Case Report
Elevated Troponin Serum Levels in Adult Onset Still’s Disease
Carlo Umberto Manzini,
1
Lucio Brugioni,
2
Michele Colaci,
1
Maurizio Tognetti,
2
Amelia Spinella,
1
Marco Sebastiani,
1
Dilia Giuggioli,
1
and Clodoveo Ferri
1
1
Chair and Rheumatology Unit, Medical School, Azienda Ospedaliero-Universitaria, University of Modena and Reggio Emilia,
Policlinico di Modena, Via del Pozzo 71, 41100 Modena, Italy
2
Critical Care Unit, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy
Correspondence should be addressed to Clodoveo Ferri; clferri@unimore.it
Received 12 December 2014; Revised 28 January 2015; Accepted 30 January 2015
Academic Editor: Mehmet Soy
Copyright © 2015 Carlo Umberto Manzini et al. Tis is an open access article distributed under the Creative Commons Attribution
License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Adult onset Still’s disease (AOSD) is a rare infammatory systemic disease that occasionally may afect myocardium. Diagnosis
is based on typical AOSD symptoms afer the exclusion of well-known infectious, neoplastic, or autoimmune/autoinfammatory
disorders. In the case of abrupt, recent onset AOSD, it could be particularly difcult to make the diferential diagnosis and in
particular to early detect the possible heart involvement. Tis latter event is suggested by the clinical history of the four patients
described here, incidentally observed at our emergency room. All cases were referred because of acute illness (high fever, malaise,
polyarthralgias, skin rash, and sore throat), successively classifed as AOSD, and they presented abnormally high levels of serum
troponin without overt symptoms of cardiac involvement. Te timely treatment with steroids (3 cases) or ibuprofen (1 case) leads to
the remission of clinicoserological manifestations within few weeks. Tese observations suggest that early myocardial injury might
be underestimated or entirely overlooked in patients with AOSD; routine cardiac assessment including troponin evaluation should
be mandatory in all patients with suspected AOSD.
1. Introduction
Adult onset Still’s disease (AOSD) is a rare infamma-
tory disease characterized by spiking fevers >39
∘
C, arthri-
tis/arthralgias, typical salmon-colored bumpy rash, and pos-
sible involvement of visceral organs [1]. Te etiology of AOSD
remains obscure, but an autoimmune pathogenesis has been
suggested [2]. Te age peak of disease onset is between 16
and 35 years, with an incidence below 1/100,000 inhabitants,
without gender diferences. Te diagnosis is exclusively based
on clinical symptoms, according to the criteria proposed
by Yamaguchi et al. [3] or Cush et al. [4], afer the exclu-
sion of well-known infectious, neoplastic, or other autoim-
mune/autoinfammatory disorders [5]. Corticosteroids and
nonsteroidal anti-infammatory drugs represent the frst
line therapy in the acute phase, while disease-modifying
antirheumatic drugs (sulphasalazine, hydroxychloroquine, or
methotrexate) or anti-TNF blockers can be considered in
the chronic subsets.
Among AOSD clinical manifestations, serositises, includ-
ing pericardial efusion, are also described (approximately
30–40% of cases), as well as myocardium/endocardium
involvement [6–13]. Pericardial complication can be one
of the presenting symptoms [8, 14, 15] or manifestation of
relapsing disease [16–18]. In this context, increased troponin
levels have also been reported in anecdotal observations,
probably related to heart involvement [14]. Troponin is a
protein found only in cardiac myocytes; it exerts an impor-
tant role in regulating the interaction of actin and myosin
flaments during cardiac contraction. Because of its cardiac
origin, troponin is commonly used as a very sensitive and
specifc marker of myocardial damage, not only in the case
of myocardial ischemia, but also in infammatory diseases
inducing cardiac injury, such as AOSD [19].
We describe four consecutive patients, referred to the
emergency room of our university-based hospital, who pre-
sented abnormally increased levels of serum troponin in the
Hindawi Publishing Corporation
Case Reports in Rheumatology
Volume 2015, Article ID 732095, 4 pages
http://dx.doi.org/10.1155/2015/732095