A 46 year old woman presented with a one month history of rash and mylagia. The history, clinical findings and blood tests all supported a diagnosis of dermatomyositis. The patient later developed dysphagia and was successfully treated with intravenous immunoglobulin. Investigations and treatment of dysphagia in the context of dermatomyositis are discussed

Joshi, Deepak

Kitchen, Paul

Mahmood, Rizwan

Williams, Peter

English

PubMed

Springer - Publisher Connector

BioMed Central
International Archives of Medicine
ssOpen AcceCase report
Dysphagia secondary to dermatomyositis treated successfully with 
intravenous immunoglobulin: a case report
Deepak Joshi*1, Rizwan Mahmood1, Peter Williams2 and Paul Kitchen1
Address: 1Department of Gastroenterology, Medway Maritime Hospital, Kent, UK and 2Department of Rheumatology, Medway Maritime Hospital, 
Kent, UK
Email: Deepak Joshi* - djosh78@hotmail.com; Rizwan Mahmood - rizwan.mahmood@kch.nhs.uk; 
Peter Williams - peter.williams@medway.nhs.uk; Paul Kitchen - paul.kitchen@medway.nhs.uk
* Corresponding author    
Abstract
A 46 year old woman presented with a one month history of rash and mylagia. The history, clinical
findings and blood tests all supported a diagnosis of dermatomyositis. The patient later developed
dysphagia and was successfully treated with intravenous immunoglobulin. Investigations and
treatment of dysphagia in the context of dermatomyositis are discussed.
Background
Dermatomyositis commomly presents with fatigue, prox-
imal weakness, muscle tenderness and a characteristic
rash. It can occur in conjunction with an underlying
malignancy. Dysphagia can also occur and is associated
with a 31% one year mortality [1]. The authors present a
case of dermatomyositis in a 46 year old woman who
developed dysphagia and was successfully treated with
intravenous immunoglobulin.
Case report
A 46 year old caucasian female presented with a one
month history of a rash on the face and hands. She had
also noticed generalised myalgia. There was no history of
dysphagia, weight loss or night sweats. There had been no
recent foreign travel. A past medical history of pernicious
anaemia, treated with hydroxycobalamin injections, was
noted. No other regular medications were being taken or
had been started in the last month. Cardio-respiratory and
abdominal examination was unremarkable. Power in all
four limbs was graded MRC 5/5. A helitropic rash affect-
ing both upper eyelids and a scaly violaceous eruption on
the knuckles was noted. The full blood count, renal func-
tion and coagulation studies were normal. Serum B12 and
folate levels were also normal. Serum creatine kinase (CK)
was 9831 U/L (NR 20–180) and ESR was 54 mm/h (NR
0–20).
The history, clinical findings and blood tests all supported
a diagnosis of dermatomyositis and therefore high dose
prednisolone (60 mg OD) and methotrexate 10 mg per
week with folic acid supplementation was commenced.
The patient underwent a deltoid muscle biopsy which
demonstrated patchy atrophy of fibres and inflammation
around the fasicles compatible with dermatomyositis.
Computed tomography of the abdomen, chest and pelvis
was normal. Breast mammography revealed a normal
parenchymal pattern with no features supportive of
malignancy. Auto-antibodies including ANA and anti Jo 1
were negative. The patient was discharged 12 days later
with significant improvement in symptoms. The CK con-
tinued to normalise 1888 U/L.
The patient was readmitted 21 days later with worsening
dysphagia. Upper gastro-intestinal endoscopy demon-
strated poor primary contractions with swallowing. Fibro-
Published: 23 July 2008
International Archives of Medicine 2008, 1:12 doi:10.1186/1755-7682-1-12
Received: 16 March 2008
Accepted: 23 July 2008
This article is available from: http://www.intarchmed.com/content/1/1/12
© 2008 Joshi et al; licensee BioMed Central Ltd. 
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), 
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.Page 1 of 3
(page number not for citation purposes)
International Archives of Medicine 2008, 1:12 http://www.intarchmed.com/content/1/1/12optic endoscopic evaluation of swallowing highlighted
velo-pharyngeal dysfunction, inadequate clearance of
bolus from the pharynx and evidence of residue in the val-
leculae and piriform fossa. Oesophageal and gastric tran-
sit studies confirmed oesophageal dysmotility affecting
the medial and lower third of the oesophagus and mild
gastric stasis. The prednisolone was increased to 60 mg.
Given the findings of oesophageal and gastric delay, Intra-
venous immunoglobulin (IV IG) 40 g OD for three days
was given, with a significant improvement in symptoms.
