Pulmonary amyloidosis in Sjogren's syndrome: a case report and systematic review of the literature.
Identifieur interne : 000136 ( Ncbi/Merge ); précédent : 000135; suivant : 000137Pulmonary amyloidosis in Sjogren's syndrome: a case report and systematic review of the literature.
Auteurs : Srinivas Rajagopala [Inde] ; Navneet Singh ; Kirti Gupta ; Dheeraj GuptaSource :
- Respirology (Carlton, Vic.) [ 1440-1843 ] ; 2010.
Descripteurs français
- KwdFr :
- Adulte, Amyloïdose (imagerie diagnostique), Amyloïdose (traitement médicamenteux), Antibactériens (usage thérapeutique), Antituberculeux (usage thérapeutique), Biopsie, Femelle, Hormones corticosurrénaliennes (usage thérapeutique), Humains, Hydroxychloroquine (usage thérapeutique), Hypoxie (sang), Infections à Escherichia coli (diagnostic), Infections à Escherichia coli (traitement médicamenteux), Infections à Klebsiella (diagnostic), Infections à Klebsiella (traitement médicamenteux), Klebsiella pneumoniae (), Klebsiella pneumoniae (isolement et purification), Maladies pulmonaires (imagerie diagnostique), Maladies pulmonaires (physiopathologie), Maladies pulmonaires (traitement médicamenteux), Mycobacterium tuberculosis (), Mycobacterium tuberculosis (isolement et purification), Méthylprednisolone (usage thérapeutique), Radiographie, Syndrome de Gougerot-Sjögren (imagerie diagnostique), Syndrome de Gougerot-Sjögren (traitement médicamenteux), Tuberculose pulmonaire (diagnostic), Tuberculose pulmonaire (traitement médicamenteux).
- MESH :
- diagnostic : Infections à Escherichia coli, Infections à Klebsiella, Tuberculose pulmonaire.
- imagerie diagnostique : Amyloïdose, Maladies pulmonaires, Syndrome de Gougerot-Sjögren.
- isolement et purification : Klebsiella pneumoniae, Mycobacterium tuberculosis.
- physiopathologie : Maladies pulmonaires.
- sang : Hypoxie.
- traitement médicamenteux : Amyloïdose, Infections à Escherichia coli, Infections à Klebsiella, Maladies pulmonaires, Syndrome de Gougerot-Sjögren, Tuberculose pulmonaire.
- usage thérapeutique : Antibactériens, Antituberculeux, Hormones corticosurrénaliennes, Hydroxychloroquine, Méthylprednisolone.
- Adulte, Biopsie, Femelle, Humains, Klebsiella pneumoniae, Mycobacterium tuberculosis, Radiographie.
English descriptors
- KwdEn :
- Adrenal Cortex Hormones (therapeutic use), Adult, Amyloidosis (diagnostic imaging), Amyloidosis (drug therapy), Anti-Bacterial Agents (therapeutic use), Antitubercular Agents (therapeutic use), Biopsy, Escherichia coli Infections (diagnosis), Escherichia coli Infections (drug therapy), Female, Humans, Hydroxychloroquine (therapeutic use), Hypoxia (blood), Klebsiella Infections (diagnosis), Klebsiella Infections (drug therapy), Klebsiella pneumoniae (drug effects), Klebsiella pneumoniae (isolation & purification), Lung Diseases (diagnostic imaging), Lung Diseases (drug therapy), Lung Diseases (physiopathology), Methylprednisolone (therapeutic use), Mycobacterium tuberculosis (drug effects), Mycobacterium tuberculosis (isolation & purification), Radiography, Sjogren's Syndrome (diagnostic imaging), Sjogren's Syndrome (drug therapy), Tuberculosis, Pulmonary (diagnosis), Tuberculosis, Pulmonary (drug therapy).
