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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   §
1   Internal Medicine, Department of Pathophysiology and Transplantation, University of Milan, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy
,
Angelo V. Marzano   §
2   Dermatology Unit, Department of Pathophysiology and Transplantation, University of Milan, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy
,
Paolo Bucciarelli
3   A. Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy
,
Ylenia Balice
2   Dermatology Unit, Department of Pathophysiology and Transplantation, University of Milan, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy
,
Giuseppe Cianchini
4   Department of Immunodermatology, Istituto Dermopatico dell’Immacolata, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Rome, Italy
,
Pietro Quaglino
5   Department of Medical Sciences Dermatologic Division, University of Turin, Turin, Italy
,
Piergiacomo Calzavara Pinton
6   Department of Dermatology, University Hospital Spedali Civili, Brescia, Italy
,
Marzia Caproni
7   Department of Surgery and Translational Medicine, Section of Dermatology, University of Florence, Florence, Italy
,
Mauro Alaibac
8   Unit of Dermatology, University of Padua, Padua, Italy
,
Clara De Simone
9   Department of Dermatology, Catholic University of the Sacred Heart, Rome, Italy
,
Annalisa Patrizi
10   Dermatology, Department of Specialized, Experimental and Diagnostic Medicine, Sant’Orsola -Malpighi Hospital, University of Bologna, Italy
,
Emanuele Cozzani
11   IRCCS AOU San Martino Di.S.Sal., Section of Dermatology, University of Genoa, Genoa, Italy
,
Manuela Papini
12   Department of Surgical and Biomedical Sciences, Dermatology Unit of Terni, University of Perugia, Terni, Italy
,
Alberto Tedeschi
1   Internal Medicine, Department of Pathophysiology and Transplantation, University of Milan, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy
,
Emilio Berti
2   Dermatology Unit, Department of Pathophysiology and Transplantation, University of Milan, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy
,
Frits R. Rosendaal
13   Department of Clinical Epidemiology, and Department of Thrombosis and Hemostasis, Leiden University Medical Center, Leiden, The Netherlands
,
for the INVENTEP Study Group › Author Affiliations Financial support:This work was supported by “Ricerca corrente”, Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Milano, Italy. The funder had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.
Further Information

Publication History

Received: 15 April 2015

Accepted after major revision: 19 June 2015

Publication Date:
22 November 2017 (online)

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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

§ Drs Cugno and Marzano contributed equally to this article


* INVENTEP Study Group: Paolo Fabbri7, Aurora Parodi11, Carlo Crosti2, Giovanna Zambruno4 , Ornella De Pità4, Emiliano Antiga7, Federico Bardazzi10, Michelangelo La Placa10, Elena Marra5, Marta B. Brumana8, Giorgio Pasolini6, Annalisa Vascellaro6, Ivana Zacconi12


 

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