Clinically amyopathic dermatomyositis: clinical features, response to medications and malignancy‐associated risk factors in a specific tertiary‐care‐centre cohort
Identifieur interne : 000E22 ( Main/Exploration ); précédent : 000E21; suivant : 000E23Clinically amyopathic dermatomyositis: clinical features, response to medications and malignancy‐associated risk factors in a specific tertiary‐care‐centre cohort
Auteurs : F. Galimberti [États-Unis] ; Y. Li [États-Unis] ; A. P. Fernandez [États-Unis]Source :
- British Journal of Dermatology [ 0007-0963 ] ; 2016-01.
Abstract
Background: Clinically amyopathic dermatomyositis (CADM) is a subset of dermatomyositis (DM) characterized by the typical DM cutaneous manifestations but without myositis. There is a relative paucity of characterized cases of CADM in the peer‐reviewed medical literature. Objectives: To characterize the clinical features, response to medications and malignancy‐associated risk factors of patients with CADM with available baseline data seen at a single tertiary‐care centre. Methods: A retrospective review was undertaken of 44 patients with CADM with available clinical and serological data prior to onset of treatment. Results: Patients with CADM comprised 18% of all patients with DM with baseline data available at our institution. Although the majority of patients showed improvement with the first prescribed treatment, most required additional medications to control their CADM. Six of 44 patients had an associated malignancy. Photosensitivity and periungual erythema were found to be associated with absence of malignancy (P = 0·03 and P = 0·02, respectively). Patients with malignancy‐associated CADM were found to be more likely to have a cutaneous response with the first prescribed treatment than patients without malignancy (P = 0·04). Conclusions: CADM represents a significant subset of the DM population. As with classic DM, the cutaneous manifestations of CADM often represent a therapeutic challenge. A subset of patients with CADM has underlying malignancies, and these may differ from those typically associated with classic DM. Differences in serological abnormalities, cutaneous manifestations and response to first treatment among patients with CADM with and without malignancy were found, and suggest distinct pathophysiologies among CADM subsets. Characterization of this cohort expands the knowledge about this unique DM subset.
What's already known about this topic? Clinically amyopathic dermatomyositis (CADM) comprises a significant subset of patients with dermatomyositis (DM). Like classic DM, CADM presents with similar cutaneous manifestations and may be associated with lung disease or malignancy. What does this study add? Although patients with CADM may initially respond to conservative therapy, most eventually require more aggressive treatment. Malignancies and clinical features found in patients with malignancy‐associated (MA)‐CADM may differ from those in MA classic DM. Clinical, serological and treatment response differences between patients with idiopathic and MA‐CADM suggest distinct pathophysiologies.
Url:
DOI: 10.1111/bjd.14227
Affiliations:
Links toward previous steps (curation, corpus...)
- to stream Istex, to step Corpus: 000A75
- to stream Istex, to step Curation: 000A75
- to stream Istex, to step Checkpoint: 000041
- to stream Main, to step Merge: 000E23
- to stream Main, to step Curation: 000E22
Le document en format XML
<record><TEI wicri:istexFullTextTei="biblStruct"><teiHeader><fileDesc><titleStmt><title xml:lang="en">Clinically amyopathic dermatomyositis: clinical features, response to medications and malignancy‐associated risk factors in a specific tertiary‐care‐centre cohort</title><author><name sortKey="Galimberti, F" sort="Galimberti, F" uniqKey="Galimberti F" first="F." last="Galimberti">F. Galimberti</name></author><author><name sortKey="Li, Y" sort="Li, Y" uniqKey="Li Y" first="Y." last="Li">Y. Li</name></author><author><name sortKey="Fernandez, A P" sort="Fernandez, A P" uniqKey="Fernandez A" first="A. P." last="Fernandez">A. P. Fernandez</name></author></titleStmt><publicationStmt><idno type="wicri:source">ISTEX</idno><idno type="RBID">ISTEX:F59145BD86FA0D2D387E684876A83B82FD32A7A6</idno><date when="2016" year="2016">2016</date><idno type="doi">10.1111/bjd.14227</idno><idno type="url">https://api.istex.fr/ark:/67375/WNG-72QTZS9Z-3/fulltext.pdf</idno><idno type="wicri:Area/Istex/Corpus">000A75</idno><idno type="wicri:explorRef" wicri:stream="Istex" wicri:step="Corpus" wicri:corpus="ISTEX">000A75</idno><idno type="wicri:Area/Istex/Curation">000A75</idno><idno type="wicri:Area/Istex/Checkpoint">000041</idno><idno type="wicri:explorRef" wicri:stream="Istex" wicri:step="Checkpoint">000041</idno><idno type="wicri:doubleKey">0007-0963:2016:Galimberti F:clinically:amyopathic:dermatomyositis</idno><idno type="wicri:Area/Main/Merge">000E23</idno><idno type="wicri:Area/Main/Curation">000E22</idno><idno type="wicri:Area/Main/Exploration">000E22</idno></publicationStmt><sourceDesc><biblStruct><analytic><title level="a" type="main">Clinically amyopathic dermatomyositis: clinical features, response to medications and malignancy‐associated risk factors in a specific tertiary‐care‐centre cohort</title><author><name sortKey="Galimberti, F" sort="Galimberti, F" uniqKey="Galimberti F" first="F." last="Galimberti">F. Galimberti</name><affiliation