Henoch-Schönlein purpura nephritis associated with monoclonal gammopathy of renal significance: a case report
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 gammo...
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doaj-3088cfdf3ebd4eab80eb31693b7daf182020-11-25T02:04:07ZengAssociação Brasileira de Divulgação CientíficaBrazilian Journal of Medical and Biological Research1414-431X52710.1590/1414-431x20198222S0100-879X2019000700501Henoch-Schönlein purpura nephritis associated with monoclonal gammopathy of renal significance: a case reportHui ZhaoWen-hui HuangJun-yue HuangShou-yan LuYa-hong YangZhi-gang MaMonoclonal 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.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2019000700501&lng=en&tlng=enMonoclonal gammopathy of renal significanceHenoch-Schönlein purpura nephritisChemotherapy |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Hui Zhao Wen-hui Huang Jun-yue Huang Shou-yan Lu Ya-hong Yang Zhi-gang Ma |
spellingShingle |
Hui Zhao Wen-hui Huang Jun-yue Huang Shou-yan Lu Ya-hong Yang Zhi-gang Ma Henoch-Schönlein purpura nephritis associated with monoclonal gammopathy of renal significance: a case report Brazilian Journal of Medical and Biological Research Monoclonal gammopathy of renal significance Henoch-Schönlein purpura nephritis Chemotherapy |
author_facet |
Hui Zhao Wen-hui Huang Jun-yue Huang Shou-yan Lu Ya-hong Yang Zhi-gang Ma |
author_sort |
Hui Zhao |
title |
Henoch-Schönlein purpura nephritis associated with monoclonal gammopathy of renal significance: a case report |
title_short |
Henoch-Schönlein purpura nephritis associated with monoclonal gammopathy of renal significance: a case report |
title_full |
Henoch-Schönlein purpura nephritis associated with monoclonal gammopathy of renal significance: a case report |
title_fullStr |
Henoch-Schönlein purpura nephritis associated with monoclonal gammopathy of renal significance: a case report |
title_full_unstemmed |
Henoch-Schönlein purpura nephritis associated with monoclonal gammopathy of renal significance: a case report |
title_sort |
henoch-schönlein purpura nephritis associated with monoclonal gammopathy of renal significance: a case report |
publisher |
Associação Brasileira de Divulgação Científica |
series |
Brazilian Journal of Medical and Biological Research |
issn |
1414-431X |
description |
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. |
topic |
Monoclonal gammopathy of renal significance Henoch-Schönlein purpura nephritis Chemotherapy |
url |
http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2019000700501&lng=en&tlng=en |
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