Further IV IG was deemed unnecessary due to a paucity of
symptoms. The patient was continued on prednisolone
60 mg for a further four weeks before being reduced and
the methotrexate was increased to 15 mg per week. The
patient continued to be followed up by both the gastroen-
terologists and rheumatologists. One year after her initial
presentation, the patient described no dysphagia. Repeat
fibro-optic endoscopy and oesophageal transit studies
were not repeated given the resolution of symptoms.
Discussion
The idiopathic inflammatory myopathies are divided into
four subgroups: dermatomyositis (DM), polymyositis
(PM), inclusion body myositis (IBM) and an overlap syn-
drome with mixed characteristics [2]. DM is a comple-
ment mediated microangiopathy affecting the skin and
muscles [3]. Endocapillary deposition of the complement
C5b-9 membraneolytic attack complex leads to the loss of
capillaries, ischaemia and necrosis of muscle fibres [4].
The characteristic sign associated with DM is a heliotrope
(blue-purple) rash of the upper eyelids. A flat, red rash on
the face (often associated with periorbital oedema) and
upper trunk is also often noted. In the hands, Gottron's
papules (raised violaceous scaly eruption on the knuck-
les) and periungal erythema may be found. DM is associ-
ated with an occult malignancy in 20–30% of cases
especially colorectal, bladder, lung, non-Hodgkin's lym-
phoma, ovarian, pancreatic and stomach cancers. The risk
for developing a cancer remains for up to five years [5]. A
poorer prognosis is associated with old age, non-white
race, occurrence with an underlying malignancy, bulbar
involvement, cardio-respiratory involvement and delayed
treatment [6].
An incidence of 5–10 cases per 100,000 individuals has
been reported, affecting women more commonly with a
ratio of 2:1. Adults and children are equally affected, with
the peak incidence between the ages of 45 and 64 in
adults. Clinical features include fatigue, proximal muscle
weakness and muscle tenderness. Dysphonia, dysphagia
and breathlessness can all occur if the respective muscle
groups are affected. Extra-muscular manifestations
include fever, Raynaud's phenomenon, subcutaneous cal-
cifications and arthralgia. Subcutaneous calcifications are
rare in adults [5]. The characteristic rash, previously
described, can occur over the shoulders, upper arm and
back resulting in the termed "shawl sign". Investigations
should include full blood count, urea and electrolytes,
liver function tests (transaminases may be raised), creat-
ine kinase (CK) and a chest radiograph. An ECG, echocar-
diogram and pulmonary function tests should be
performed if cardio-respiratory involvement is suspected.
Anti-nuclear antibodies (ANA) are positive in 80% of
patients, whilst anti Jo-1 antibodies are positive in
approximately 20% [5]. CK levels are used to monitor dis-
ease activity but levels may have poor correlation with
clinical disability [6]. It is imperative that patients are
screened for an occult malignancy with investigations that
include computed tomography of the abdomen, chest
and pelvis, mammography and serum tumour markers.
MRI has been used to successfully to identify myositis of
affected muscle groups, as muscle oedema correlates well
with inflammatory changes. A muscle biopsy is still
required to make the diagnosis. Classical findings include
endomysial inflammation predominant in the perivascu-
lar regions or in the interfascicular septae, and around the
fascicles [3].
Principal treatment for DM is corticosteroids. Steroid
resistant disease is not uncommon, and methotrexate,
azathioprine, cyclosporine and cyclophosphamide have
all been used successfully. However a Cochrane Review
2005 [7] demonstrated a lack of high quality randomised
controlled trials that have assessed the efficacy and toxic-
ity of immunosuppression in the inflammatory myopa-
thies. Intravenous Immunoglobulin (IV IG) has been used
in patients refractory to steroid sparing treatment. The
mechanisms of action are not clear but it is known to
block the Fc receptors on the vascular wall [4] thus pre-
venting the membrane attack complex deposits from
entering endomysial capillaries. IV IG has been to shown
to have positive effect on myopathy [3] and on cutaneous
ulceration [8]. The dose recommended in the literature is
2 g/kg, to be repeated in six weeks depending upon clini-
cal response. Side effects are mild and include nausea and
headaches.
Dysphagia occurs in 10–73% of patients with an inflam-
matory myopathy [9]. The skeletal muscle activated-
oropharyngeal phase of swallowing is clearly affected
