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Vol. 45. Issue 3.
Pages 177-185 (March 2022)
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Vol. 45. Issue 3.
Pages 177-185 (March 2022)
Original article
Characteristics and impact of sex in a cohort of patients with primary sclerosing cholangitis: Experience of a transplant center in the Mediterranean basin
Características e impacto del sexo en una cohorte de pacientes con colangitis esclerosante primaria: experiencia de un centro trasplantador de la cuenca mediterránea
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Alejandro Mínguez Sabatera,
Corresponding author
alejandromsab11@gmail.com

Corresponding author.
, Isabel Conde Amiela,b, Pablo Ladrón Abiaa, Sara Martínez Delgadoa, Ángel Camarasa Pérezc, Marina Berenguera,b,d,e
a Departamento de Gastroenterología, Hepatología y Unidad de Trasplante Hepático, Hospital Universitari y Politècnic La Fe, Valencia, Spain
b Instituto de Investigación Sanitaria La Fe, Valencia, Spain
c Cirugía General y del Aparato Digestivo, Hospital Universitario Nuestra Señora de la Candelaria, Santa Cruz de Tenerife, Tenerife, Spain
d Centro de Investigación Biomédica en Red del Área de Enfermedades Hepáticas y Digestivas (CIBEREHD), Instituto de Salud Carlos III, Madrid, Spain
e Departamento de Medicina, Universitat de València, Valencia, Spain
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Figures (2)
Tables (4)
Table 1. Description of baseline variables in patients with PSC.
Table 2. Inflammatory bowel disease.
Table 3. Differences in clinical manifestations according to sex.
Table 4. Differences in laboratory parameters according to sex.
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Abstract
Background and aims

Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease that typically affects middle-aged men with ulcerative colitis (UC). However, recent studies point out to epidemiological changes. Our aim was to determine if the epidemiology, clinical course and outcome of patients with PSC followed at a reference hepatology centre resemble what is described in the literature.

Patients and method

Retrospective search of patients with a diagnosis of PSC treated in our centre between 2000 and 2019.

Results

Cohort of 55 patients (mean age 37 years), 44% women. Most were large duct type (79%). Most diagnoses were made after 2011. At time of diagnosis, 63% of patients were asymptomatic. The median time from suspicion to diagnosis was 2 years. After a mean follow-up time of 7 years, one third developed cirrhosis, and 25% required liver transplantation (LT); among these, the disease recurred in almost half. Inflammatory bowel disease (IBD) was present in 45%, especially UC. Although statistical significance was not reached, PSC in women was characterised by higher rate of asymptomatic presentation and more frequent association with UC versus other forms of IBD. Women also had more frequently cirrhosis at diagnosis and required LT more often than men.

Conclusion

The epidemiology of PSC is changing. The number of women affected is greater than what was expected from the literature, with a recent increase in incidence. There seems to be differences between sexes in the form of presentation and disease course that should be confirmed in subsequent studies.

Keywords:
Primary sclerosing cholangitis
Liver transplantation
Sex
Inflammatory bowel disease
Resumen
Introducción y objetivos

La colangitis esclerosante primaria (CEP) es una enfermedad hepática colestásica rara que típicamente afecta a varones de mediana edad con colitis ulcerosa. No obstante, estudios recientes apuntan a cambios epidemiológicos. Nuestro objetivo es determinar si la epidemiología, presentación clínica y curso evolutivo de pacientes con CEP seguidos en un centro de referencia se asemejan a lo descrito en la literatura.

Pacientes y métodos

Búsqueda retrospectiva de pacientes con diagnóstico de CEP atendidos en nuestro centro entre los años 2000 y 2019.

Resultados

Cohorte de 55 pacientes (media de edad 37 años), 44% mujeres, afectos de CEP, 79% de ducto grande. Casi dos tercios fueron diagnosticados a partir de 2011. En el momento del diagnóstico, un 63% de los pacientes se encontraba asintomático. La mediana de tiempo desde la sospecha hasta el diagnóstico fue de 2 años. Un 34% desarrolló cirrosis en el seguimiento, y 25% requirió trasplante hepático (TH) tras una media de tiempo de 7 años; entre estos, la enfermedad recurrió en un 46%. Un 45% presentaba una EII, sobre todo colitis ulcerosa (CU). Si bien no se alcanzó significación estadística, la CEP en mujeres se caracterizó por mayor tasa de presentación asintomática, mayor asociación con CU frente a otras formas de EII, así como cirrosis al diagnóstico y necesidad de TH con mayor frecuencia que los hombres.

Conclusiones

La epidemiología de la CEP está cambiando. El número de mujeres afectas es mayor al descrito previamente, objetivándose un aumento reciente de la incidencia. Podrían existir diferencias entre sexos en la forma de presentación y evolución que deberán confirmarse en estudios posteriores.

Palabras clave:
Colangitis esclerosante primaria
Trasplante hepático
Sexo
Enfermedad inflamatoria intestinal

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