- MESH :
- chemical , therapeutic use : Adrenal Cortex Hormones, Anti-Bacterial Agents, Antitubercular Agents, Hydroxychloroquine, Methylprednisolone.
- blood : Hypoxia.
- diagnosis : Escherichia coli Infections, Klebsiella Infections, Tuberculosis, Pulmonary.
- diagnostic imaging : Amyloidosis, Lung Diseases, Sjogren's Syndrome.
- drug effects : Klebsiella pneumoniae, Mycobacterium tuberculosis.
- drug therapy : Amyloidosis, Escherichia coli Infections, Klebsiella Infections, Lung Diseases, Sjogren's Syndrome, Tuberculosis, Pulmonary.
- isolation & purification : Klebsiella pneumoniae, Mycobacterium tuberculosis.
- physiopathology : Lung Diseases.
- Adult, Biopsy, Female, Humans, Radiography.
Abstract
Amyloidosis is a heterogeneous group of disorders characterized by misfolding of extracellular proteins. The two most common forms are light-chain (AL) and reactive (AA) amyloidosis. Pulmonary amyloidosis secondary to Sjogren's syndrome (SS) is rare, and there is a paucity of data on its clinical and radiological features. We describe the case of a 41-year-old woman with diffuse septal pulmonary AL (lambda) amyloidosis related to SS, in the absence of systemic amyloidosis. A systematic search for pulmonary amyloidosis in SS yielded 37 cases. Amyloidosis occurred almost exclusively in women (96.5%). Cough and dyspnoea (56%) were the most common symptoms. The diagnosis of pulmonary amyloidosis was made subsequent to that of SS, with a median delay of 7 years. Radiologically, diffuse nodules with (45.5%) or without (33.3%) multiple cysts were the most common pattern. Most cases were related to nodular AL amyloidosis (both kappa and lambda). Diffuse septal AA amyloidosis has been reported in SS. The index patient is the first documented case of diffuse septal pulmonary AL amyloidosis in SS, without systemic amyloidosis. Surgical lung biopsy (78%) is usually required to establish the diagnosis and rule out lymphoma. There is no data to support any definitive therapeutic intervention for SS-related pulmonary amyloidosis. Observation is sufficient for nodular amyloidosis. Further studies are necessary to assess the efficacy of current therapies in diffuse septal amyloidosis secondary to SS.
DOI: 10.1111/j.1440-1843.2010.01772.x
PubMed: 20546191
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pubmed:20546191Le document en format XML
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amyloidosis in Sjogren's syndrome: a case report and systematic review of the literature.</title><author><name sortKey="Rajagopala, Srinivas" sort="Rajagopala, Srinivas" uniqKey="Rajagopala S" first="Srinivas" last="Rajagopala">Srinivas Rajagopala</name><affiliation wicri:level="1"><nlm:affiliation>Department of Pulmonary Medicine, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. visitsrinivasan@gmail.com</nlm:affiliation><country xml:lang="fr">Inde</country><wicri:regionArea>Department of Pulmonary Medicine, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh</wicri:regionArea><wicri:noRegion>Chandigarh</wicri:noRegion></affiliation></author><author><name sortKey="Singh, Navneet" sort="Singh, Navneet" uniqKey="Singh N" first="Navneet" last="Singh">Navneet Singh</name></author><author><name sortKey="Gupta, Kirti" sort="Gupta, Kirti" uniqKey="Gupta K" first="Kirti" last="Gupta">Kirti Gupta</name></author><author><name 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pulmonaires</term></keywords><keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Lung Diseases</term></keywords><keywords scheme="MESH" qualifier="sang" xml:lang="fr"><term>Hypoxie</term></keywords><keywords scheme="MESH" qualifier="traitement médicamenteux" xml:lang="fr"><term>Amyloïdose</term><term>Infections à Escherichia coli</term><term>Infections à Klebsiella</term><term>Maladies