wicri:level="1"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Cleveland Clinic Lerner College of Medicine, OH, Cleveland</wicri:regionArea><wicri:noRegion>Cleveland</wicri:noRegion></affiliation></author><author><name sortKey="Li, Y" sort="Li, Y" uniqKey="Li Y" first="Y." last="Li">Y. Li</name><affiliation wicri:level="1"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Cleveland Clinic Lerner College of Medicine, OH, Cleveland</wicri:regionArea><wicri:noRegion>Cleveland</wicri:noRegion></affiliation></author><author><name sortKey="Fernandez, A P" sort="Fernandez, A P" uniqKey="Fernandez A" first="A. P." last="Fernandez">A. P. Fernandez</name><affiliation wicri:level="1"><country xml:lang="fr">États-Unis</country><wicri:regionArea>Departments of Dermatology and Pathology, Cleveland Clinic, 9500 Euclid Avenue, OH, A61, Cleveland</wicri:regionArea><wicri:noRegion>Cleveland</wicri:noRegion></affiliation><affiliation></affiliation><affiliation></affiliation></author></analytic><monogr></monogr><series><title level="j" type="main">British Journal of Dermatology</title><title level="j" type="alt">BRITISH JOURNAL OF DERMATOLOGY</title><idno type="ISSN">0007-0963</idno><idno type="eISSN">1365-2133</idno><imprint><biblScope unit="vol">174</biblScope><biblScope unit="issue">1</biblScope><biblScope unit="page" from="158">158</biblScope><biblScope unit="page" to="164">164</biblScope><biblScope unit="page-count">7</biblScope><date type="published" when="2016-01">2016-01</date></imprint><idno type="ISSN">0007-0963</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0007-0963</idno></seriesStmt></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract">Background: Clinically amyopathic dermatomyositis (CADM) is a subset of dermatomyositis (DM) characterized by the typical DM cutaneous manifestations but without myositis. There is a relative paucity of characterized cases of CADM in the peer‐reviewed medical literature. Objectives: To characterize the clinical features, response to medications and malignancy‐associated risk factors of patients with CADM with available baseline data seen at a single tertiary‐care centre. Methods: A retrospective review was undertaken of 44 patients with CADM with available clinical and serological data prior to onset of treatment. Results: Patients with CADM comprised 18% of all patients with DM with baseline data available at our institution. Although the majority of patients showed improvement with the first prescribed treatment, most required additional medications to control their CADM. Six of 44 patients had an associated malignancy. Photosensitivity and periungual erythema were found to be associated with absence of malignancy (P = 0·03 and P = 0·02, respectively). Patients with malignancy‐associated CADM were found to be more likely to have a cutaneous response with the first prescribed treatment than patients without malignancy (P = 0·04). Conclusions: CADM represents a significant subset of the DM population. As with classic DM, the cutaneous manifestations of CADM often represent a therapeutic challenge. A subset of patients with CADM has underlying malignancies, and these may differ from those typically associated with classic DM. Differences in serological abnormalities, cutaneous manifestations and response to first treatment among patients with CADM with and without malignancy were found, and suggest distinct pathophysiologies among CADM subsets. Characterization of this cohort expands the knowledge about this unique DM subset.</div><div type="abstract" xml:lang="en">What's already known about this topic? Clinically amyopathic dermatomyositis (CADM) comprises a significant subset of patients with dermatomyositis (DM). Like classic DM, CADM presents with similar cutaneous manifestations and may be associated with lung disease or malignancy. What does this study add? Although patients with CADM may initially respond to conservative therapy, most eventually require more aggressive treatment. Malignancies and clinical features found in patients with malignancy‐associated (MA)‐CADM may differ from those in MA classic DM. Clinical, serological and treatment response differences between patients with idiopathic and MA‐CADM suggest distinct pathophysiologies.</div></front></TEI><affiliations><list><country><li>États-Unis</li></country></list><tree><country name="États-Unis"><noRegion><name sortKey="Galimberti, F" sort="Galimberti, F" uniqKey="Galimberti F" first="F." last="Galimberti">F. Galimberti</name></noRegion><name sortKey="Fernandez, A P" sort="Fernandez, A P" uniqKey="Fernandez A" first="A. P." last="Fernandez">A. P. Fernandez</name><name sortKey="Li, Y" sort="Li, Y" uniqKey="Li Y" first="Y." last="Li">Y. Li</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
EXPLOR_STEP=$WICRI_ROOT/Sante/explor/ChloroquineV1/Data/Main/Exploration
HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 000E22 | SxmlIndent | more
Ou
HfdSelect -h $EXPLOR_AREA/Data/Main/Exploration/biblio.hfd -nk 000E22 | SxmlIndent | more
Pour mettre un lien sur cette page dans le réseau Wicri
{{Explor lien
|wiki= Sante
|area= ChloroquineV1
|flux= Main
|étape= Exploration
|type= RBID
|clé= ISTEX:F59145BD86FA0D2D387E684876A83B82FD32A7A6
|texte= Clinically amyopathic dermatomyositis: clinical features, response to medications and malignancy‐associated risk factors in a specific tertiary‐care‐centre cohort
}}
| This area was generated with Dilib version V0.6.33. |  |