leading to the increased incidence of aspiration pneumo-
nia [2]. Horowitz et al [10] showed a high incidence of
gastric and oesophageal motor dysfunction particularly in
DM and PM, therefore suggesting that smooth muscle is
also affected. This leads to ineffective peristalsis and clini-
cal manifestation with gastro-oesophageal reflux, gas-
troparesis and constipation due to colonic inertia [11]. A
multi-disciplinary approach is essential, with the roles of
dieticians and speech and language therapists being
imperative. Assessment with video-fluoroscopic studiesPage 2 of 3
(page number not for citation purposes)
International Archives of Medicine 2008, 1:12 http://www.intarchmed.com/content/1/1/12Publish with BioMed Central   and  every 
scientist can read your work free of charge
"BioMed Central will be the most significant development for 
disseminating the results of biomedical research in our lifetime."
Sir Paul Nurse, Cancer Research UK
Your research papers will be:
available free of charge to the entire biomedical community
peer reviewed and published immediately upon acceptance
cited in PubMed and archived on PubMed Central 
yours — you keep the copyright
Submit your manuscript here:
http://www.biomedcentral.com/info/publishing_adv.asp
BioMedcentral
can help identify pharyngeal pooling, impaired tongue
base retraction, decreased laryngeal elevation and
cricopharyngeal dysfunction. Oesophageal and gastric
radionuclide transit studies are a safe, simple, non inva-
sive method of highlighting oesophageal and gastric
delay. Treatment options include botulinum injection,
cricopharyneal dilatation and myotomy. Swallowing
compensation techniques also have an important role to
play. PEG tube insertion may be required if swallowing
remains unsafe but the highest mortality has been
described in these patients [2]. Conventional immuno-
suppressive treatment is recommended but the treatment
of choice is IV IG [12].
Conclusion
Dysphagia commonly occurs in DM and can occur due to
oropharyngeal skeletal muscle or distal oesophageal and
gastric smooth muscle involvement. Malignancy needs to
be excluded. Investigations should include OGD, video
fluoroscopy and oesophageal and gastric transit studies.
The treatment of choice is IV IG.
Competing interests
The authors declare that they have no competing interests.
Consent
Written consent was obtained from the patient prior to
submission.
Authors' contributions
DJ and RM involved in writing of the case report. PW and
PK were involved in the review and re-writing of the case
report.
References
1. Williams R, Grehan M, Hersch M, Andre J, Cook I: Biomechanics,
diagnosis and treatment outcome in inflammatory myopa-
thies presenting as oropharyngeal dysphagia.  Gut 2003,
52:471-478.
2. Oh T, Brumfield K, Hoskin T, Stolp KA, Murray JA, Bassford JR: Dys-
phagia in inflammatory myopathy: clinical characteristics,
treatment strategies and outcome in 62 patients.  Mayo Clinic
Proceedings 2007, 82(4):441-447.
3. Dalakas M: The role of high dose immune globulin intravenous
in the treatment of dermatomyositis.  International Immunophar-
macology 2006, 6:550-556.
4. Dalakas M, Illa I, Dambrosia J: A controlled trial of high-dose
intravenous Immune Globulin infusions as treatment for
dermatomyositis.  N Engl J Med 1993, 329:1993-2000.
5. Behan W, Sturrock R: Idiopathic inflammatory myopathies.
Reports on the rheumatic diseases series 5; topical reviews 2004, 2:1-8.
6. Cordeiro A, Isenberg D: Treatment of inflammatory myopa-
thies.  Postgrad Med J 2006, 82(969):417-424.
7. Choy E, Hoogendijk J, Lecky B, Winer JB: Immunosuppresants
and immunomodulatory treatment for dermatomyositis
and polymyositis.  Cochrane Database Syst Rev 2005:CD003643.
8. Kuwano Y, Ihn H, Yazowa N, Kakinuma T, Asahina A, Kikuchi K, Tam-
aki K: Successful treatment of dermatomysositis with high
dose intravenous immune globulin.  Acta Derm Venereol 2006,
86:158-159.
9. Maugars Y, Berthelot J, Abbas Mussini JM, Nguyen JM, Prost AM:
Long term prognosis of 69 patients with dermatomyositis or
polymyositis.  Clin Exp Rheumatol 1996, 14:263-274.
10. Horrowitz M, McNeil J, Maddern G, Collins PJ, Shearman DJ: Abnor-
malities of gastric and oesophageal emptying in polymyositis
and dermatomyositis.  Gastroenterology 1986, 90:434-439.
11. Spiera R, Kager L: Extra muscular manifestations in idiopathic
inflammatory myopathies.  Curr Opin Rheumatol 1998,
10:556-561.
12. Kiely P, Heron C, Bruckner F: Presentation and management of
idiopathic inflammatory muscle disease: four case reports
and commentary from a series of 78 patients.  Rheumatology
2003, 42:575-582.Page 3 of 3
(page number not for citation purposes)