pulmonaires</term><term>Syndrome de Gougerot-Sjögren</term><term>Tuberculose pulmonaire</term></keywords><keywords scheme="MESH" qualifier="usage thérapeutique" xml:lang="fr"><term>Antibactériens</term><term>Antituberculeux</term><term>Hormones corticosurrénaliennes</term><term>Hydroxychloroquine</term><term>Méthylprednisolone</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Biopsy</term><term>Female</term><term>Humans</term><term>Radiography</term></keywords><keywords scheme="MESH" xml:lang="fr"><term>Adulte</term><term>Biopsie</term><term>Femelle</term><term>Humains</term><term>Klebsiella pneumoniae</term><term>Mycobacterium tuberculosis</term><term>Radiographie</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Amyloidosis is a heterogeneous group of disorders characterized by misfolding of extracellular proteins. The two most common forms are light-chain (AL) and reactive (AA) amyloidosis. Pulmonary amyloidosis secondary to Sjogren's syndrome (SS) is rare, and there is a paucity of data on its clinical and radiological features. We describe the case of a 41-year-old woman with diffuse septal pulmonary AL (lambda) amyloidosis related to SS, in the absence of systemic amyloidosis. A systematic search for pulmonary amyloidosis in SS yielded 37 cases. Amyloidosis occurred almost exclusively in women (96.5%). Cough and dyspnoea (56%) were the most common symptoms. The diagnosis of pulmonary amyloidosis was made subsequent to that of SS, with a median delay of 7 years. Radiologically, diffuse nodules with (45.5%) or without (33.3%) multiple cysts were the most common pattern. Most cases were related to nodular AL amyloidosis (both kappa and lambda). Diffuse septal AA amyloidosis has been reported in SS. The index patient is the first documented case of diffuse septal pulmonary AL amyloidosis in SS, without systemic amyloidosis. Surgical lung biopsy (78%) is usually required to establish the diagnosis and rule out lymphoma. There is no data to support any definitive therapeutic intervention for SS-related pulmonary amyloidosis. Observation is sufficient for nodular amyloidosis. Further studies are necessary to assess the efficacy of current therapies in diffuse septal amyloidosis secondary to SS.</div></front></TEI><pubmed><MedlineCitation Status="MEDLINE" Owner="NLM"><PMID Version="1">20546191</PMID><DateCompleted><Year>2010</Year><Month>11</Month><Day>22</Day></DateCompleted><DateRevised><Year>2018</Year><Month>12</Month><Day>01</Day></DateRevised><Article PubModel="Print-Electronic"><Journal><ISSN IssnType="Electronic">1440-1843</ISSN><JournalIssue CitedMedium="Internet"><Volume>15</Volume><Issue>5</Issue><PubDate><Year>2010</Year><Month>Jul</Month></PubDate></JournalIssue><Title>Respirology (Carlton, Vic.)</Title><ISOAbbreviation>Respirology</ISOAbbreviation></Journal><ArticleTitle>Pulmonary amyloidosis in Sjogren's syndrome: a case report and systematic review of the literature.</ArticleTitle><Pagination><MedlinePgn>860-6</MedlinePgn></Pagination><ELocationID EIdType="doi" ValidYN="Y">10.1111/j.1440-1843.2010.01772.x</ELocationID><Abstract><AbstractText>Amyloidosis is a heterogeneous group of disorders characterized by misfolding of extracellular proteins. The two most common forms are light-chain (AL) and reactive (AA) amyloidosis. Pulmonary amyloidosis secondary to Sjogren's syndrome (SS) is rare, and there is a paucity of data on its clinical and radiological features. We describe the case of a 41-year-old woman with diffuse septal pulmonary AL (lambda) amyloidosis related to SS, in the absence of systemic amyloidosis. A systematic search for pulmonary amyloidosis in SS yielded 37 cases. Amyloidosis occurred almost exclusively in women (96.5%). Cough and dyspnoea (56%) were the most common symptoms. The diagnosis of pulmonary amyloidosis