RESUMO
BACKGROUND & AIMS: Discontinuation of anti-tumor necrosis factor-α treatment (anti-TNF) (infliximab and adalimumab) in patients with inflammatory bowel disease (IBD) is associated with a high relapse risk that may be influenced by endoscopic activity at the time of stopping. We assessed the relapse rate after anti-TNF withdrawal in patients with endoscopic healing and studied predictors of relapse including the depth of endoscopic healing. METHODS: This was a multicenter, prospective study in adult patients with Crohn's disease (CD), ulcerative colitis (UC), or IBD-unclassified (IBDU), with ≥6 months of corticosteroid-free clinical remission (confirmed at baseline) and endoscopic healing (Mayo <2/SES-CD <5 without large ulcers), who discontinued anti-TNF between 2018 and 2020 in the Netherlands. We performed Kaplan-Meier and Cox regression analyses to assess the relapse rate and evaluate potential predictors: partial (Mayo 1/SES-CD 3-4) versus complete (Mayo 0/SES-CD 0-2) endoscopic healing, anti-TNF trough levels, and immunomodulator and/or mesalamine use. RESULTS: Among 81 patients (CD: n = 41, 51%) with a median follow-up of 2.0 years (interquartile range, 1.6-2.1), 40 patients (49%) relapsed. Relapse rates in CD and UC/IBDU patients were comparable. At 12 months, 70% versus 35% of patients with partial versus complete endoscopic healing relapsed, respectively (adjusted hazard rate [aHR], 3.28; 95% confidence interval [CI], 1.43-7.50). Mesalamine use was associated with fewer relapses in UC/IBDU patients (aHR, 0.08; 95% CI, 0.01-0.67). Thirty patients restarted anti-TNF, and clinical remission was regained in 73% at 3 months. CONCLUSIONS: The relapse risk was high after anti-TNF withdrawal in IBD patients with endoscopic healing, but remission was regained in most cases after anti-TNF reintroduction. Complete endoscopic healing and mesalamine treatment in UC/IBDU patients decreased the risk of relapse.
Assuntos
Colite Ulcerativa , Doença de Crohn , Doenças Inflamatórias Intestinais , Adulto , Humanos , Inibidores do Fator de Necrose Tumoral/uso terapêutico , Mesalamina/uso terapêutico , Estudos Prospectivos , Doenças Inflamatórias Intestinais/tratamento farmacológico , Doença de Crohn/tratamento farmacológico , Infliximab/uso terapêutico , Colite Ulcerativa/tratamento farmacológico , Doença Crônica , Recidiva , Indução de RemissãoAssuntos
Valvas Cardíacas/anormalidades , Insuficiência da Valva Mitral/epidemiologia , Prolapso da Valva Mitral/epidemiologia , Rim Policístico Autossômico Dominante/complicações , Adulto , Idoso , Sequência de Bases , Proteínas de Ligação ao Cálcio , Feminino , Glucosidases/genética , Valvas Cardíacas/diagnóstico por imagem , Humanos , Peptídeos e Proteínas de Sinalização Intracelular/genética , Masculino , Proteínas de Membrana/genética , Pessoa de Meia-Idade , Chaperonas Moleculares , Dados de Sequência Molecular , Mutação/genética , Prevalência , Estudos Prospectivos , Proteínas de Ligação a RNA , Análise de Sequência de DNA , UltrassonografiaRESUMO
BACKGROUND AND AIM: Isolated polycystic liver disease (PCLD) is characterized by the presence of multiple cysts in the liver in the absence of polycystic kidneys. The clinical profile of PCLD is poorly defined and we set up a study for the clinical characteristics of PCLD. METHODS: We collected clinical data on 188 PCLD patients (defined as >10 liver cysts) from five tertiary referral centres, and 137 patients were selected for the purpose of this study. We performed molecular analysis of the PCLD associated genes PRKCSH and SEC63 in 91 patients. RESULTS: A total of 118 (86%) patients were female. The majority of patients (88%) had >20 cysts. The median age at diagnosis was 47 years (range 23-84). 37 (41%) patients carried a mutation. Clinical symptoms at presentation were present in 111 (84%) patients. γ-glutamyl transferase was elevated to 1.4 times upper limit of normal (interquartile range 1.0-2.7). The presence of a mutation and female gender predicted a more severe course: female patients were 9 years younger at the time of diagnosis (47 years; range 23-84) and 91% had symptoms (P<0.01); likewise, mutation carriers were younger at presentation (39 years; range 35-48) and 95% of this cohort had symptoms (P<0.01). During follow-up [median 8.2 years (range 0-35)], 10% of untreated and 51% of treated patients developed complications. Mortality in this cohort was 8%, but only 2% died of PCLD-related causes. 58% of patients were treated a median of 2 years (range 0-25) after diagnosis. CONCLUSION: Symptomatic PCLD patients are mainly females. Females and mutation carriers were younger at diagnosis and had a more severe course of disease.
