S-51
Division of Rheumatology
1
, Internal
Medicine
2
, Pedriatrics
3
, Pathology
4
, and
Dermatology
5
. Hospital Universitario
Marqués de Valdecilla, IDIVAL, University
of Cantabria. Santander, Spain.
Francisco Ortiz-Sanjuán, MD*
Ricardo Blanco, MD, PhD*
Javier Loricera, MD
José Luis Hernández MD, PhD**
Trinitario Pina MD
Vanesa Calvo-Río, MD
Lino Álvarez, MD
M Carmen González-Vela, MD, PhD
Javier Rueda-Gotor, MD
Marcos A. González-López MD, PhD
Miguel A. González-Gay, MD, PhD**
*Drs. Ortiz-Sanjuán and Blanco shared
frst authorship.
**Drs. González-Gay and Hernández
shared senior authorship.
Please address correspondence to:
Miguel A. González-Gay or
Ricardo Blanco,
Rheumatology Division,
Hospital Universitario Marqués
de Valdecilla,
Avda. Valdecilla s/n.,
39008, Santander. Spain.
E-mail: miguelaggay@hotmail.com
rblanco@humv.es
Received on February 24, 2014; accepted
in revised form on March 27, 2014.
Clin Exp Rheumatol 2014; 32 (Suppl. 82):
S51-S54.
© Copyright CliniCal and
ExpErimEntal rhEumatology 2014.
Funding: this study was supported by a
grant from “Fondo de Investigaciones
Sanitarias” PI12/00193 (Spain), and
was also partially supported by RETICS
Programs, RD08/0075 (RIER) and
RD12/0009/0013 from ‘‘Instituto de Salud
Carlos III’’ (ISCIII) (Spain).
Competing interests: none declared.
ABSTRACT
Objective. The term cutaneous vascu-
litis (CV) includes a wide and heteroge-
neous group of entities. The American
College of Rheumatology (ACR) estab-
lished a set of criteria to classify vas-
culitis in 1990. Our aim was to further
investigate into the applicability of
these criteria for the classifcation of
patients with primary CV.
Methods. We analysed a large and
unselected series of patients with CV
attended to a university referral centre
from January 1976 to December 2011.
Patients were classifed according to
the methodology and criteria proposed
by the ACR1990 core data set. Patients
were also classifed according to the
same ACR 1990 database as proposed
by Michel et al. in 1992 to differenti-
ate Henoch-Schönlein purpura (HSP)
from hypersensitivity vasculitis (HV).
Results. We assessed 766 patients (346
women and 420 men) with a mean age
of 34 years. Patients with cutaneous
lesions in the setting of conditions dif-
ferent from primary CV were excluded.
According to the 1990 ACR criteria,
405 (63.1%) of the 642 patients with
primary CV were classifed as having
HSP and 230 (35.8%) as HV. However,
119 (18.5%) patients fulflled the ACR
1990 criteria for both entities. In ad-
dition, 7 (1.1%) did not meet the ACR
1990 criteria for any of them and,
therefore, they were considered as non-
classifed vasculitis. When patients with
primary CV were tested for the Michel
et al. criteria, 392 (61.1%) were classi-
fed as having HSP and 250 (38.9%) as
HV. Frequent discordance between the
ACR 1990 and the Michel et al criteria
was observed. It ranged between 18.4
and 21.7% for HSP and 32.2 to 38%
for HV.
Conclusion. According to our data, the
ACR 1990 criteria are of limited value
for the classifcation of patients with
primary CV.
Vasculitis is a general term for a group
of related disorders characterised by
blood vessel infammation leading to
tissue or organ injury. Tissue biopsy is
the cornerstone of the diagnosis of vas-
culitis (1). However, in the daily clini-
cal practice it is not exempt of potential
limitations as sometimes it provides an
incomplete or non-specifc pathologic
result. In addition, biopsy is not always
feasible and it is not routinely performed
in typical cases of cutaneous vasculitis
(CV), in particular in children.
Classifcation of vasculitis has been
a challenging problem for decades.
Diagnosis often relies on a combina-
tion of clinical, laboratory and histo-
pathologic fndings. For this reason,
in 1990, the American College of
Rheumatology (ACR) proposed a se-
ries of classifcation criteria based on
data comparing clinical features of
patients with seven types of primary
vasculitis: Giant Cell Arteritis (GCA),
Takayasu Arteritis (TA), Wegener’s
Granulomatosis (WG), Churg-Strauss
Syndrome (CSS), Polyarteritis Nodosa
(PAN), Hypersensitivity Vasculitis (HV)
and Henoch-Schönlein Purpura (HSP)
(2).
The term “cutaneous vasculitis” (CV)
refers to a wide and heterogeneous
group of entities linked by the presence
of necrotising infammatory lesions in
the cutaneous blood vessels (3). The
clinical spectrum and epidemiology of
CV may range from an isolated cutane-
ous involvement to a life-threatening
syndrome (3, 4), including conditions
that following defnitions proposed
Reappraisal of the 1990 American College of Rheumatology
criteria for the classifcation of cutaneous vasculitis:
an analysis based on 766 patients
F. Ortiz-Sanjuán
1
, R. Blanco
1
, J. Loricera
1
, J.L. Hernández
2
, T. Pina
1
, V. Calvo-Río
1
,
L. Álvarez
3
, M.C. González-Vela
4
, J. Rueda-Gotor
1
, M.A. González-López
5
,
M.A. González-Gay
1