Pulmonary amyloidosis in Sjogren's syndrome: a case report and systematic review of the literature.
Identifieur interne : 001619 ( Main/Exploration ); précédent : 001618; suivant : 001620Pulmonary 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
Affiliations:
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Le document en format XML
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K" first="Kirti" last="Gupta">Kirti Gupta</name></author><author><name sortKey="Gupta, Dheeraj" sort="Gupta, Dheeraj" uniqKey="Gupta D" first="Dheeraj" last="Gupta">Dheeraj Gupta</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2010">2010</date><idno type="RBID">pubmed:20546191</idno><idno type="pmid">20546191</idno><idno type="doi">10.1111/j.1440-1843.2010.01772.x</idno><idno type="wicri:Area/PubMed/Corpus">000397</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">000397</idno><idno type="wicri:Area/PubMed/Curation">000397</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Curation">000397</idno><idno type="wicri:Area/PubMed/Checkpoint">000382</idno><idno type="wicri:explorRef" wicri:stream="Checkpoint" wicri:step="PubMed">000382</idno><idno type="wicri:Area/Ncbi/Merge">000136</idno><idno type="wicri:Area/Ncbi/Curation">000136</idno><idno 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Pulmonary (drug therapy)</term></keywords><keywords scheme="KwdFr" xml:lang="fr"><term>Adulte</term><term>Amyloïdose (imagerie diagnostique)</term><term>Amyloïdose (traitement médicamenteux)</term><term>Antibactériens (usage thérapeutique)</term><term>Antituberculeux (usage thérapeutique)</term><term>Biopsie</term><term>Femelle</term><term>Hormones corticosurrénaliennes (usage thérapeutique)</term><term>Humains</term><term>Hydroxychloroquine (usage thérapeutique)</term><term>Hypoxie (sang)</term><term>Infections à Escherichia coli (diagnostic)</term><term>Infections à Escherichia coli (traitement médicamenteux)</term><term>Infections à Klebsiella (diagnostic)</term><term>Infections à Klebsiella (traitement médicamenteux)</term><term>Klebsiella pneumoniae ()</term><term>Klebsiella pneumoniae (isolement et purification)</term><term>Maladies pulmonaires (imagerie diagnostique)</term><term>Maladies pulmonaires (physiopathologie)</term><term>Maladies pulmonaires (traitement médicamenteux)</term><term>Mycobacterium tuberculosis ()</term><term>Mycobacterium tuberculosis (isolement et purification)</term><term>Méthylprednisolone (usage thérapeutique)</term><term>Radiographie</term><term>Syndrome de Gougerot-Sjögren (imagerie diagnostique)</term><term>Syndrome de Gougerot-Sjögren (traitement médicamenteux)</term><term>Tuberculose pulmonaire (diagnostic)</term><term>Tuberculose pulmonaire (traitement médicamenteux)</term></keywords><keywords scheme="MESH" type="chemical" qualifier="therapeutic use" xml:lang="en"><term>Adrenal Cortex Hormones</term><term>Anti-Bacterial Agents</term><term>Antitubercular Agents</term><term>Hydroxychloroquine</term><term>Methylprednisolone</term></keywords><keywords scheme="MESH" qualifier="blood" xml:lang="en"><term>Hypoxia</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Escherichia coli Infections</term><term>Klebsiella Infections</term><term>Tuberculosis, Pulmonary</term></keywords><keywords 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xml:lang="en"><term>Klebsiella pneumoniae</term><term>Mycobacterium tuberculosis</term></keywords><keywords scheme="MESH" qualifier="isolement et purification" xml:lang="fr"><term>Klebsiella pneumoniae</term><term>Mycobacterium tuberculosis</term></keywords><keywords scheme="MESH" qualifier="physiopathologie" xml:lang="fr"><term>Maladies 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><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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