A Primary Sjögren`s Syndrome Patient Presented with Severe

Case
Report
JOMP
pISSN 2288-9272 eISSN 2383-8493
J Oral Med Pain 2015;40(3):130-134
http://dx.doi.org/10.14476/jomp.2015.40.3.130
Journal of Oral Medicine and Pain
A Primary Sjögren’
s Syndrome Patient Presented with
Severe General Toothache
Yeon-Hee Lee, Hong-Seop Kho
Department of Oral Medicine and Oral Diagnosis, School of Dentistry and Dental Research Institute,
Seoul National University, Seoul, Korea
Received August 10, 2015
Revised September 1, 2015
Accepted September 2, 2015
Correspondence to:
Hong-Seop Kho
Department of Oral Medicine and
Oral Diagnosis, School of Dentistry
and Dental Research Institute, Seoul
National University, 101 Daehak-ro,
Jongno-gu, Seoul 03080, Korea
Tel: +82-2-2072-3989
Fax: +82-2-744-9135
E-mail: hkho@snu.ac.kr
This work was supported by the
National Research Foundation of Korea
Grant through the Oromaxillofacial
Dysfunction Research Center for the
Elderly (No. 2014-050477) at Seoul
National University in Korea.
Sjögren’s syndrome (SS) is an autoimmune disease characterized by an autoimmune exocrinopathy involving mainly salivary and lacrimal glands. Apart from manifestations due to
involvement of exocrine glands, patients with SS can present with muscular and neurological
manifestations. Here, we report a rare case of a 59-year-old woman with primary SS, who presented with severe general toothache and masticatory muscle myalgia successfully treated with
clonazepam. Although it was not certain that these symptoms could be originated from focal
muscle dystonia or neurological changes that are associated with primary SS, our case suggested that comprehensive evaluation including neuromuscular examinations in the oral and
maxillofacial area is needed in patients with SS.
Key Words: Autoimmunity; Clonazepam; Myalgia; Neuropathy; Sjogren’s syndrome; Toothache
myopathy of other parts of the body have been reported.3,4)
INTRODUCTION
Diverse types of muscle involvements have been reported
Sjögren’s syndrome (SS) is an autoimmune disease, char-
in patients with primary SS. Myalgia and muscular weak-
acterized by autoantibody secretion, lymphocytic infiltra-
ness symptoms are very common complaints and appear
tion, and destruction of salivary and lacrimal glands lead-
about one-third of primary SS patients.5) According to re-
ing to xerostomia and xerophthalmia. SS is the second most
cent studies, neurologic involvements occur with a high
common autoimmune rheumatic disease following rheuma-
frequency of 10% to 60% in patients with primary SS, and
toid arthritis and can be encountered alone (primary SS) or
may affect both the central nervous system (CNS) and the
in association with other autoimmune diseases (secondary
peripheral nervous system (PNS).6,7) Orofacial dystonia,
SS).1) In addition to significant discomfort from oral dryness
which involves an uncontrolled spasmodic contraction of
itself, dry mouth may also lead to various oral complica-
facial and masticatory muscles, has been reported in SS.8) In
tions such as increased dental caries, oral burning sensa-
the present report, we described a case of a primary SS pa-
tion, bacterial sialadenitis, oral candidiasis, and oral mal-
tient who presented with severe general toothache, which
odor. Although few studies have been reported on temporo-
might be caused by orofacial muscle hyperactivity and/or
2)
mandibular disorders (TMDs) in SS patients, pathologic in-
trigeminal neuropathy, and subsequently resolved almost
volvements of nervous system, myalgia, and inflammatory
completely after treatment with clonazepam. This study
Copyright Ⓒ 2015 Korean Academy of Orofacial Pain and Oral Medicine. All rights reserved.
CC This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/),
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www.journalomp.org
131
Yeon-Hee Lee et al. Sjögren’
s Syndrome with General Toothache
was approved by the Institutional Review Board of Seoul
included for complete blood counts with leukocyte differen-
National University Dental Hospital (#ER15001).
tial counts, erythrocyte sedimentation rate, blood glucose,
liver function tests (total protein, albumin, total bilirubin,
CASE REPORT
alkaline phosphatase, aspartate transaminase, alanine transaminase, and cholesterol), kidney function tests (blood urea
A 59-year-old woman visited the Department of Oral
nitrogen and creatinine), thyroid function tests (T3, free T4,
Medicine, Seoul National University Dental Hospital (Seoul,
and TSH), calcium, phosphorus, ferritin, vitamin B12, folate,
Korea) with severe general tooth pain which had suddenly
zinc, and magnesium levels. Aceclofenac, a non-steroidal
occurred 10 days prior to seeking treatments. Her toothache
anti-inflammatory analgesic drug, was prescribed for tooth
was continuous and spontaneous type of pain which oc-
pain for 5 days and questionnaires for orofacial pain and
curred in all teeth. The degree of severity was 8 in visual
dry mouth were given.
