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A case of eosinophilic granulomatosis with polyangiitis complicated with a similar condition to IgG4 related lung disease

BACKGROUND: Atypical manifestations, such as elevated serum immunoglobulin-G4 (IgG4) and extra-pulmonary IgG4 positive plasmacyte infiltration, have been described in patients with eosinophilic granulomatosis with polyangiitis (EGPA), such complicated situation might not be readily differentiated fr...

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Autores principales: Zhou, Li, Cao, Fen, Fan, Songqing, Chen, Ping, Ding, Shuizi, Liu, Guiqian, Ouyang, Ruoyun
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6701049/
https://www.ncbi.nlm.nih.gov/pubmed/31426769
http://dx.doi.org/10.1186/s12890-019-0917-4
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author Zhou, Li
Cao, Fen
Fan, Songqing
Chen, Ping
Ding, Shuizi
Liu, Guiqian
Ouyang, Ruoyun
author_facet Zhou, Li
Cao, Fen
Fan, Songqing
Chen, Ping
Ding, Shuizi
Liu, Guiqian
Ouyang, Ruoyun
author_sort Zhou, Li
collection PubMed
description BACKGROUND: Atypical manifestations, such as elevated serum immunoglobulin-G4 (IgG4) and extra-pulmonary IgG4 positive plasmacyte infiltration, have been described in patients with eosinophilic granulomatosis with polyangiitis (EGPA), such complicated situation might not be readily differentiated from IgG4-related disease. CASE PRESENTATION: Here, we report an interesting and rare case of EGPA in a 41 year-old male with negative anti-neutrophil cytoplasmic antibodies (ANCAs), which showed abundant pulmonary IgG4-positive plasma cells infiltration and markedly elevated serum IgG4 levels without extra-pulmonary lesions of IgG4-related disease. The clinical characteristics hesitated us whether the diagnosis as EGPA overlapping with IgG4-related lung disease should be concluded because of the absence of storiform fibrosis and obliterative phlebitis with lymphoplasmacytic infiltration. The patient’s systemic symptoms, pulmonary lesions, blood eosinophils count / percentage, and serum IgG4 levels were significantly improved with immunosuppressive therapy. CONCLUSIONS: We consider that the overlapping pathogenesis exists in the disease course of EGPA and IgG4-related disease, which may lead to interaction during the course of the diseases. And the utilization of diagnostic criteria for IgG4-related lung disease with the careful differentiation is needed in such cases.
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spelling pubmed-67010492019-08-26 A case of eosinophilic granulomatosis with polyangiitis complicated with a similar condition to IgG4 related lung disease Zhou, Li Cao, Fen Fan, Songqing Chen, Ping Ding, Shuizi Liu, Guiqian Ouyang, Ruoyun BMC Pulm Med Case Report BACKGROUND: Atypical manifestations, such as elevated serum immunoglobulin-G4 (IgG4) and extra-pulmonary IgG4 positive plasmacyte infiltration, have been described in patients with eosinophilic granulomatosis with polyangiitis (EGPA), such complicated situation might not be readily differentiated from IgG4-related disease. CASE PRESENTATION: Here, we report an interesting and rare case of EGPA in a 41 year-old male with negative anti-neutrophil cytoplasmic antibodies (ANCAs), which showed abundant pulmonary IgG4-positive plasma cells infiltration and markedly elevated serum IgG4 levels without extra-pulmonary lesions of IgG4-related disease. The clinical characteristics hesitated us whether the diagnosis as EGPA overlapping with IgG4-related lung disease should be concluded because of the absence of storiform fibrosis and obliterative phlebitis with lymphoplasmacytic infiltration. The patient’s systemic symptoms, pulmonary lesions, blood eosinophils count / percentage, and serum IgG4 levels were significantly improved with immunosuppressive therapy. CONCLUSIONS: We consider that the overlapping pathogenesis exists in the disease course of EGPA and IgG4-related disease, which may lead to interaction during the course of the diseases. And the utilization of diagnostic criteria for IgG4-related lung disease with the careful differentiation is needed in such cases. BioMed Central 2019-08-19 /pmc/articles/PMC6701049/ /pubmed/31426769 http://dx.doi.org/10.1186/s12890-019-0917-4 Text en © The Author(s). 2019 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
Zhou, Li
Cao, Fen
Fan, Songqing
Chen, Ping
Ding, Shuizi
Liu, Guiqian
Ouyang, Ruoyun
A case of eosinophilic granulomatosis with polyangiitis complicated with a similar condition to IgG4 related lung disease
title A case of eosinophilic granulomatosis with polyangiitis complicated with a similar condition to IgG4 related lung disease
title_full A case of eosinophilic granulomatosis with polyangiitis complicated with a similar condition to IgG4 related lung disease
title_fullStr A case of eosinophilic granulomatosis with polyangiitis complicated with a similar condition to IgG4 related lung disease
title_full_unstemmed A case of eosinophilic granulomatosis with polyangiitis complicated with a similar condition to IgG4 related lung disease
title_short A case of eosinophilic granulomatosis with polyangiitis complicated with a similar condition to IgG4 related lung disease
title_sort case of eosinophilic granulomatosis with polyangiitis complicated with a similar condition to igg4 related lung disease
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6701049/
https://www.ncbi.nlm.nih.gov/pubmed/31426769
http://dx.doi.org/10.1186/s12890-019-0917-4
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