VANCA-associated vasculitis: a likely overlap with lupus?
ANCA-associated vasculitides are defined as necrotizing vasculitis with infiltration of neutrophils and monocytes in the vessel walls and characterized by the absence of immunoglobulin deposition (pauciimmune). They may be confused with Systemic Lupus Erythematosus (SLE) or may be superimposed on th...
| Publicado en: | Minas Gerais Medical Journal / Revista Médica de Minas Gerais Vol. 34; no. 4; pp. 1 - 8 |
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| Autores principales: | , |
| Formato: | case study diagnostic images pictorial tables/charts Journal Article |
| Publicado: |
Associacao Medica de Minas Gerais
2024
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=183017603&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 183017603 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 0103880X MXCZ jtl: Minas Gerais Medical Journal / Revista Médica de Minas Gerais issn: 0103880X maglogo: N pubinfo: dt: 2024 vid: 34 iid: 4 pid: 38810 pub: Associacao Medica de Minas Gerais artinfo: ui: 183017603 183017603 183017603 10.5935/2238-3182.2024e34403-en 183017603 ppf: 1 ppct: 7 formats: fmt: @attributes: type: P tig: atl: VANCA-associated vasculitis: a likely overlap with lupus? aug: au: Bianca Manfredini, Gabriela Larocca Skare, Thelma affil: Hospital Evangélico Mackenzie, Curitiba, Paraná, Brazil sug: subj: Antibodies, Antineutrophil Cytoplasmic Adverse Effects Vasculitis Risk Factors Risk Assessment Vasculitis Diagnosis Lupus Erythematosus, Systemic Diagnosis, Differential Vasculitis Drug Therapy Female Adult Brazil Physical Examination Tomography, X-Ray Computed Kidney Pathology Biopsy Methylprednisolone Therapeutic Use Prednisone Therapeutic Use Cyclophosphamide Therapeutic Use Adult: 19-44 years Female ab: ANCA-associated vasculitides are defined as necrotizing vasculitis with infiltration of neutrophils and monocytes in the vessel walls and characterized by the absence of immunoglobulin deposition (pauciimmune). They may be confused with Systemic Lupus Erythematosus (SLE) or may be superimposed on this disease. In this case report, the possible concomitance of an ANCA-associated vasculitis (granulomatosis with polyangiitis or GPA and microscopic polyangiitis or PAM) with SLE is shown. The patient opened the picture with cutaneous manifestations, arthralgias and arthritis, in addition to respiratory symptoms. She had renal dysfunction requiring hemodialysis, with a renal biopsy compatible with GPA, pauci-immune glomerulonephritis type, and alveolar hemorrhage. Furthermore, she was positive for the anti-dsDNA antibody suggesting the diagnostic possibility of SLE. The presented ANCA had an ANCA-p pattern, while the one expected in GPA would be ANCA-c. For this reason, microscopic polyangiitis (MPA) can also be considered as a possible diagnosis, given the similarities of manifestations. As for the overlap with SLE, its concomitance with GPA is known in the literature. Therefore, the probable final diagnosis of the condition presented was ANCA-p vasculitis with overlapping SLE. As vasculites associadas ao ANCA são definidas como vasculites necrosantes com infiltração de neutrófilos e monócitos na parede dos vasos e que se caracterizam por ausência de deposição de imunoglobulinas (pauci-imune). Podem ser confundidas com Lúpus Eritematoso Sistêmico (LES) ou podem estar sobrepostas a essa doença. Neste relato de caso, mostra-se a possível concomitância de uma vasculite ANCA associada (granulomatose com poliangeíte ou GPA e poliangeíte microscópica ou PAM) com LES. A paciente abriu o quadro com manifestações cutâneas, artralgias e artrites, além de sintomas respiratórios. Apresentou disfunção renal com necessidade de hemodiálise, com biópsia renal compatível com GPA, do tipo glomerulonefrite pauci-imune, e hemorragia alveolar. Além disso, tinha positividade para o anticorpo anti-dsDNA sugerindo a possibilidade diagnóstica de LES. O ANCA apresentado possuía padrão do tipo ANCA-p, enquanto o esperado em GPA seria o de ANCA-c. Por esse motivo, pode-se, também, considerar a poliangeíte microscópica (PAM) como possível diagnóstico, visto as semelhanças das manifestações. Quanto à sobreposição com LES, a sua concomitância com GPA é conhecida na literatura. Portanto, o diagnóstico final provável do quadro apresentado foi o de uma vasculite ANCA-p com sobreposição de LES. pubtype: Academic Journal doctype: case study diagnostic images pictorial tables/charts Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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