analogue scale (VAS; 0-10, with 10 being the worst pos-
In the 2nd visit, her tooth pain had partially decreased,
sible). She also had dry mouth and tongue pain which had
but persisted. The questionnaires showed that the severity
progressively increased for 4 to 5 years prior to presenta-
of her tongue pain was 9 in VAS and burning characteris-
tion. She told her tongue pain had begun with menopause.
tic increased as the day went. The aggravating factors were
She had history of visiting otolaryngology and oriental
spicy and hot food and talking. She had difficulties in eat-
medicine several times for treating dry mouth and tongue
ing and swallowing due to dry mouth and had taste distur-
pain, but the symptoms had persisted. Oral examination re-
bances. She had suffered from awakening from sleeping due
vealed erythema of the tongue and mild cheilitis of both
to dry mouth every day and she always needed water to
mouth corners (Fig. 1), but could not detect any causes of
swallow dry foods. She also reported dry eyes. Sialometry
her chief complaint, general tooth pain. Many teeth were
showed that the amount of unstimulated whole saliva col-
tender to percussion with various degrees. She had some
lected for 10 minutes was undetectable and the flow rate
level of generalized alveolar bone loss and calculus deposi-
of stimulated whole saliva using chewing a gum base as a
tion, but it could not explain her general tooth pain (Fig. 2).
stimulant was 0.08 mL/min. Psychometry using symptom
She was under estrogen replacement therapy for meno-
checklist-90 revision was within normal range. The results
pause and was taking multivitamin. She received university
of blood examinations were within normal range. The pos-
graduated level of education and was well communicable.
sibility of SS had to be evaluated. Blood tests including
She did not smoke and reported no alcohol consumption.
anti-SSa/anti-SSb, antinuclear antibody, rheumatoid factor,
The tentative diagnoses were dry mouth, atrophic can-
and C-reactive protein were performed. Artificial saliva and
didiasis, and burning mouth syndrome. Blood tests were
nystatin suspension were prescribed for 2 weeks.
At the 3rd visit, her tongue pain has decreased to almost
a half, but tooth pain has increased again and she reported
Fig. 1. Tongue atrophy and depapillation in the patient.
Fig. 2. Panoramic view in the patient.
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132
J Oral Med Pain Vol. 40 No. 3, September 2015
that she had felt a strange sensation on her teeth as well as
parafunctional habits such as bruxism and clenching.
tooth pain. In more detail, she had not felt that she had her
Conservative management was instituted with moist hot
own teeth in the right position. A habit of tooth clenching
pack, exercise, and control and monitoring of possible con-
was suspected and clonazepam 0.5 mg at bedtime was pre-
tributing factors such as parafunctional habits.
scribed for 2 weeks.
After dramatic improvement of general tooth pain in the
At the 4th visit, she reported that her tooth pain had de-
first 2 weeks of clonazepam administration, her symptom
creased to a half, but she felt dizziness after taking clonaz-
had been maintained and gradually decreased with 0.25 mg
epam. Her tongue pain was partially responsive to nystatin
clonazepam at bedtime, finally she did not need clonaz-
suspension but she had felt a little itchness of her skin. The
epam anymore after 1 year. She had been followed up for
results of her blood examinations had increased C-reactive
another 2 years. The patient was free of toothache and fa-
protein (1.07 mg/dL; normal value, 0 to 0.5 mg/dL) and
cial and masticatory muscle pain.
rheumatoid factor (15 IU/mL; normal value, <15 IU/mL),
and positive anti-SSa (>8.0 AI) and anti-SSb (>8.0 AI).
Serum antinuclear antibodies were also positive at titer 1:160
DISCUSSION
(speckled and discrete speckled pattern). She was prescribed
Above may be a unique case on a primary SS patient
artificial saliva, fluconazole 50 mg once a day, and clonaz-
whose initial chief complaint was a severe tooth pain with-
epam 0.25 mg at bedtime, and referred to rheumatology.