was made subsequent to that of SS, with a median delay of 7 years. Radiologically, diffuse nodules with (45.5%) or without (33.3%) multiple cysts were the most common pattern. Most cases were related to nodular AL amyloidosis (both kappa and lambda). Diffuse septal AA amyloidosis has been reported in SS. The index patient is the first documented case of diffuse septal pulmonary AL amyloidosis in SS, without systemic amyloidosis. Surgical lung biopsy (78%) is usually required to establish the diagnosis and rule out lymphoma. There is no data to support any definitive therapeutic intervention for SS-related pulmonary amyloidosis. Observation is sufficient for nodular amyloidosis. Further studies are necessary to assess the efficacy of current therapies in diffuse septal amyloidosis secondary to SS.</AbstractText></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Rajagopala</LastName><ForeName>Srinivas</ForeName><Initials>S</Initials><AffiliationInfo><Affiliation>Department of Pulmonary Medicine, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. visitsrinivasan@gmail.com</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Singh</LastName><ForeName>Navneet</ForeName><Initials>N</Initials></Author><Author ValidYN="Y"><LastName>Gupta</LastName><ForeName>Kirti</ForeName><Initials>K</Initials></Author><Author ValidYN="Y"><LastName>Gupta</LastName><ForeName>Dheeraj</ForeName><Initials>D</Initials></Author></AuthorList><Language>eng</Language><PublicationTypeList><PublicationType UI="D002363">Case Reports</PublicationType><PublicationType UI="D016428">Journal Article</PublicationType><PublicationType UI="D016454">Review</PublicationType><PublicationType UI="D000078182">Systematic Review</PublicationType></PublicationTypeList><ArticleDate DateType="Electronic"><Year>2010</Year><Month>06</Month><Day>04</Day></ArticleDate></Article><MedlineJournalInfo><Country>Australia</Country><MedlineTA>Respirology</MedlineTA><NlmUniqueID>9616368</NlmUniqueID><ISSNLinking>1323-7799</ISSNLinking></MedlineJournalInfo><ChemicalList><Chemical><RegistryNumber>0</RegistryNumber><NameOfSubstance UI="D000305">Adrenal Cortex Hormones</NameOfSubstance></Chemical><Chemical><RegistryNumber>0</RegistryNumber><NameOfSubstance UI="D000900">Anti-Bacterial Agents</NameOfSubstance></Chemical><Chemical><RegistryNumber>0</RegistryNumber><NameOfSubstance UI="D000995">Antitubercular Agents</NameOfSubstance></Chemical><Chemical><RegistryNumber>4QWG6N8QKH</RegistryNumber><NameOfSubstance UI="D006886">Hydroxychloroquine</NameOfSubstance></Chemical><Chemical><RegistryNumber>X4W7ZR7023</RegistryNumber><NameOfSubstance UI="D008775">Methylprednisolone</NameOfSubstance></Chemical></ChemicalList><CitationSubset>IM</CitationSubset><MeshHeadingList><MeshHeading><DescriptorName UI="D000305" MajorTopicYN="N">Adrenal Cortex Hormones</DescriptorName><QualifierName UI="Q000627" MajorTopicYN="N">therapeutic use</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D000328" MajorTopicYN="N">Adult</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D000686" MajorTopicYN="N">Amyloidosis</DescriptorName><QualifierName UI="Q000000981" MajorTopicYN="Y">diagnostic imaging</QualifierName><QualifierName UI="Q000188" MajorTopicYN="N">drug therapy</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D000900" MajorTopicYN="N">Anti-Bacterial Agents</DescriptorName><QualifierName UI="Q000627" MajorTopicYN="N">therapeutic use</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D000995" MajorTopicYN="N">Antitubercular Agents</DescriptorName><QualifierName UI="Q000627" MajorTopicYN="N">therapeutic use</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D001706" MajorTopicYN="N">Biopsy</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D004927" MajorTopicYN="N">Escherichia coli Infections</DescriptorName><QualifierName