Lupus nephritis with an unusual histopathological pattern

Detalhes bibliográficos
Autor(a) principal: Aveiro,Marcelo
Data de Publicação: 2021
Outros Autores: Pestana,Nicole, Silva,Francisca, Vieira,Pedro, Rosa,Nuno, Silva,Gil
Tipo de documento: Relatório
Idioma: eng
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692021000100051
Resumo: ABSTRACT Introduction: Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect multiple organs. When the kidney is involved it is generally termed lupus nephritis (LN), and is a major contributor to the morbidity and mortality associated with SLE. The classic pattern of lupus nephritis in a renal biopsy is an immune complex mediated glomerulonephritis. The presence of crescents, in diffuse proliferative lupus nephritis, and serologic positivity for antineutrophil cytoplasmatic antibodies (ANCA), more often antimyeloperoxidase than anti-proteinase 3, has been linked with specific clinicopathological features, poor treatment response and a worse kidney survival. Clinical case: We present the case of a 19-year-old male, without relevant past medical history, who presented severe headaches, hypertension and peripheral edema. The blood analysis revealed hemoglobin (Hb) 7.6 g/dL, creatinine (Cr) 2.64 mg/dL, blood urea nitrogen (BUN) 101 mg/dL, and urine analysis, hematuria and nephrotic proteinuria. The autoimmunity panel results were consistent with SLE paired with positivity for ANCA-proteinase 3 antibody. A renal biopsy revealed crescentic glomerulonephritis with fibrinoid necrosis and Bowman capsule rupture. The patient was diagnosed with class IV LN. The initiation treatment consisted of cyclophosphamide (CIPH) and prednisolone (PDN). At the 6-month follow-up, CIPH was stopped and mycophenolate mofetil (MMF) initiated as maintenance therapy, combined with PDN. Although microscopic hematuria and C3 consumption were still present (a new biopsy was pondered but the patient refused any further invasive diagnostic measures), C1q levels decreased from 26.6 to 19.3 U/ml (negative if < 20 U/ml) and anti-dsDNA was also negative with progressive declination of the degree of proteinuria. The creatinine levels returned to normal. Conclusion: The authors emphasize the importance of this class IV LN, influenced by the association with anti-proteinase 3, due to the implications in the histopathological pattern and in therapy selection. In this specific case kidney function, proteinuria and lupus activity had an important decrease without significant complications with the chosen treatment.
id RCAP_ccccc65dfb4236eb523979b8d585bcc6
oai_identifier_str oai:scielo:S0872-01692021000100051
network_acronym_str RCAP
network_name_str Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
repository_id_str 7160
spelling Lupus nephritis with an unusual histopathological patternLupus NephritisCyclophosphamideCrescentic GlomerulonephritisABSTRACT Introduction: Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect multiple organs. When the kidney is involved it is generally termed lupus nephritis (LN), and is a major contributor to the morbidity and mortality associated with SLE. The classic pattern of lupus nephritis in a renal biopsy is an immune complex mediated glomerulonephritis. The presence of crescents, in diffuse proliferative lupus nephritis, and serologic positivity for antineutrophil cytoplasmatic antibodies (ANCA), more often antimyeloperoxidase than anti-proteinase 3, has been linked with specific clinicopathological features, poor treatment response and a worse kidney survival. Clinical case: We present the case of a 19-year-old male, without relevant past medical history, who presented severe headaches, hypertension and peripheral edema. The blood analysis revealed hemoglobin (Hb) 7.6 g/dL, creatinine (Cr) 2.64 mg/dL, blood urea nitrogen (BUN) 101 mg/dL, and urine analysis, hematuria and nephrotic proteinuria. The autoimmunity panel results were consistent with SLE paired with positivity for ANCA-proteinase 3 antibody. A renal biopsy revealed crescentic glomerulonephritis with fibrinoid necrosis and Bowman capsule rupture. The patient was diagnosed with class IV LN. The initiation treatment consisted of cyclophosphamide (CIPH) and prednisolone (PDN). At the 6-month follow-up, CIPH was stopped and mycophenolate mofetil (MMF) initiated as maintenance therapy, combined with PDN. Although microscopic hematuria and C3 consumption were still present (a new biopsy was pondered but the patient refused any further invasive diagnostic measures), C1q levels decreased from 26.6 to 19.3 U/ml (negative if < 20 U/ml) and anti-dsDNA was also negative with progressive declination of the degree of proteinuria. The creatinine levels returned to normal. Conclusion: The authors emphasize the importance of this class IV LN, influenced by the association with anti-proteinase 3, due to the implications in the histopathological pattern and in therapy selection. In this specific case kidney function, proteinuria and lupus activity had an important decrease without significant complications with the chosen treatment.Sociedade Portuguesa de Nefrologia2021-03-01info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/reporttext/htmlhttp://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692021000100051Portuguese Journal of Nephrology &amp; Hypertension v.35 n.1 2021reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPenghttp://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692021000100051Aveiro,MarceloPestana,NicoleSilva,FranciscaVieira,PedroRosa,NunoSilva,Gilinfo:eu-repo/semantics/openAccess2024-02-06T17:05:09Zoai:scielo:S0872-01692021000100051Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-20T02:19:05.566736Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse
