Enfermedades ampollares autoinmunes: caracterización clínica, respuesta terapéutica y mortalidad en un centro universitario de Chile

Daniela Carvajal Aguilera, Javier Fernández Moraga, Fernando Valenzuela Ahumada, Claudia Morales Huber, Alejandra Fernández Moraga

Resultado de la investigación: Article

Resumen

Introduction: Autoimmune Bullous Diseases (ABDs) are acquired dermatoses characterised by blisters on the skin and mucous membranes. The aim of this study is to describe the epidemiology, clinical features, therapeutic response, and mortality rates in patients with ABDs treated at a university medical centre in Chile. Method: Retrospective cohort study was conducted over a 12 year period (2005-2017) of patients with ABDs confirmed by histology and direct immunofluorescence at a university medical centre in Chile. Results: Of a total of 89 patients, 50.9% were diagnosed with Bullous Pemphigoid (BP), 24.7% with Pemphigus Vulgaris (PV), and 24.7% with other ABD. The mean age in BP was 72.2 years, and 46.3 years in PV. Blisters appeared in 93.2% of BP, and mucous compromise appeared in 77.3% of PV. In BP, the most used treatment was prednisone and topical corticosteroids (TC), while in PV it was prednisone with azathioprine and TC. Half (50%) of BP achieved remission after 2 months of treatment, while in PV it was achieved after 5 months. The one-year survival rate in patients with BP was 88.7%, and 96.3% in PV. Conclusions: BP occurred at an older age, with limb and trunk blisters, whereas PV occurred in middle-aged patients, with significant mucosal involvement. Patients with PV required greater immunosuppression, reaching remission later than in BP. However, survival was lower in patients with BP. This study, the first in Chile, allowed ABDs to be characterised in Latin America.

Idioma originalSpanish
PublicaciónPiel
DOI
EstadoPublished - 1 ene 2019

Huella dactilar

Bullous Pemphigoid
Chile
Pemphigus
Autoimmune Diseases
Mortality
Blister
Therapeutics
Prednisone
Adrenal Cortex Hormones
Direct Fluorescent Antibody Technique
Latin America
Azathioprine
Skin Diseases
Immunosuppression
Histology
Mucous Membrane
Epidemiology
Cohort Studies
Survival Rate
Extremities

ASJC Scopus subject areas

  • Dermatology

Citar esto

Carvajal Aguilera, Daniela ; Fernández Moraga, Javier ; Valenzuela Ahumada, Fernando ; Morales Huber, Claudia ; Fernández Moraga, Alejandra. / Enfermedades ampollares autoinmunes : caracterización clínica, respuesta terapéutica y mortalidad en un centro universitario de Chile. En: Piel. 2019.
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title = "Enfermedades ampollares autoinmunes: caracterizaci{\'o}n cl{\'i}nica, respuesta terap{\'e}utica y mortalidad en un centro universitario de Chile",
abstract = "Introduction: Autoimmune Bullous Diseases (ABDs) are acquired dermatoses characterised by blisters on the skin and mucous membranes. The aim of this study is to describe the epidemiology, clinical features, therapeutic response, and mortality rates in patients with ABDs treated at a university medical centre in Chile. Method: Retrospective cohort study was conducted over a 12 year period (2005-2017) of patients with ABDs confirmed by histology and direct immunofluorescence at a university medical centre in Chile. Results: Of a total of 89 patients, 50.9{\%} were diagnosed with Bullous Pemphigoid (BP), 24.7{\%} with Pemphigus Vulgaris (PV), and 24.7{\%} with other ABD. The mean age in BP was 72.2 years, and 46.3 years in PV. Blisters appeared in 93.2{\%} of BP, and mucous compromise appeared in 77.3{\%} of PV. In BP, the most used treatment was prednisone and topical corticosteroids (TC), while in PV it was prednisone with azathioprine and TC. Half (50{\%}) of BP achieved remission after 2 months of treatment, while in PV it was achieved after 5 months. The one-year survival rate in patients with BP was 88.7{\%}, and 96.3{\%} in PV. Conclusions: BP occurred at an older age, with limb and trunk blisters, whereas PV occurred in middle-aged patients, with significant mucosal involvement. Patients with PV required greater immunosuppression, reaching remission later than in BP. However, survival was lower in patients with BP. This study, the first in Chile, allowed ABDs to be characterised in Latin America.",
keywords = "Autoimmune bullous diseases, Bullous pemphigoid, Epidemiology, Pemphigus vulgaris, Survival, Treatment",
author = "{Carvajal Aguilera}, Daniela and {Fern{\'a}ndez Moraga}, Javier and {Valenzuela Ahumada}, Fernando and {Morales Huber}, Claudia and {Fern{\'a}ndez Moraga}, Alejandra",
year = "2019",
month = "1",
day = "1",
doi = "10.1016/j.piel.2019.03.002",
language = "Spanish",
journal = "Piel",
issn = "0213-9251",
publisher = "Ediciones Doyma, S.L.",

