Reumatol Clin. 2015;11(4):244–246
ww w . r eumatologiaclinica.org
Case Report
Dermatomyositis and Myasthenia Gravis: An Uncommon Association
With Therapeutic Implications
Clara Sangüesa Gómez, Bryan Josué Flores Robles, Clara Méndez Perles, Carmen Barbadillo,
Hildegarda Godoy, José Luis Andréu
*
Servicio de Reumatología, Hospital Puerta de Hierro-Majadahonda, Majadahonda, Madrid, Spain
a r t i c l e i n f o
Article history:
Received 23 July 2014
Accepted 10 October 2014
Available online 21 January 2015
Keywords:
Dermatomyositis
Myasthenia gravis
Inflammatory myopathies
a b s t r a c t
The association of dermatomyositis with myasthenia gravis (MG) is uncommon, having been reported
so far in only 26 cases. We report the case of a 69 year-old man diagnosed with MG two years ago and
currently treated with piridostigmyne. The patient developed acute proximal weakness, shoulder pain
and elevated creatine-kinase (CK). He also developed generalized facial erythema and Gottron’s papules.
Laboratory tests showed positive antinuclear and anti-Mi2 antibodies. Further analysis confirmed CK
levels above 1000 U/l. The clinical management of the patient and the therapeutic implications derived
from the coexistence of both entities are discussed.
© 2014 Elsevier Espa ˜ na, S.L.U. All rights reserved.
Dermatomiositis y miastenia gravis: una asociación infrecuente
con implicaciones terapéuticas
Palabras clave:
Dermatomiositis
Miastenia gravis
Miopatías inflamatorias
r e s u m e n
La asociación de dermatomiositis y miastenia gravis (MG) es infrecuente, habiéndose comunicado
hasta la actualidad únicamente 26 casos. Se presenta el caso de un varón de 69 a˜ nos diagnosticado de
MG 2 a˜ nos atrás, en tratamiento con piridostigmina, que inicia cuadro agudo de debilidad muscular
proximal, artralgias en hombros y elevación de creatincinasa (CK); así como aparición de eritema facial
generalizado y pápulas de Gottron. En el estudio de laboratorio se evidenció positividad de anticuerpos
antinucleares y anti-Mi2. Ulteriores determinaciones de CK mostraron niveles por encima de 1.000 U/l.
Se discute el manejo clínico de este paciente y las implicaciones terapéuticas que plantea la coexistencia
de ambas entidades.
© 2014 Elsevier Espa ˜ na, S.L.U. Todos los derechos reservados.
Introduction
Idiopathic inflammatory myopathies (IIM), including der-
matomyositis (DM), are a heterogeneous group of systemic
autoimmune diseases whose main clinical feature is predomi-
nantly proximal muscle weakness.
1
They are rare diseases, with
an estimated annual incidence of 6 cases/10
5
individuals
2
and may
be associated with other autoimmune diseases such as lupus or
Sjogren’s syndrome.
Please cite this article as: Sangüesa Gómez C, Flores Robles BJ, Méndez Perles C,
Barbadillo C, Godoy H, Andréu JL. Dermatomiositis y miastenia gravis: una asociación
infrecuente con implicaciones terapéuticas. Reumatol Clin. 2015;11:244–246.
*
Corresponding author.
E-mail address: jlandreu@arrakis.es (J.L. Andréu).
Another autoimmune disease that causes muscle weak-
ness, myasthenia gravis (MG), can also be associated with the
IIM.
3–9
MG is rare, with an estimated prevalence of 140 cases/
10
6
inhabitants.
10
Its pathogenesis derives from the compromise
resulting from the binding of autoantibodies to proteins involved
in the neuromuscular transmission signaling. Recognizing the
association between the two entities is important due to its
therapeutic implications. We present a case of DM in a patient
previously diagnosed with MG.
Clinical Case
The patient was a 69 year-old-male Caucasian, diagnosed two
years prior with MG, positive to anti-AChR antibodies and treated
with pyridostigmine (60 mg/8 h). He was referred to our center
due to the appearance of weakness which appeared a month
2173-5743/© 2014 Elsevier Espa ˜ na, S.L.U. All rights reserved.