Publicação
Can cryoglobulinemia trigger ANCA vasculitis?
| Resumo: | Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a rare and heterogeneous group of autoimmune diseases. These pauciimmune vasculitis differ from the cryoglobulinemic vasculitis mediated by immune complexes. We report the case of a 79-year-old male with silicosis and chronic alcoholic liver disease, presenting with a rapidly progressive glomerulonephritis and pancytopenia. Work-up revealed hypocomplementemia, polyclonal hypergammaglobulinemia, type 3 cryoglobulinemia, p-ANCA positivity and elevated anti-MPO. Renal biopsy showed a pattern of chronic interstitial nephritis with fibrocellular crescents, and immunofluorescence staining was negative. Treatment was started with corticosteroids and rituximab with improvement of the renal function, decrease of the anti-MPO titer and disappearance of the cryoglobulinemia. In this case, renal injury was caused by ANCA vasculitis, whose etiology remains unknown despite the recognizable risk factor for ANCA formation (silicosis). The importance of the cryoglobulinemia is not clear, as it could be part of the pathogenesis or just an epiphenomenon secondary to the autoimmune and the chronic liver diseases. |
|---|---|
| Autores principais: | Cunha,Ivo |
| Outros Autores: | Santos,M. Teresa; Pereira,P. Ricardo |
| Assunto: | Crescents cryoglobulinemia interstitial nephritis rapidly progressive glomerulonephritis vasculitis |
| Ano: | 2020 |
| País: | Portugal |
| Tipo de documento: | relatório |
| Tipo de acesso: | acesso aberto |
| Instituição associada: | Fundação para a Ciência e Tecnologia |
| Idioma: | inglês |
| Origem: | SciELO Portugal |
| _version_ | 1868441695742328832 |
|---|---|
| author | Cunha,Ivo |
| author2 | Santos,M. Teresa Pereira,P. Ricardo |
| author2_role | author author |
| author_facet | Cunha,Ivo Santos,M. Teresa Pereira,P. Ricardo |
| author_role | author |
| country_str | PT |
| creators_json_txt | [{\"Person.name\":\"Cunha,Ivo\"},{\"Person.name\":\"Santos,M. Teresa\"},{\"Person.name\":\"Pereira,P. Ricardo\"}] |
| datacite.creators.creator.creatorName.fl_str_mv | Cunha,Ivo Santos,M. Teresa Pereira,P. Ricardo |
| datacite.rights.fl_str_mv | http://purl.org/coar/access_right/c_abf2 |
| datacite.subjects.subject.fl_str_mv | Crescents cryoglobulinemia interstitial nephritis rapidly progressive glomerulonephritis vasculitis |
| datacite.titles.title.fl_str_mv | Can cryoglobulinemia trigger ANCA vasculitis? |
| dc.creator.none.fl_str_mv | Cunha,Ivo Santos,M. Teresa Pereira,P. Ricardo |
| dc.format.none.fl_str_mv | text/html |
| dc.identifier.none.fl_str_mv | http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692020000100009 |
| dc.language.none.fl_str_mv | eng |
| dc.publisher.none.fl_str_mv | Sociedade Portuguesa de Nefrologia |
| dc.rights.none.fl_str_mv | http://purl.org/coar/access_right/c_abf2 |
| dc.source.none.fl_str_mv | Portuguese Journal of Nephrology & Hypertension v.34 n.1 2020 |
| dc.subject.none.fl_str_mv | Crescents cryoglobulinemia interstitial nephritis rapidly progressive glomerulonephritis vasculitis |
| dc.title.fl_str_mv | Can cryoglobulinemia trigger ANCA vasculitis? |
| dc.type.none.fl_str_mv | http://purl.org/coar/resource_type/c_93fc |
| description | Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a rare and heterogeneous group of autoimmune diseases. These pauciimmune vasculitis differ from the cryoglobulinemic vasculitis mediated by immune complexes. We report the case of a 79-year-old male with silicosis and chronic alcoholic liver disease, presenting with a rapidly progressive glomerulonephritis and pancytopenia. Work-up revealed hypocomplementemia, polyclonal hypergammaglobulinemia, type 3 cryoglobulinemia, p-ANCA positivity and elevated anti-MPO. Renal biopsy showed a pattern of chronic interstitial nephritis with fibrocellular crescents, and immunofluorescence staining was negative. Treatment was started with corticosteroids and rituximab with improvement of the renal function, decrease of the anti-MPO titer and disappearance of the cryoglobulinemia. In this case, renal injury was caused by ANCA vasculitis, whose etiology remains unknown despite the recognizable risk factor for ANCA formation (silicosis). The importance of the cryoglobulinemia is not clear, as it could be part of the pathogenesis or just an epiphenomenon secondary to the autoimmune and the chronic liver diseases. |