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Enfermedades ampollares autoinmunes : caracterización clínica, respuesta terapéutica y mortalidad en un centro universitario de Chile. / Carvajal Aguilera, Daniela; Fernández Moraga, Javier; Valenzuela Ahumada, Fernando; Morales Huber, Claudia; Fernández Moraga, Alejandra.

En: Piel, 01.01.2019.

Resultado de la investigación: Article

TY - JOUR

T1 - Enfermedades ampollares autoinmunes

T2 - caracterización clínica, respuesta terapéutica y mortalidad en un centro universitario de Chile

AU - Carvajal Aguilera, Daniela

AU - Fernández Moraga, Javier

AU - Valenzuela Ahumada, Fernando

AU - Morales Huber, Claudia

AU - Fernández Moraga, Alejandra

PY - 2019/1/1

Y1 - 2019/1/1

N2 - Introduction: Autoimmune Bullous Diseases (ABDs) are acquired dermatoses characterised by blisters on the skin and mucous membranes. The aim of this study is to describe the epidemiology, clinical features, therapeutic response, and mortality rates in patients with ABDs treated at a university medical centre in Chile. Method: Retrospective cohort study was conducted over a 12 year period (2005-2017) of patients with ABDs confirmed by histology and direct immunofluorescence at a university medical centre in Chile. Results: Of a total of 89 patients, 50.9% were diagnosed with Bullous Pemphigoid (BP), 24.7% with Pemphigus Vulgaris (PV), and 24.7% with other ABD. The mean age in BP was 72.2 years, and 46.3 years in PV. Blisters appeared in 93.2% of BP, and mucous compromise appeared in 77.3% of PV. In BP, the most used treatment was prednisone and topical corticosteroids (TC), while in PV it was prednisone with azathioprine and TC. Half (50%) of BP achieved remission after 2 months of treatment, while in PV it was achieved after 5 months. The one-year survival rate in patients with BP was 88.7%, and 96.3% in PV. Conclusions: BP occurred at an older age, with limb and trunk blisters, whereas PV occurred in middle-aged patients, with significant mucosal involvement. Patients with PV required greater immunosuppression, reaching remission later than in BP. However, survival was lower in patients with BP. This study, the first in Chile, allowed ABDs to be characterised in Latin America.

AB - Introduction: Autoimmune Bullous Diseases (ABDs) are acquired dermatoses characterised by blisters on the skin and mucous membranes. The aim of this study is to describe the epidemiology, clinical features, therapeutic response, and mortality rates in patients with ABDs treated at a university medical centre in Chile. Method: Retrospective cohort study was conducted over a 12 year period (2005-2017) of patients with ABDs confirmed by histology and direct immunofluorescence at a university medical centre in Chile. Results: Of a total of 89 patients, 50.9% were diagnosed with Bullous Pemphigoid (BP), 24.7% with Pemphigus Vulgaris (PV), and 24.7% with other ABD. The mean age in BP was 72.2 years, and 46.3 years in PV. Blisters appeared in 93.2% of BP, and mucous compromise appeared in 77.3% of PV. In BP, the most used treatment was prednisone and topical corticosteroids (TC), while in PV it was prednisone with azathioprine and TC. Half (50%) of BP achieved remission after 2 months of treatment, while in PV it was achieved after 5 months. The one-year survival rate in patients with BP was 88.7%, and 96.3% in PV. Conclusions: BP occurred at an older age, with limb and trunk blisters, whereas PV occurred in middle-aged patients, with significant mucosal involvement. Patients with PV required greater immunosuppression, reaching remission later than in BP. However, survival was lower in patients with BP. This study, the first in Chile, allowed ABDs to be characterised in Latin America.

KW - Autoimmune bullous diseases

KW - Bullous pemphigoid

KW - Epidemiology

KW - Pemphigus vulgaris

KW - Survival

KW - Treatment

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U2 - 10.1016/j.piel.2019.03.002

DO - 10.1016/j.piel.2019.03.002

M3 - Article

AN - SCOPUS:85064901761

JO - Piel

JF - Piel

SN - 0213-9251

ER -