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When Anti-Neutrophil Cytoplasmic Antibody Fails: A Case of Anti-Neutrophil Cytoplasmic Antibody Negative Granulomatosis With Polyangiitis

Granulomatosis with polyangiitis (GPA) is a vasculitis of small and medium-sized vessels and presents with varying signs and symptoms. It includes upper and lower airway manifestations and glomerulonephritis with a positive antineutrophil cytoplasmic antibody (ANCA) in serology in 90% of cases. Howe...

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Autores principales: Gangireddy, Mounika, Kanderi, Tejaswi, Chan Gomez, Janet, Kundoor, Vishwa, Cunningham, Jessica
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7388802/
https://www.ncbi.nlm.nih.gov/pubmed/32742850
http://dx.doi.org/10.7759/cureus.8883
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author Gangireddy, Mounika
Kanderi, Tejaswi
Chan Gomez, Janet
Kundoor, Vishwa
Cunningham, Jessica
author_facet Gangireddy, Mounika
Kanderi, Tejaswi
Chan Gomez, Janet
Kundoor, Vishwa
Cunningham, Jessica
author_sort Gangireddy, Mounika
collection PubMed
description Granulomatosis with polyangiitis (GPA) is a vasculitis of small and medium-sized vessels and presents with varying signs and symptoms. It includes upper and lower airway manifestations and glomerulonephritis with a positive antineutrophil cytoplasmic antibody (ANCA) in serology in 90% of cases. However, about 10% of cases with GPA can have negative serology, often resulting in a diagnostic delay. Obtaining a tissue pathology is needed to confirm GPA. Here we present a 77-year-old male who presented with generalized weakness and loss of appetite and was found to have glomerulonephritis and bilateral opacities in the lungs with a negative ANCA. He was diagnosed with ANCA negative granulomatosis with polyangiitis after a renal biopsy revealed necrotizing inflammation with crescent formation. He was successfully treated with systemic glucocorticoids and rituximab. In conclusion, prompt diagnosis and treatment of ANCA negative vasculitis are required to decrease mortality.
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spelling pubmed-73888022020-07-31 When Anti-Neutrophil Cytoplasmic Antibody Fails: A Case of Anti-Neutrophil Cytoplasmic Antibody Negative Granulomatosis With Polyangiitis Gangireddy, Mounika Kanderi, Tejaswi Chan Gomez, Janet Kundoor, Vishwa Cunningham, Jessica Cureus Internal Medicine Granulomatosis with polyangiitis (GPA) is a vasculitis of small and medium-sized vessels and presents with varying signs and symptoms. It includes upper and lower airway manifestations and glomerulonephritis with a positive antineutrophil cytoplasmic antibody (ANCA) in serology in 90% of cases. However, about 10% of cases with GPA can have negative serology, often resulting in a diagnostic delay. Obtaining a tissue pathology is needed to confirm GPA. Here we present a 77-year-old male who presented with generalized weakness and loss of appetite and was found to have glomerulonephritis and bilateral opacities in the lungs with a negative ANCA. He was diagnosed with ANCA negative granulomatosis with polyangiitis after a renal biopsy revealed necrotizing inflammation with crescent formation. He was successfully treated with systemic glucocorticoids and rituximab. In conclusion, prompt diagnosis and treatment of ANCA negative vasculitis are required to decrease mortality. Cureus 2020-06-28 /pmc/articles/PMC7388802/ /pubmed/32742850 http://dx.doi.org/10.7759/cureus.8883 Text en Copyright © 2020, Gangireddy et al. http://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Internal Medicine
Gangireddy, Mounika
Kanderi, Tejaswi
Chan Gomez, Janet
Kundoor, Vishwa
Cunningham, Jessica
When Anti-Neutrophil Cytoplasmic Antibody Fails: A Case of Anti-Neutrophil Cytoplasmic Antibody Negative Granulomatosis With Polyangiitis
title When Anti-Neutrophil Cytoplasmic Antibody Fails: A Case of Anti-Neutrophil Cytoplasmic Antibody Negative Granulomatosis With Polyangiitis
title_full When Anti-Neutrophil Cytoplasmic Antibody Fails: A Case of Anti-Neutrophil Cytoplasmic Antibody Negative Granulomatosis With Polyangiitis
title_fullStr When Anti-Neutrophil Cytoplasmic Antibody Fails: A Case of Anti-Neutrophil Cytoplasmic Antibody Negative Granulomatosis With Polyangiitis
title_full_unstemmed When Anti-Neutrophil Cytoplasmic Antibody Fails: A Case of Anti-Neutrophil Cytoplasmic Antibody Negative Granulomatosis With Polyangiitis
title_short When Anti-Neutrophil Cytoplasmic Antibody Fails: A Case of Anti-Neutrophil Cytoplasmic Antibody Negative Granulomatosis With Polyangiitis
title_sort when anti-neutrophil cytoplasmic antibody fails: a case of anti-neutrophil cytoplasmic antibody negative granulomatosis with polyangiitis
topic Internal Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7388802/
https://www.ncbi.nlm.nih.gov/pubmed/32742850
http://dx.doi.org/10.7759/cureus.8883
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