joint pain, and hemorrhagic gastroenteritis and, in some cases, manifest as asymptomatic urine abnormalities. Generally, as the disease progresses, there will be renal injury of varying severity, glomerular damage, and interference with the reabsorption function of trace albumin 1,2 . While as a serious complication of HSP, Henoch‐Schonlein purpura nephritis (HSPN) is a kind of systemic disease with impaired renal parenchyma, which is more prevalent in children 3,4 .…”

“…Generally, as the disease progresses, there will be renal injury of varying severity, glomerular damage, and interference with the reabsorption function of trace albumin. 1,2 While as a serious complication of HSP, Henoch-Schonlein purpura nephritis (HSPN) is a kind of systemic disease with impaired renal parenchyma, which is more prevalent in children. 3,4 In most cases, it can be diagnosed by the occurrence of hematuria or proteinuria in the course of disease within 6 months in clinic.…”