Assuntos
Centros Médicos Acadêmicos/estatística & dados numéricos , Glucosidases/genética , Peptídeos e Proteínas de Sinalização Intracelular/genética , Proteínas de Membrana/genética , Mutação , Encaminhamento e Consulta/estatística & dados numéricos , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Doenças Assintomáticas , Bélgica , Biomarcadores/sangue , Proteínas de Ligação ao Cálcio , Distribuição de Qui-Quadrado , Cistos/diagnóstico , Cistos/genética , Cistos/mortalidade , Cistos/terapia , Feminino , Predisposição Genética para Doença , Humanos , Estimativa de Kaplan-Meier , Hepatopatias/diagnóstico , Hepatopatias/genética , Hepatopatias/mortalidade , Hepatopatias/terapia , Masculino , Pessoa de Meia-Idade , Chaperonas Moleculares , Países Baixos , Fenótipo , Proteínas de Ligação a RNA , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Índice de Gravidade de Doença , Fatores Sexuais , Fatores de Tempo , Adulto JovemRESUMO
PURPOSE: To study the extent to which aspiration-sclerotherapy reduces liver volume and whether this therapy results in relief of symptoms. RESULTS: Four patients, group I, with isolated large liver cysts, and 11 patients, group II, with polycystic livers, underwent aspiration-sclerotherapy. Average volume of aspirated cyst fluid was 1,044 ml (range 225-2,000 ml) in group I and 1,326 ml (range 40-4,200 ml) in group II. Mean liver volume before the procedure was 2,157 ml (range 1,706-2,841 ml) in group I and 4,086 ml (range 1,553-7,085 ml) in group II. This decreased after the procedure to 1,757 ml (range 1,479-2,187 ml) in group I. In group II there was a statistically significant decrease to 3,347 ml (range 1,249-6,930 ml, P = 0.008). Volume reduction was 17.1% (range -34.7% to -4.1%) and 19.2% (range -53.9% to +2.4%) in groups I and II, respectively. Clinical severity of all symptoms decreased, except for involuntary weight loss and pain in group II. CONCLUSION: Aspiration-sclerotherapy is an effective means of achieving liver volume reduction and relief of symptoms.
Assuntos
Cistos/terapia , Hepatopatias/terapia , Fígado/patologia , Escleroterapia , Sucção , Adulto , Idoso , Idoso de 80 Anos ou mais , Cistos/complicações , Cistos/patologia , Feminino , Humanos , Hepatopatias/complicações , Hepatopatias/patologia , Masculino , Pessoa de Meia-Idade , Tamanho do Órgão , Índice de Gravidade de Doença , Inquéritos e Questionários , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Ultrassonografia de IntervençãoRESUMO
We present a 53-year-old female suffering from familial adenomatous polyposis, who was found to have a positive nodus, lateral to the hilus of the left lung, on routine FDG-PET scan. This lesion was found to be a sclerosing hemangioma. We found an aberrant beta-catenin expression on immunohistochemical staining, suggesting that sclerosing hemangioma and familial adenomatous polyposis share the same pathophysiology. It is important to be aware of the association of familial adenomatous polyposis and sclerosing hemangioma.