out overt causes in the teeth or periodontal tissues. While
From the 5th visit, her tongue pain was partially under
toothache usually shows classical remission when aceclof-
control by fluconazole 50 mg once a day as needed and ar-
enac as an analgesic administered, our patient did not ex-
tificial saliva and her tooth pain was partially under control
perience pain relief. Several factors may be responsible for
by clonazepam 0.25 to 0.5 mg at bedtime. She was referred
sudden toothache affecting several or all teeth. Without any
to ophthalmology by a rheumatologist and was confirmed
reported nocturnal parafunctional habits, the patient had
as a patient with primary SS in the rheumatology clinic by
myofascial pain on the masticatory muscles which can be
the American-European consensus criteria. She did not re-
one of the reasons of the pain. Many disorders in the head
ceive minor salivary gland biopsy. A diagnostic delay was
and neck region are known to refer pain to dental struc-
about 3 months after she visited with general tooth pain as
tures and imitate dental pain. Especially, myofascial pain is
a chief complaint. She was prescribed with hydroxychloro-
frequently associated with toothache of non-odontogenic
quine 200 mg twice a day in the rheumatology clinic.
origin referred to multiple teeth and alveolus, which causes
Although her symptoms had decreased, she had some
spontaneous and continuous pain of various levels of se-
stiffness in her jaw as well as tooth and gingival pain,
verity and quality.9,10) In 37.2% of patients with myofas-
therefore evaluation for TMDs was performed at 4 months
cial pain of the head and neck, the teeth were referred pain
after the first visit. She could open her mouth up to 47 mm
area.11) Simons et al.12) stated that trigger points in the mus-
of interincisal distance and had normal lateral range of the
cles of mastication, particularly in the masseter and tempo-
mandible movement without pain. She showed mandibu-
ralis, may refer pain to the maxillary and mandibular teeth.
lar deviation with an S-shape on mouth opening without
In our patient, both anterior parts of masseter and temporal
temporomandibular joint (TMJ) noise. She had pain on pal-
muscles, which were tender on palpation, could evoke the
pation of anterior parts of masseter and temporal muscles
symptoms. There have been few reliable studies on the effi-
bilaterally, but toothache was not reproduced at that time.
cacy of pharmacological therapy on the masticatory muscle
There was no pain on palpation of either TMJ capsular area.
pain. One randomized clinical study suggested that cyclo-
On panoramic and transcranial radiographs, no specific
benzaprine and clonazepam were effective for the manage-
condylar bony changes were detected. The patient was clin-
ment of jaw pain on awakening in myofascial pain patients
ically diagnosed with myofascial pain of the masticatory
and cyclobenzaprine was superior to clonazepam.13)
muscles. She told that she did not recognize any nocturnal
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Considering subjective xerophthalmia and xerostomia,
133
Yeon-Hee Lee et al. Sjögren’
s Syndrome with General Toothache
we had a tentative diagnosis with SS which was confirmed
comprehensive evaluation including neuromuscular exami-
with Schirmer’s test, sialometry, and blood test. While the
nations in the oral and maxillofacial area is needed in pa-
primary SS accompanied by masticatory muscle myalgia
tients with SS. Further studies on possible relationships be-
and/or myopathy has been rarely reported in dental litera-
tween SS and masticatory muscle pain and trigeminal neu-
ture, it has been reported in rheumatology fields that mus-
ropathy are needed.
cle pain, especially fibromyalgia, is common in primary SS
patients.14)
CONFLICT OF INTEREST
There has been only one case reported on orofacial dystonia in a patient with SS. The patient did not respond to
clonazepam and levetiracetam (an anticonvulsant). The patient was successfully treated with corticosteroids (1 g of
methylprednisolone intravenous for 3 days, which was followed by 48 mg of prednisone by oral administration daily
for several months).8) In our case, the muscular pain and
general toothache in the patient was successfully treated by
clonazepam. Benzodiazepines including clonazepam have
been used in patients with dystonia involving the arms, jaw,
and head and with musculoskeletal pains with various degrees of efficacy.15,16) Role of botulinum toxin injections has
also been extensively studied in focal dystonia and found
to be effective.15)
Neurological complications in primary SS are diverse and
both CNS and PNS can be involved. Infiltration of inflammatory cells into the nervous systems and vascular injuries
have been suggested as pathogenic mechanisms.6,7) Sensory
neuropathies including small fiber neuropathy are the predominant types of peripheral neuropathies in SS.17) Sensory
and motor involvements of trigeminal neuropathies have
been reported.18,19) Considering that the patient in our case
reported severe toothache in all teeth and her jaw pain itself was not a chief complaint, her toothache might be due
to mainly sensory neuropathy, but masticatory muscle pain
could contribute the symptoms. Because oral dystonia was
not observed in the patient, possibility of motor involvements of trigeminal neuropathies was low.
In conclusion, we reported a rare case of a patient with
primary SS, who presented with severe general toothache
and masticatory muscle myalgia successfully treated with
clonazepam. It was not certain that the masticatory muscle
pain and general toothache in the patient were associated
with pathophysiology of primary SS. However, considering
muscle pain and neurological involvements frequently reported in patients with primary SS, our case suggested that
No potential conflict of interest relevant to this article
was reported.
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