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Symptomatic macroglossia and tongue myositis in polymyositisTreatment with corticosteroids and intravenous immunoglobulin.

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ARTHRITIS & RHEUMATISM
Vol. 46, No. 10, October 2002, pp 2762–2764
DOI 10.1002/art.10548
© 2002, American College of Rheumatology
Symptomatic Macroglossia and Tongue Myositis in Polymyositis
Treatment With Corticosteroids and Intravenous Immunoglobulin
E. Chauvet, L. Sailler, M. Carreiro, J. R. Paoli, P. Arrue, L. Astudillo, F. Oksmann,
M. B. Delisle, and Ph. Arlet
Symptomatic macroglossia occurs in some rare
congenital muscle diseases, such as Becker’s and Duchenne’s dystrophies or Pompe’s disease. Herein we describe a case of symptomatic macroglossia with myositis
of the tongue occurring in a patient with polymyositis.
Tongue myositis was evidenced by dysarthria, frequent
biting during mastication, swallowing difficulties without aspiration, and noisy breathing. Magnetic resonance imaging showed homogeneous hypertrophy of the
tongue, especially the mouth’s floor muscles. The diagnosis of tongue myositis was established by electromyography and biopsy. No other cause for the macroglossia
was found. Symptoms resolved quickly with corticosteroid and intravenous immunoglobulin treatment. To
our knowledge, this is the first reported case of symptomatic tongue myositis occurring in the course of
polymyositis.
CASE REPORT
The patient was referred for consultation in
March 1999, at the age of 58 years, for fatigability due to
muscular weakness involving the shoulder and pelvic
girdles and the limbs. The symptoms had been worsening for 2 years. She was obese (height 1.60 meters,
weight 97 kg) and had diabetes mellitus, chronic obstructive pulmonary disease, and hypertension. Her treatment included insulin, captopril, and bambuterol. On
physical examination, a slight decrease in muscle
strength in all 4 limbs and girdles was noted. She had
pain on muscle pressure. No skin abnormalities were
observed. The patient reported that her tongue had
started to become abnormally large 6–12 months previously. The erythrocyte sedimentation rate was 4 mm/
hour. The creatinine phosphokinase (CPK) level was
elevated (2,086 IU; normal ⬍135). Antinuclear antibodies were weakly positive (1:80) by immunofluorescence
(IF) on rat liver, and negative by IF on HEp-2 cells. SSA,
SSB, RNP, PM-Scl, Mi-2, signal recognition particle,
and Jo-1 autoantibodies were not detected by immunodiffusion and immunoblot. Electromyography confirmed
a diffuse typical myositic process. Muscle biopsy revealed necrotic muscle fibers, regenerating fibers, and
an endo- and perimysial inflammatory infiltrate. Immunohistochemical analyses for dystrophins 1, 2, and 3
showed no deficiency. Electron microscopy revealed no
specific ultrastructural abnormality. The final diagnosis
was polymyositis meeting the Bohan and Peter criteria (5).
Treatment with methotrexate (10 mg/week) was
instituted. Fatigability improved slowly. In October
1999, the patient reported progressive dysarthria, frequent tongue-biting during mastication, dysphagia, and
noisy breathing. On physical examination, macroglossia
was noted (Figure 1) and the submental region appeared
indurated, as if the muscles of the region were hypertro-
Tongue myositis has been described to occur
rarely in the course of some inflammatory muscle diseases (1–4), but has not been reported in association
with polymyositis. Herein we describe the case of a
patient with polymyositis in whom macroglossia and
symptomatic tongue myositis developed. These symptoms were treated successfully with corticosteroids and
intravenous immunoglobulin (IVIG).
E. Chauvet, MD, L. Sailler, MD, M. Carreiro, MD, J. R.
Paoli, MD, P. Arrue, MD, L. Astudillo, MD, F. Oksmann, MD, M. B.
Delisle, MD, Ph. Arlet, MD: Centre Hospitalier Universitaire
Rangueil-Larrey, Toulouse Cedex, France.
Address correspondence and reprint requests to L. Sailler,
MD, Service de Médecine Interne, CHU Rangueil-Larrey, 24 Chemin
de Pouvourville, 31403 Toulouse Cedex, France. E-mail: sailler.l@chutoulouse.fr.
Submitted for publication February 8, 2002; accepted in
revised form June 18, 2002.
2762
TONGUE MYOSITIS ASSOCIATED WITH POLYMYOSITIS
2763
Figure 2. Magnetic resonance imaging of the tongue and oropharynx
(T1-weighted sequence). 1, Muscle hypertrophy in the floor of the
mouth. 2, Disappearance of the tongue vallecula. 3, Narrowing of the
upper airways.
