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[Au] Autor:Yan X; Jin J
[Ad] Endereço:Department of Hepatology, The First Hospital of Jilin University, Changchun, Jilin, China.
[Ti] Título:Primary cutaneous amyloidosis associated with autoimmune hepatitis-primary biliary cirrhosis overlap syndrome and Sjögren syndrome: A case report.
[So] Source:Medicine (Baltimore);97(8):e0004, 2018 Feb.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:RATIONALE: Primary cutaneous amyloidosis (PCA) is a localized skin disorder characterized by the abnormal deposition of amyloid in the extracellular matrix of the dermis. The association between PCA and other diseases, although rare, has been documented for various autoimmune diseases. PCA associated with autoimmune hepatitis-primary biliary cirrhosis (AIH-PBC) overlap syndrome and Sjögren syndrome (SS) has not been previously reported in the literature. PATIENT CONCERNS: A 50-year-old woman presented with progressive abnormal liver enzyme levels and was referred to our department. DIAGNOSES: Due to the patient's symptoms, laboratory test results, radiographic findings, and pathologic results, she was diagnosed with PCA associated with AIH-PBC overlap syndrome and SS. INTERVENTIONS: She was subsequently treated with a combination of ursodeoxycholic acid (UDCA), prednisone, and azathioprine. OUTCOMES: While this treatment can achieve therapeutic success, it cannot prevent complications from cirrhosis. This patient remains alive but experienced an emergent gastrointestinal hemorrhage. LESSONS: While we acknowledge that this is a single case, these findings extend our knowledge of immunological diseases associated with PCA and suggest a common, immune-mediated pathogenic pathway between PCA, AIH-PBC overlap syndrome, and SS. After 12 years of follow up, clinical manifestations have developed, and these autoimmune diseases have progressed. The combination of UDCA, prednisone, and azathioprine can achieve therapeutic success but cannot prevent disease progression. Routine follow up for this patient is necessary to document disease progression.
[Mh] Termos MeSH primário: Amiloidose Familiar/imunologia
Hepatite Autoimune/complicações
Cirrose Hepática Biliar/complicações
Síndrome de Sjogren/complicações
Dermatopatias Genéticas/imunologia
Doenças do Tecido Conjuntivo Indiferenciado/complicações
[Mh] Termos MeSH secundário: Amiloidose Familiar/tratamento farmacológico
Anti-Inflamatórios/administração & dosagem
Azatioprina/administração & dosagem
Colagogos e Coleréticos/administração & dosagem
Quimioterapia Combinada
Hepatite Autoimune/tratamento farmacológico
Hepatite Autoimune/imunologia
Seres Humanos
Imunossupressores/administração & dosagem
Cirrose Hepática Biliar/tratamento farmacológico
Cirrose Hepática Biliar/imunologia
Prednisona/administração & dosagem
Síndrome de Sjogren/tratamento farmacológico
Síndrome de Sjogren/imunologia
Dermatopatias Genéticas/tratamento farmacológico
Resultado do Tratamento
Doenças do Tecido Conjuntivo Indiferenciado/tratamento farmacológico
Doenças do Tecido Conjuntivo Indiferenciado/imunologia
Ácido Ursodesoxicólico/administração & dosagem
[Nm] Nome de substância:
0 (Anti-Inflammatory Agents); 0 (Cholagogues and Choleretics); 0 (Immunosuppressive Agents); 724L30Y2QR (Ursodeoxycholic Acid); MRK240IY2L (Azathioprine); VB0R961HZT (Prednisone)
[Em] Mês de entrada:1803
[Cu] Atualização por classe:180302
[Lr] Data última revisão:
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:180222
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000010004

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[Au] Autor:Class-Vázquez W; Olivero-Rivera S; Rosa-Cruz S; Laboy-Olivieri C; Toro DH
[Ti] Título:The Perils of Diagnosing and Treating Overlap Syndrome.
[So] Source:Bol Asoc Med P R;108(2):57-9, 2016.
[Is] ISSN:0004-4849
[Cp] País de publicação:Puerto Rico
[La] Idioma:eng
[Ab] Resumo:Autoimmune hepatitis (AIH) may present with clinical, laboratory abnormalities and histological features suggestive of primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC). These variants of AIH are known as overlap syndromes. We present a case of a 62 year-old-male who presented with altered liver function tests, anemia and unintentional weight loss. Initial laboratories revealed anemia and a cholestatic pattern. Diagnostic work-up was remarkable for a positive antinuclear antibodies (ANA) test and a liver biopsy suggestive of an AIH-PBC overlap syndrome. This case illustrates the complexity of establishing the diagnosis and effective therapy in this condition.
[Mh] Termos MeSH primário: Hepatite Autoimune/diagnóstico
Cirrose Hepática Biliar/diagnóstico
Doenças do Tecido Conjuntivo Indiferenciado/diagnóstico
[Mh] Termos MeSH secundário: Anticorpos Antinucleares/imunologia
Hepatite Autoimune/imunologia
Seres Humanos
Cirrose Hepática Biliar/imunologia
Doenças do Tecido Conjuntivo Indiferenciado/imunologia
[Nm] Nome de substância:
0 (Antibodies, Antinuclear)
[Em] Mês de entrada:1712
[Cu] Atualização por classe:171214
[Lr] Data última revisão:
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171128
[St] Status:MEDLINE

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