dc.title.none.fl_str_mv Lupus nephritis with an unusual histopathological pattern
title Lupus nephritis with an unusual histopathological pattern
spellingShingle Lupus nephritis with an unusual histopathological pattern
Aveiro,Marcelo
Lupus Nephritis
Cyclophosphamide
Crescentic Glomerulonephritis
title_short Lupus nephritis with an unusual histopathological pattern
title_full Lupus nephritis with an unusual histopathological pattern
title_fullStr Lupus nephritis with an unusual histopathological pattern
title_full_unstemmed Lupus nephritis with an unusual histopathological pattern
title_sort Lupus nephritis with an unusual histopathological pattern
author Aveiro,Marcelo
author_facet Aveiro,Marcelo
Pestana,Nicole
Silva,Francisca
Vieira,Pedro
Rosa,Nuno
Silva,Gil
author_role author
author2 Pestana,Nicole
Silva,Francisca
Vieira,Pedro
Rosa,Nuno
Silva,Gil
author2_role author
author
author
author
author
dc.contributor.author.fl_str_mv Aveiro,Marcelo
Pestana,Nicole
Silva,Francisca
Vieira,Pedro
Rosa,Nuno
Silva,Gil
dc.subject.por.fl_str_mv Lupus Nephritis
Cyclophosphamide
Crescentic Glomerulonephritis
topic Lupus Nephritis
Cyclophosphamide
Crescentic Glomerulonephritis
description ABSTRACT Introduction: Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect multiple organs. When the kidney is involved it is generally termed lupus nephritis (LN), and is a major contributor to the morbidity and mortality associated with SLE. The classic pattern of lupus nephritis in a renal biopsy is an immune complex mediated glomerulonephritis. The presence of crescents, in diffuse proliferative lupus nephritis, and serologic positivity for antineutrophil cytoplasmatic antibodies (ANCA), more often antimyeloperoxidase than anti-proteinase 3, has been linked with specific clinicopathological features, poor treatment response and a worse kidney survival. Clinical case: We present the case of a 19-year-old male, without relevant past medical history, who presented severe headaches, hypertension and peripheral edema. The blood analysis revealed hemoglobin (Hb) 7.6 g/dL, creatinine (Cr) 2.64 mg/dL, blood urea nitrogen (BUN) 101 mg/dL, and urine analysis, hematuria and nephrotic proteinuria. The autoimmunity panel results were consistent with SLE paired with positivity for ANCA-proteinase 3 antibody. A renal biopsy revealed crescentic glomerulonephritis with fibrinoid necrosis and Bowman capsule rupture. The patient was diagnosed with class IV LN. The initiation treatment consisted of cyclophosphamide (CIPH) and prednisolone (PDN). At the 6-month follow-up, CIPH was stopped and mycophenolate mofetil (MMF) initiated as maintenance therapy, combined with PDN. Although microscopic hematuria and C3 consumption were still present (a new biopsy was pondered but the patient refused any further invasive diagnostic measures), C1q levels decreased from 26.6 to 19.3 U/ml (negative if < 20 U/ml) and anti-dsDNA was also negative with progressive declination of the degree of proteinuria. The creatinine levels returned to normal. Conclusion: The authors emphasize the importance of this class IV LN, influenced by the association with anti-proteinase 3, due to the implications in the histopathological pattern and in therapy selection. In this specific case kidney function, proteinuria and lupus activity had an important decrease without significant complications with the chosen treatment.
publishDate 2021
dc.date.none.fl_str_mv 2021-03-01
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
dc.type.driver.fl_str_mv info:eu-repo/semantics/report
format report
status_str publishedVersion
dc.identifier.uri.fl_str_mv http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692021000100051
url http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692021000100051
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692021000100051
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv text/html
dc.publisher.none.fl_str_mv Sociedade Portuguesa de Nefrologia
publisher.none.fl_str_mv Sociedade Portuguesa de Nefrologia
dc.source.none.fl_str_mv Portuguese Journal of Nephrology &amp; Hypertension v.35 n.1 2021
reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron:RCAAP
instname_str Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron_str RCAAP
institution RCAAP
reponame_str Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
collection Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
repository.name.fl_str_mv Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
repository.mail.fl_str_mv
_version_ 1799137280761266176