A Rare Cause of Diffuse Parenchymal Lung Disease together with Granulomatous Reaction: Pulmonary Amyloidosis

Amyloidosis is a heterogeneous group of disorder associated with the deposition of protein in an abnormal fibrillar form. Primary Sjögren's syndrome (PSS) is a systemic inflammatory disorder that commonly affects the exocrine glands. The reported frequency of pulmonary involvement in PSS varies...

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Autores principales: Ozer Simsek, Zuhal, Oymak, Fatma Sema, Tutar, Nuri, Canoz, Ozlem, Demir, Ramazan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2013
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3600268/
https://www.ncbi.nlm.nih.gov/pubmed/23533903
http://dx.doi.org/10.1155/2013/837190
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author Ozer Simsek, Zuhal
Oymak, Fatma Sema
Tutar, Nuri
Canoz, Ozlem
Demir, Ramazan
author_facet Ozer Simsek, Zuhal
Oymak, Fatma Sema
Tutar, Nuri
Canoz, Ozlem
Demir, Ramazan
author_sort Ozer Simsek, Zuhal
collection PubMed
description Amyloidosis is a heterogeneous group of disorder associated with the deposition of protein in an abnormal fibrillar form. Primary Sjögren's syndrome (PSS) is a systemic inflammatory disorder that commonly affects the exocrine glands. The reported frequency of pulmonary involvement in PSS varies widely, ranging from 9% to 75%. Pulmonary involvement occurs in light-chain (AL) amyloidosis and is uncommon in the reactive (AA) and hereditary forms. Herein we present a case of PSS associated diffuse multinodular amyloidosis in the lung. We followed up the patient without treatment for three years. There are only minimal lung symptoms related to lung infiltration. In conclusion, pulmonary involvement in SS is an extremely rare clinical manifestation and usually has a good survival rate without treatment.
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spelling pubmed-36002682013-03-26 A Rare Cause of Diffuse Parenchymal Lung Disease together with Granulomatous Reaction: Pulmonary Amyloidosis Ozer Simsek, Zuhal Oymak, Fatma Sema Tutar, Nuri Canoz, Ozlem Demir, Ramazan Case Rep Pulmonol Case Report Amyloidosis is a heterogeneous group of disorder associated with the deposition of protein in an abnormal fibrillar form. Primary Sjögren's syndrome (PSS) is a systemic inflammatory disorder that commonly affects the exocrine glands. The reported frequency of pulmonary involvement in PSS varies widely, ranging from 9% to 75%. Pulmonary involvement occurs in light-chain (AL) amyloidosis and is uncommon in the reactive (AA) and hereditary forms. Herein we present a case of PSS associated diffuse multinodular amyloidosis in the lung. We followed up the patient without treatment for three years. There are only minimal lung symptoms related to lung infiltration. In conclusion, pulmonary involvement in SS is an extremely rare clinical manifestation and usually has a good survival rate without treatment. Hindawi Publishing Corporation 2013 2013-02-28 /pmc/articles/PMC3600268/ /pubmed/23533903 http://dx.doi.org/10.1155/2013/837190 Text en Copyright © 2013 Zuhal Ozer Simsek et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Ozer Simsek, Zuhal
Oymak, Fatma Sema
Tutar, Nuri
Canoz, Ozlem
Demir, Ramazan
A Rare Cause of Diffuse Parenchymal Lung Disease together with Granulomatous Reaction: Pulmonary Amyloidosis
title A Rare Cause of Diffuse Parenchymal Lung Disease together with Granulomatous Reaction: Pulmonary Amyloidosis
title_full A Rare Cause of Diffuse Parenchymal Lung Disease together with Granulomatous Reaction: Pulmonary Amyloidosis
title_fullStr A Rare Cause of Diffuse Parenchymal Lung Disease together with Granulomatous Reaction: Pulmonary Amyloidosis
title_full_unstemmed A Rare Cause of Diffuse Parenchymal Lung Disease together with Granulomatous Reaction: Pulmonary Amyloidosis
title_short A Rare Cause of Diffuse Parenchymal Lung Disease together with Granulomatous Reaction: Pulmonary Amyloidosis
title_sort rare cause of diffuse parenchymal lung disease together with granulomatous reaction: pulmonary amyloidosis
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3600268/
https://www.ncbi.nlm.nih.gov/pubmed/23533903
http://dx.doi.org/10.1155/2013/837190
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