BACKGROUND: Pauci-immune crescentic glomerulonephritis is one of the most common causes of rapidly progressive glomerulonephritis, usually associated with anti-neutrophil cytoplasmic antibody (ANCA) but a minority of patients lack ANCAs. To date no effective treatments have been addressed for ANCA-negative glomerulonephritis despite several studies describe the possibility to adopt pharmacologic therapies. Cellular, molecular and preclinical studies demonstrate that mesenchymal stem cells (MSCs) contribute to glomerular cell turnover and repair and might represent a new therapeutic approach for ANCA-negative glomerulonephritis. AIM: In our study we presented a case report of a patient with ANCA-negative pauci-immune glomerulonephritis in order to underlie the growing need of an alternative therapy for glomerular diseases. MATERIALS AND METHODS: We report the case of a 69 year-old woman who presented to our department with diarrhea, vomiting and increased serum creatinine levels. At the First Aid Department, renal ultrasonography excluded dilatations of urinary tract and since the occurrence of progressive dyspnea, anuria and severe hypertension renal replacement therapy was started. RESULTS: A second kidney Doppler ultrasonography showed hyperechoic parenchyma, reduced thickness and increased resistive index. Urinalysis revealed persistent active sediment and proteinuria. Autoimmunity profile showed C3, IgE and IgM increased levels. Renal biopsy was performed showing diffuse extracapillary proliferative glomerulonephritis with negative Immunofluorescence (IF). ANCA test was negative for a second time by both IF analysis and antigen-specific ELISA. The diagnosis was ANCA-negative glomerulonephritis and steroid bolus was administered, improving urinary sediment and proteinuria but not glomerular filtration rate. DISCUSSION: Our data demonstrated that pharmacological treatment of ANCA-negative glomerulonephritis correlated with poor response and is not effective, pointing out the importance of new therapeutic options for this disease. In this regard MCSs may represent a valid alternative in the treatment of ANCA-negative glomerulonephritis. CONCLUSIONS: MSCs may provide an effective therapeutic option in glomerular diseases and future studies should be performed in order to pave the way for this cell-based therapy.

ANCA-Negative Paucimmune Glomerulonephritis and Glomerular Recovery: Possible Role of Mesenchymal Stem Cells in a 69 Year-Old Patient / Zavatto, Assunta; Umbro, Ilaria; Fiacco, Federica; Valentina Di, Natale; Nodale, Cristina; Tinti, Francesca; Mitterhofer, Anna Paola. - In: CELLR4. - ISSN 2329-7042. - ELETTRONICO. - (2014).

ANCA-Negative Paucimmune Glomerulonephritis and Glomerular Recovery: Possible Role of Mesenchymal Stem Cells in a 69 Year-Old Patient

ZAVATTO, ASSUNTA;UMBRO, ILARIA;FIACCO, FEDERICA;NODALE, CRISTINA;TINTI, FRANCESCA;MITTERHOFER, Anna Paola
2014

Abstract

BACKGROUND: Pauci-immune crescentic glomerulonephritis is one of the most common causes of rapidly progressive glomerulonephritis, usually associated with anti-neutrophil cytoplasmic antibody (ANCA) but a minority of patients lack ANCAs. To date no effective treatments have been addressed for ANCA-negative glomerulonephritis despite several studies describe the possibility to adopt pharmacologic therapies. Cellular, molecular and preclinical studies demonstrate that mesenchymal stem cells (MSCs) contribute to glomerular cell turnover and repair and might represent a new therapeutic approach for ANCA-negative glomerulonephritis. AIM: In our study we presented a case report of a patient with ANCA-negative pauci-immune glomerulonephritis in order to underlie the growing need of an alternative therapy for glomerular diseases. MATERIALS AND METHODS: We report the case of a 69 year-old woman who presented to our department with diarrhea, vomiting and increased serum creatinine levels. At the First Aid Department, renal ultrasonography excluded dilatations of urinary tract and since the occurrence of progressive dyspnea, anuria and severe hypertension renal replacement therapy was started. RESULTS: A second kidney Doppler ultrasonography showed hyperechoic parenchyma, reduced thickness and increased resistive index. Urinalysis revealed persistent active sediment and proteinuria. Autoimmunity profile showed C3, IgE and IgM increased levels. Renal biopsy was performed showing diffuse extracapillary proliferative glomerulonephritis with negative Immunofluorescence (IF). ANCA test was negative for a second time by both IF analysis and antigen-specific ELISA. The diagnosis was ANCA-negative glomerulonephritis and steroid bolus was administered, improving urinary sediment and proteinuria but not glomerular filtration rate. DISCUSSION: Our data demonstrated that pharmacological treatment of ANCA-negative glomerulonephritis correlated with poor response and is not effective, pointing out the importance of new therapeutic options for this disease. In this regard MCSs may represent a valid alternative in the treatment of ANCA-negative glomerulonephritis. CONCLUSIONS: MSCs may provide an effective therapeutic option in glomerular diseases and future studies should be performed in order to pave the way for this cell-based therapy.
File allegati a questo prodotto
Non ci sono file associati a questo prodotto.

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11573/614182
 Attenzione

Attenzione! I dati visualizzati non sono stati sottoposti a validazione da parte dell'ateneo

Citazioni
  • ???jsp.display-item.citation.pmc??? ND
  • Scopus ND
  • ???jsp.display-item.citation.isi??? ND
social impact