Dysphagia secondary to dermatomyositis treated successfully with intravenous immunoglobulin: a case report

Deepak Joshi

Rizwan Mahmood

Peter Williams

Paul Kitchen

Crossref

A controlled trial of high-dose intravenous Immune Globulin infusions as treatment for dermatomyositis.

AM: Long term prognosis of 69 patients with dermatomyositis or polymyositis. Clin Exp Rheumatol

DJ: Abnormalities of gastric and oesophageal emptying in polymyositis and dermatomyositis. Gastroenterology

Extra muscular manifestations in idiopathic inflammatory myopathies. Curr Opin Rheumatol

I: Biomechanics, diagnosis and treatment outcome in inflammatory myopathies presenting as oropharyngeal dysphagia. Gut

Idiopathic inflammatory myopathies. Reports on the rheumatic diseases series 5; topical reviews

Immunosuppresants and immunomodulatory treatment for dermatomyositis and polymyositis. Cochrane Database Syst Rev

Isenberg D: Treatment of inflammatory myopathies.

JR: Dysphagia in inflammatory myopathy: clinical characteristics, treatment strategies and outcome in 62 patients. Mayo Clinic Proceedings

Presentation and management of idiopathic inflammatory muscle disease: four case reports and commentary from a series of 78 patients. Rheumatology

Tamaki K: Successful treatment of dermatomysositis with high dose intravenous immune globulin. Acta Derm Venereol

The role of high dose immune globulin intravenous in the treatment of dermatomyositis. International Immunopharmacology

http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=2516510

Dysphagia secondary to dermatomyositis treated successfully with intravenous immunoglobulin: a case report

Abstract

Similar works

Full text

Available Versions

Springer - Publisher Connector

Springer - Publisher Connector

Crossref