Increased risk of venous thromboembolism in patients with bullous pemphigoid

Massimo Cugno; Angelo V. Marzano; Paolo Bucciarelli; Ylenia Balice; Giuseppe Cianchini; Pietro Quaglino; Piergiacomo Calzavara Pinton; Marzia Caproni; Mauro Alaibac; Clara De Simone; Annalisa Patrizi; Emanuele Cozzani; Manuela Papini; Alberto Tedeschi; Emilio Berti; Frits R. Rosendaal; for the INVENTEP Study Group

doi:10.1160/TH15-04-0309

Thrombosis and Haemostasis, Table of Contents

Thromb Haemost 2016; 115(01): 193-199
DOI: 10.1160/TH15-04-0309

Stroke, Systemic or Venous Thromboembolism

Schattauer GmbH Schattauer

Increased risk of venous thromboembolism in patients with bullous pemphigoid

The INVENTEP (INcidence of VENous ThromboEmbolism in bullous Pemphigoid) study

Massimo Cugno^§

¹Internal Medicine, Department of Pathophysiology and Transplantation, University of Milan, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy

,

Angelo V. Marzano^§

²Dermatology Unit, Department of Pathophysiology and Transplantation, University of Milan, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy

,

Paolo Bucciarelli

³A. Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy

,

Ylenia Balice

²Dermatology Unit, Department of Pathophysiology and Transplantation, University of Milan, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy

,

Giuseppe Cianchini

⁴Department of Immunodermatology, Istituto Dermopatico dell’Immacolata, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Rome, Italy

,

Pietro Quaglino

⁵Department of Medical Sciences Dermatologic Division, University of Turin, Turin, Italy

,

Piergiacomo Calzavara Pinton

⁶Department of Dermatology, University Hospital Spedali Civili, Brescia, Italy

,

Marzia Caproni

⁷Department of Surgery and Translational Medicine, Section of Dermatology, University of Florence, Florence, Italy

,

Mauro Alaibac

⁸Unit of Dermatology, University of Padua, Padua, Italy

,

Clara De Simone

⁹Department of Dermatology, Catholic University of the Sacred Heart, Rome, Italy

,

Annalisa Patrizi

¹⁰Dermatology, Department of Specialized, Experimental and Diagnostic Medicine, Sant’Orsola -Malpighi Hospital, University of Bologna, Italy

,

Emanuele Cozzani

¹¹IRCCS AOU San Martino Di.S.Sal., Section of Dermatology, University of Genoa, Genoa, Italy

,

Manuela Papini

¹²Department of Surgical and Biomedical Sciences, Dermatology Unit of Terni, University of Perugia, Terni, Italy

,

Alberto Tedeschi

¹Internal Medicine, Department of Pathophysiology and Transplantation, University of Milan, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy

,

Emilio Berti

²Dermatology Unit, Department of Pathophysiology and Transplantation, University of Milan, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy

,

Frits R. Rosendaal

¹³Department of Clinical Epidemiology, and Department of Thrombosis and Hemostasis, Leiden University Medical Center, Leiden, The Netherlands

,

for the INVENTEP Study Group

› Author Affiliations

Abstract

Summary

Activation of blood coagulation has been demonstrated in bullous pemphigoid (BP), a rare autoimmune blistering disease, potentially leading to a prothrombotic state. In order to evaluate the incidence of venous thromboembolism (VTE) in BP, a cohort study was carried out on 432 BP patients (59 % females; median age 76 years, interquartile range [IQR]: 68–82). At diagnosis, autoimmune bullous skin disorder intensity score (ABSIS) was calculated. VTE incidence was standardised with rates of the general population. Multivariable Cox proportional hazard model was used to estimate the hazard ratio of VTE according to ABSIS and concomitant risk factors. During a median follow-up of 4.2 years, 31 objectively-diagnosed VTE events were recorded. The incidence rate of VTE (per 1000 patient-years) was 17.2 overall (95 % confidence interval [CI]: 11.1–23.2), 56.7 (95 %CI: 33.0–80.4) during acute phase (22 VTE) and 6.3 (95 %CI: 2.8–11.3) during remission (9 VTE). The standardised incidence ratio was 4.06 (95 %CI: 2.73–5.65), higher during the acute phase (14.86, 95 %CI: 9.20–21.88) than during remission (1.48, 0.66–2.63). The adjusted hazard ratio of VTE was 2.74 (95 %CI: 1.07–7.04) for ABSIS > 48 vs ABSIS < 28, and 2.56 (95 %CI: 1.00–6.70) in patients with [uni2265] 2 concomitant risk factors. In conclusion, BP patients have a 15-fold increased VTE risk during acute phase, proportional to disease severity and heightened by concomitant risk factors.

Keywords

Bullous pemphigoid - autoimmune bullous skin disorders - thromboembolism - thrombotic risk

Full Text

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