| [PMID]: | 29465536 |
| [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
Feminino
Hepatite Autoimune/tratamento farmacológico
Hepatite Autoimune/imunologia
Seres Humanos
Imunossupressores/administração & dosagem
Cirrose Hepática Biliar/tratamento farmacológico
Cirrose Hepática Biliar/imunologia
Meia-Idade
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
|
| [Pt] Tipo de publicação: | CASE REPORTS; JOURNAL ARTICLE |
[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:
| 180302 |
| [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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