Long-Term, High-Dose Intravenous
Immunoglobulin Therapy in a Patient withBanker-Type Juvenile Dermatomyositis
George Imataka & Osamu Arisaka

Cell Biochemistry and Biophysics
ISSN 1085-9195
Volume 69
Number 3
Cell Biochem Biophys (2014) 69:747-748
DOI 10.1007/s12013-014-9833-7

1 23

Your article is published under the Creative
Commons Attribution license which allows
users to read, copy, distribute and makederivative works, as long as the author of
the original work is cited. You may selfarchive this article on your own website, aninstitutional repository or funder’s repository
and make it publicly available immediately.

1 23

Cell Biochem Biophys (2014) 69:747–748
DOI 10.1007/s12013-014-9833-7

TRANSLATIONAL BIOMEDICAL RESEARCH

Long-Term, High-Dose Intravenous Immunoglobulin Therapy
in a Patient with Banker-Type Juvenile Dermatomyositis
George Imataka • Osamu Arisaka

Published online: 6 February 2014
Ó The Author(s) 2014. This article is published with open access at Springerlink.com

Abstract Juvenile dermatomyositis (JDM) is a multisystem disease characterized by non-purulent inflammationin the striated muscle and skin. Banker-type JDM is difficult to treat and control, especially when it is a form that isresistant to steroid treatment. Here, we report a 2-year-oldgirl with Banker-type JDM resistant to steroid treatment.
The patient received intravenous immunoglobulin (IVIg) at
400 mg/kg/5 days a week every 6 weeks. Motor function
improved and the patient was able to walk after six cycles.
IVIg was administered every four weeks for six cycles
thereafter, and the patient was able to walk more quickly
with an improvement in quality of life. No apparent
adverse effects were observed during IVIg treatment.
Keywords Juvenile dermatomyositis  Steroid 
Immunoglobulin  Banker type

Introduction
Juvenile dermatomyositis (JDM) is an idiopathic, multisystem, collagen disease characterized by non-purulent
inflammation in the striated muscle and skin. Clinicalpathology differs from that of the adult type; there is alower frequency of malignant tumors and many cases
respond well to steroids. Clinically, JDM can be classified
into three subtypes: Banker, Brunsting, and fulminant [1].
There is no established therapy for steroid-resistantBanker-type JDM, and symptoms are very difficult toG. Imataka (&)  O. Arisaka
Department of Pediatrics, Dokkyo Medical University School ofMedicine, 880 Kitakobayashi, Mibu, Shimotsuga,Tochigi 321-0293, Japane-mail: geo@dokkyomed.ac.jp

control [1]. In the following case report, long-term, highdose therapy, with intravenous immunoglobulin (IVIg),
was performed in a 2-year-old girl with steroid-resistant,
Banker-type JDM. This is the first reported case of IVIg
treatment in a child as young as 2 years old.

Case report
A 2-year-old girl was brought to the hospital because of a
waddling gait with intermittent claudication. In addition,
she was not able to stand on one leg. Manual muscle testing(MMT) of the deltoid and quadriceps femoris resulted in a
grade of three, showing muscle weakness predominantly inthe proximal muscles. Also, Gower’s sign was positive.
Muscle tonus did not restrict the range of motion of joints,
and muscle mass was normal, but the gastrocnemius was
hard and painful to the touch. Erythema and heliotrope-likeexanthema were noted in the forehead. Upon neurophysiological examination, motor and sensory nerves weredeemed normal. Muscle CT showed partial fatty degeneration in the gastrocnemius. Blood test results were as follows: creatine phosphokinase (CPK) = 14,420 IU/l;
myoglobin = 777 ng/ml;
aspartateaminotransferase(AST) = 211 IU/l; antinuclear antibodies (ANA) = 5,120times; and negative for the Jo-1 antibody. Upon biceps
brachii muscle biopsy, necrosis, regeneration and irregular
sized myofibers, and angitis accompanied by perivascularlymphocyte infiltration were noted. JDM was diagnosed
based on various tests and the clinical course.
After the initial oral prednisolone (PSL) dose at 2 mg/kg/day, CPK decreased to about 1,000 IU/l after
1.5 months; however, glaucoma developed after several
months. Dual-energy X-ray absorptiometry index was 0.3.
The PSL dose was gradually reduced, and CPK increased

123

748

Cell Biochem Biophys (2014) 69:747–748

to more than 8,000 when the dose was reduced to 1 mg/kg/
day, indicating recurrence of condition. Clinically, thepatient became unable to turn over in bed or move her legs
against gravity. Muscle MRI showed fatty degeneration
and atrophic changes predominantly in the gastrocnemius.
Administration of IVIg at 400 mg/kg/5 days every
6 weeks was initiated, and CPK levels stabilized between
4,000 and 5,000 IU/l. Although CPK did not decrease,
motor function improved and the patient became able towalk after six cycles. IVIg was administered every fourweeks for six cycles thereafter, and the patient was able towalk more quickly with an improvement in her quality oflife. No apparent adverse effects were noted during IVIg
treatment.

steroid-resistant JDM [1, 2]. In a retrospective analysis byLam et al. [3] regarding IVIg therapy in patients with poor
response to first-line corticosteroid therapy, it was foundthat IVIG may be effective in controlling JDM symptoms.
However, no consensus has yet been reached on IVIg
dosing intervals and doses. Interestingly, the patient’s daily
physical activity levels increased with every 4 weeks ofIVIg therapy. To date, long-term IVIg therapy for steroidresistant JDM in a patient as young as two years of age has
never been reported. Although the results of hematology inthis case indicated that complete remission could not berealized, the results did suggest that IVIg therapy is usefulfor controlling steroid-resistant fulminant JDM in young
children.

Discussion

Open Access This article is distributed under the terms of theCreative Commons Attribution License which permits any use, distribution, and reproduction in any medium, provided the original
author(s) and the source are credited.

In the present case, standard steroid treatment initiallyexhibited an effect but the disease recurred during steroid
tapering therapy. Thus, a 12-cycle long-term, high-dose
IVIg was performed every 6 weeks for the first six cycles
and every 4 weeks for the subsequent six cycles, focusing
on immunological abnormalities in juvenile dermatomyositis. In our case, IVIg therapy resulted in clinicalimprovement in the patient’s muscle weakness. Subsequently, quality of life improved such that the patient wasable to walk unassisted. No apparent adverse effects werenoted during the course of IVIg therapy. IVIg therapy has
recently been reported to be effective in the treatment of123

References
1. Robinson, A. B., & Reed, A. M. (2011). Clinical features,
pathogenesis and treatment of juvenile and adult dermatomyositis.
Nature Reviews Rheumatology, 7, 664–675.
2. Amano, H., Nagai, Y., Katada, K., Hashimoto, C., & Ishikawa, O.
(2007). Successful treatment of cutaneous lesions in juvenile
dermatomyositis with high-dose intravenous immunoglobulin. The
British journal of dermatology, 156, 1390–1392.
3. Lam, C. G., Manlhiot, C., Pullenayegum, E. M., & Feldman, B. M.
(2011). Efficacy of intravenous Ig therapy in juvenile dermatomyositis. Annals of the Rheumatic Diseases, 70, 2089–2094.