UI="Q000175" MajorTopicYN="N">diagnosis</QualifierName><QualifierName UI="Q000188" MajorTopicYN="N">drug therapy</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D005260" MajorTopicYN="N">Female</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D006801" MajorTopicYN="N">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D006886" MajorTopicYN="N">Hydroxychloroquine</DescriptorName><QualifierName UI="Q000627" MajorTopicYN="N">therapeutic use</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D000860" MajorTopicYN="N">Hypoxia</DescriptorName><QualifierName UI="Q000097" MajorTopicYN="N">blood</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D007710" MajorTopicYN="N">Klebsiella Infections</DescriptorName><QualifierName UI="Q000175" MajorTopicYN="N">diagnosis</QualifierName><QualifierName UI="Q000188" MajorTopicYN="N">drug therapy</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D007711" MajorTopicYN="N">Klebsiella pneumoniae</DescriptorName><QualifierName UI="Q000187" MajorTopicYN="N">drug effects</QualifierName><QualifierName UI="Q000302" MajorTopicYN="N">isolation & purification</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D008171" MajorTopicYN="N">Lung Diseases</DescriptorName><QualifierName UI="Q000000981" MajorTopicYN="Y">diagnostic imaging</QualifierName><QualifierName UI="Q000188" MajorTopicYN="N">drug therapy</QualifierName><QualifierName UI="Q000503" MajorTopicYN="N">physiopathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D008775" MajorTopicYN="N">Methylprednisolone</DescriptorName><QualifierName UI="Q000627" MajorTopicYN="N">therapeutic use</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D009169" MajorTopicYN="N">Mycobacterium tuberculosis</DescriptorName><QualifierName UI="Q000187" MajorTopicYN="N">drug effects</QualifierName><QualifierName UI="Q000302" MajorTopicYN="N">isolation & purification</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D011859" MajorTopicYN="N">Radiography</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D012859" MajorTopicYN="N">Sjogren's Syndrome</DescriptorName><QualifierName UI="Q000000981" MajorTopicYN="Y">diagnostic imaging</QualifierName><QualifierName UI="Q000188" MajorTopicYN="N">drug therapy</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D014397" MajorTopicYN="N">Tuberculosis, Pulmonary</DescriptorName><QualifierName UI="Q000175" MajorTopicYN="N">diagnosis</QualifierName><QualifierName UI="Q000188" MajorTopicYN="N">drug therapy</QualifierName></MeshHeading></MeshHeadingList></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="entrez"><Year>2010</Year><Month>6</Month><Day>16</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="pubmed"><Year>2010</Year><Month>6</Month><Day>16</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>2010</Year><Month>12</Month><Day>14</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="pubmed">20546191</ArticleId><ArticleId IdType="pii">RES1772</ArticleId><ArticleId IdType="doi">10.1111/j.1440-1843.2010.01772.x</ArticleId></ArticleIdList></PubmedData></pubmed><affiliations><list><country><li>Inde</li></country></list><tree><noCountry><name sortKey="Gupta, Dheeraj" sort="Gupta, Dheeraj" uniqKey="Gupta D" first="Dheeraj" last="Gupta">Dheeraj Gupta</name><name sortKey="Gupta, Kirti" sort="Gupta, Kirti" uniqKey="Gupta K" first="Kirti" last="Gupta">Kirti Gupta</name><name sortKey="Singh, Navneet" sort="Singh, Navneet" uniqKey="Singh N" first="Navneet" last="Singh">Navneet Singh</name></noCountry><country name="Inde"><noRegion><name sortKey="Rajagopala, Srinivas" sort="Rajagopala, Srinivas" uniqKey="Rajagopala S" first="Srinivas" last="Rajagopala">Srinivas Rajagopala</name></noRegion></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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