Figure 1. Macroglossia in the patient.
phic. There was a slight deficit in proximal and distal
muscle strength. The CPK level was 1,642 IU. Blood
␣-glucosidase activity was normal. Captopril-induced
angioedema was suspected, and the captopril treatment
was stopped and prednisone treatment started at an
initial dosage of 0.7 mg/kg/day (70 mg/day) in November
1999. The oropharyngeal symptoms improved greatly
within a few days but the diabetes became difficult to
control, so prednisone was stopped in January 2000. At
this time, the CPK level was 194 IU. The oropharyngeal
symptoms worsened shortly after discontinuation of the
corticosteroid treatment, and prednisone was reinstituted at a moderately effective dosage of 20 mg/day.
In February 2000, computed tomography scanning and magnetic resonance imaging (MRI) (T1weighted, T2-weighted, and fat-suppressed sequences)
of the oropharynx revealed the presence of a general
and homogeneous hypertrophy of the tongue and of its
constrictive muscles, particularly the genioglossus, with a
strong attenuation of the tongue vallecula (Figure 2).
The upper airways were narrowed. There was no evidence of edematous or lipomatous infiltration. Hor-
mone levels (thyrotropin, growth hormone, and insulin
growth factor 1) were within normal ranges. Electromyography of the tongue and submental muscles revealed
signs of a diffuse myogenic process. Tongue biopsy was
performed and showed a multifocal inflammatory infiltrate with septal and endomysial fibrosis (Figure 3).
Congo red staining of tongue, muscle, bone, salivary
gland, and skin biopsy specimens showed no evidence of
amyloidosis.
IVIG was given for 2 days (2 gm/kg/day). The
oropharyngeal symptoms resolved in the week following
the IVIG infusions, but recurred 3 weeks later. Six
Figure 3. Specimen from a tongue biopsy, showing 1, a multifocal
inflammatory infiltrate and 2, septal and endomysial fibrosis.
2764
CHAUVET ET AL
monthly infusions were prescribed and showed persistent efficacy, and corticosteroid treatment was gradually
tapered. Since November 2000, the patient has been
treated with azathioprine and low-dose prednisone (7.5
mg/day in April 2002). She feels well. The macroglossia
persists, but with no associated symptoms.
DISCUSSION
We have described the case of a patient who
presented with polymyositis, symptomatic macroglossia,
and tongue myositis. The diagnosis of polymyositis was
certain according to the Bohan and Peter criteria (5).
The most common causes of macroglossia (amyloidosis,
myxedema, acromegaly) were ruled out, and tongue
angioedema due to captopril (6) was excluded after this
treatment was stopped without any beneficial effect.
Both tongue electromyography and tongue biopsy confirmed the myositic process, and macroglossia-related
symptoms were clearly responsive to corticosteroids
initially, and then to intravenous immunoglobulin.
Macroglossia in association with muscle diseases
is rare. It has been described to occur in some genetic
disorders (Duchenne’s and Becker’s dystrophies,
Pompe’s disease, and C283Y mutation sarcoglycanopathy) (7–9). Duchenne’s and Becker’s dystrophies were
excluded in our patient since we did not find any
dystrophin abnormality. Blood ␣-glucosidase activity
was normal, and neither MRI nor biopsy showed the
fatty and fibrous involution of the tongue characteristic
of Pompe’s disease. C283Y mutation is a sarcoglycanopathy occurring exclusively in young gypsies (9).
Another condition to be considered in the differential diagnosis is carcinoma of the tongue, which can
simulate focal tongue myositis (10,11) or cause dermatomyositis (12). Therefore, the need for muscle biopsy
must be emphasized in patients presenting with inflammatory muscle disease and suspected to have tongue
myositis, in order to exclude the possibility of a neoplastic disorder.
In our patient, there was a question as to whether
the macroglossia was due solely to polymyositis. She had
never noted macroglossia before the disease onset. At an
early stage, polymyositis sometimes results in muscle
hypertrophy (1), and muscle hypertrophy in the floor of
the mouth was revealed by the MRI in this case. Imaging
showed no signal suggesting muscle inflammation, however, perhaps because the patient had been taking
prednisone at 20 mg/day for 2 weeks at that time. In
addition, the symptoms improved very quickly with
corticosteroid and IVIG treatment. However, the
tongue volume did not decrease. Therefore, the oropharyngeal symptoms in this patient were probably due
mainly to the hypomotility of the tongue caused by the
myositic process, and not to the macroglossia itself.
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symptomatic, intravenous, tongue, immunoglobulin, myositis, corticosterone, polymyositistreatment, macroglossia
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