Anca negative pauci-immune crescentic glomerulonephritis and mixed connective tissue disease: a case study

Abstract

One of the most common causes of rapidly progressive glomerulonephritis (RPGN) is pauci-immune crescentic glomerulonephritis (CrGN). In the majority of cases, this condition has a positive serologic marker, the anti-neutrophil cytoplasmic antibodies (ANCAs), but in approximately 10% there are no circulating ANCAs, and this subgroup has been known as the ANCA-negative pauci-immune CrGN. RPGN can be associated with systemic diseases, but there are only few case reports describing the association with mixed connective tissue disease (MCTD). The authors report a case of ANCA-negative CrGN associated with a MCTD.

Ocorrência de doencas sistêmicas do tecido conjuntivo – um olhar sobre os acometimentos antes e durante a vigência da pandemia de Covid 19 e as terapias medicamentosas e propositivas

“…Algumas doenças do tecido conjuntivo são por vezes mais dificieis de serem disgnóticadas, dado sua rara ocorrência. É o que relata Fernandes et al (2019). Segundo esses autores, uma das causas mais comuns da glomerulonefrite rapidamente progressiva (GNRP) é a glomerulonefrite crescêntica (GNC) pauci-imune.…”

Glomerulonefritis de rápida progresión pauciinmunitaria negativa a ANCA en un paciente de 18 años: a propósito de un caso

“…A pesar de representar las GNRP pauciinmunitarias negativas a ANCA un porcentaje pequeño de las GNRP de tipo III, y no conocer aún su etiología, se han descrito en fecha reciente casos en contextos de situaciones clínicas específicas, entre ellas las siguientes: el consumo de cocaína vinculado con levamisol en un paciente de 38 años 9 , la utilización de nidostaurina (fármaco quimioterápico) en un paciente de 61 años portador de leucemia mieloide aguda 10 , un caso de un paciente de 64 años con diagnóstico de cáncer de pulmón microcítico y otros ejemplos relacionados con dicha malformación oncológica 11 y un último caso vinculado con enfermedad de tejido mixto conectivo 12 .…”

“…Se ha destacado que en todos los casos clínicos ya mencionados de GNRP pauciinmunitaria negativa a ANCA se instituyó el mismo tratamiento médico: bolos de metilprednisolona y ciclos de ciclofosfamida, seguidos de prednisona oral, todos con buena respuesta clínica y paraclínica [9][10][11][12] .…”

Anti-neutrophil Cytoplasmic Antibody-Negative Rapid Progressive Glomerulonephritis With Mild Pathological Presentation in an Older Patient: A Case Report

“…The anti-neutrophil cytoplasmic antibodies (ANCA) biomarker plays a crucial role in diagnosing and managing RPGN, guiding clinicians in the prognosis and intensifying treatment with cyclophosphamide and rituximab in ANCApositive cases [3,4]. However, managing ANCA-negative RPGN presents a considerable challenge, especially in settings with limited access to diagnostic resources like renal biopsies, often seen in rural areas [5,6]. This time, we experienced a case of ANCA negative RPGN in an older man.…”

“…However, managing ANCA-negative RPGN presents a considerable challenge, especially in settings with limited access to diagnostic resources like renal biopsies, often seen in rural areas [ 5 , 6 ]. This time, we experienced a case of ANCA negative RPGN in an older man.…”