| dirty | 0 |
| eu_rights_str_mv | openAccess |
| format | report |
| id | scielopt_daf4474033efc4ff6567b4f2e97cc3da |
| identifier.url.fl_str_mv | http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692020000100009 |
| instacron_str | SciELO |
| institution | Fundação para a Ciência e Tecnologia |
| instname_str | Fundação para a Ciência e Tecnologia |
| language | eng |
| network_acronym_str | scielopt |
| network_name_str | SciELO Portugal |
| oai_identifier_str | oai:scielo:S0872-01692020000100009 |
| organization_str_mv | urn:organizationAcronym:scielo |
| person_str_mv | Cunha,Ivo Santos,M. Teresa Pereira,P. Ricardo |
| publishDate | 2020 |
| publisher.none.fl_str_mv | Sociedade Portuguesa de Nefrologia |
| reponame_str | SciELO Portugal |
| repository_id_str | urn:repositoryAcronym:scielopt |
| service_str_mv | urn:repositoryAcronym:scielopt |
| spelling | Can cryoglobulinemia trigger ANCA vasculitis?Cunha,IvoSantos,M. TeresaPereira,P. RicardoCrescentscryoglobulinemiainterstitial nephritisrapidly progressive glomerulonephritisvasculitisopen accesshttp://purl.org/coar/access_right/c_abf2http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692020000100009URLhttp://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692020000100009URLHasVersion2020-03-01Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a rare and heterogeneous group of autoimmune diseases. These pauciimmune vasculitis differ from the cryoglobulinemic vasculitis mediated by immune complexes. We report the case of a 79-year-old male with silicosis and chronic alcoholic liver disease, presenting with a rapidly progressive glomerulonephritis and pancytopenia. Work-up revealed hypocomplementemia, polyclonal hypergammaglobulinemia, type 3 cryoglobulinemia, p-ANCA positivity and elevated anti-MPO. Renal biopsy showed a pattern of chronic interstitial nephritis with fibrocellular crescents, and immunofluorescence staining was negative. Treatment was started with corticosteroids and rituximab with improvement of the renal function, decrease of the anti-MPO titer and disappearance of the cryoglobulinemia. In this case, renal injury was caused by ANCA vasculitis, whose etiology remains unknown despite the recognizable risk factor for ANCA formation (silicosis). The importance of the cryoglobulinemia is not clear, as it could be part of the pathogenesis or just an epiphenomenon secondary to the autoimmune and the chronic liver diseases.Sociedade Portuguesa de NefrologiaPortuguese Journal of Nephrology & Hypertension v.34 n.1 2020text/htmlengreporthttp://purl.org/coar/resource_type/c_93fcother research product |
| spellingShingle | Can cryoglobulinemia trigger ANCA vasculitis? Cunha,Ivo Crescents cryoglobulinemia interstitial nephritis rapidly progressive glomerulonephritis vasculitis |
| status | SINGLETON |
| subject.fl_str_mv | Crescents cryoglobulinemia interstitial nephritis rapidly progressive glomerulonephritis vasculitis |
| title | Can cryoglobulinemia trigger ANCA vasculitis? |
| title_full | Can cryoglobulinemia trigger ANCA vasculitis? |
| title_fullStr | Can cryoglobulinemia trigger ANCA vasculitis? |
| title_full_unstemmed | Can cryoglobulinemia trigger ANCA vasculitis? |
| title_short | Can cryoglobulinemia trigger ANCA vasculitis? |
| title_sort | Can cryoglobulinemia trigger ANCA vasculitis? |
| topic | Crescents cryoglobulinemia interstitial nephritis rapidly progressive glomerulonephritis vasculitis |
| topic_facet | Crescents cryoglobulinemia interstitial nephritis rapidly progressive glomerulonephritis vasculitis |
| url | http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692020000100009 |
| visible | 1 |