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Antiphospholipid antibodies-associated diffuse alveolar hemorrhage.

Identifieur interne : 000269 ( Ncbi/Merge ); précédent : 000268; suivant : 000270

Antiphospholipid antibodies-associated diffuse alveolar hemorrhage.

Auteurs : Ralph Yachoui [États-Unis] ; Rahul Sehgal [États-Unis] ; Barkha Amlani [États-Unis] ; Jerry W. Goldberg [États-Unis]

Source :

RBID : pubmed:25481816

Descripteurs français

English descriptors

Abstract

To describe the clinical features and outcomes of 17 patients with primary antiphospholipid syndrome (PAPS) or antiphospholipid antibodies (aPL) and diffuse alveolar hemorrhage (DAH).

DOI: 10.1016/j.semarthrit.2014.10.013
PubMed: 25481816

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pubmed:25481816

Le document en format XML

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<div type="abstract" xml:lang="en">To describe the clinical features and outcomes of 17 patients with primary antiphospholipid syndrome (PAPS) or antiphospholipid antibodies (aPL) and diffuse alveolar hemorrhage (DAH).</div>
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<Title>Seminars in arthritis and rheumatism</Title>
<ISOAbbreviation>Semin. Arthritis Rheum.</ISOAbbreviation>
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<ArticleTitle>Antiphospholipid antibodies-associated diffuse alveolar hemorrhage.</ArticleTitle>
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<AbstractText Label="OBJECTIVES" NlmCategory="OBJECTIVE">To describe the clinical features and outcomes of 17 patients with primary antiphospholipid syndrome (PAPS) or antiphospholipid antibodies (aPL) and diffuse alveolar hemorrhage (DAH).</AbstractText>
<AbstractText Label="METHODS" NlmCategory="METHODS">We reviewed the medical records of all patients diagnosed with PAPS-associated DAH and aPL-associated DAH between January 1, 1997, and December 31, 2013, for clinical features, laboratory and radiographic findings, management, and outcomes.</AbstractText>
<AbstractText Label="RESULTS" NlmCategory="RESULTS">A total of 17 patients met the criteria for DAH and had aPL and 10 patients met the criteria for PAPS. The mean age at DAH diagnosis was 57.6 years. Secondary causes of DAH were ruled out. Surgical lung biopsy was performed in 6 cases, 5 of whom had bland hemorrhage. Pulmonary capillaritis was present in only 1 case. Four patients (3 with aPLs and 1 with PAPS) achieved complete remission despite receiving no treatment. The majority of patients treated received initial corticosteroids. Additionally, cyclophosphamide (2 cases), rituximab (1 case), plasma exchange (2 cases), methotrexate (1 case), azathioprine (1 case), and hydroxychloroquine (2 cases) were used. In total, 10 patients (59%) achieved complete and sustained remission with a median length of follow-up of 48 months. Four patients (23%) died (2 with PAPS and 2 with aPLs), all from uncontrolled DAH. Three patients (18%) relapsed after achieving complete remission.</AbstractText>
<AbstractText Label="CONCLUSIONS" NlmCategory="CONCLUSIONS">DAH is a rare complication of PAPS that can also arise de novo in aPL-positive individuals. Lung pathology shows either bland hemorrhage or capillaritis. Recognition of this unusual but known complication is important, since early diagnosis and therapy could potentially affect outcomes.</AbstractText>
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</ArticleIdList>
</PubmedData>
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<list>
<country>
<li>États-Unis</li>
</country>
<region>
<li>Wisconsin</li>
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<tree>
<country name="États-Unis">
<region name="Wisconsin">
<name sortKey="Yachoui, Ralph" sort="Yachoui, Ralph" uniqKey="Yachoui R" first="Ralph" last="Yachoui">Ralph Yachoui</name>
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<name sortKey="Amlani, Barkha" sort="Amlani, Barkha" uniqKey="Amlani B" first="Barkha" last="Amlani">Barkha Amlani</name>
<name sortKey="Goldberg, Jerry W" sort="Goldberg, Jerry W" uniqKey="Goldberg J" first="Jerry W" last="Goldberg">Jerry W. Goldberg</name>
<name sortKey="Sehgal, Rahul" sort="Sehgal, Rahul" uniqKey="Sehgal R" first="Rahul" last="Sehgal">Rahul Sehgal</name>
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