25
Case
Report
Severe
Retinopathy
in Fulminant
Juvenile
Dermatomyositis
Naoki Gushiken, MD', Hiroshi Yoshimura, MD2, Makiko Toyoura, MD2, Naoki Fujiwara, MD2, Yasuharu Tokuda, MD, MPH, FACP3 Department of 'Ophthalmology, Department of 2Pediatrics, Okinawa Chubu Hospital 3Clinical Practice Evaluation and Research Center , St. Luke's Life Science Institute
;
ABSTRACT: We present a case of juvenile type dermatomyositis and severe retinopathy. A l0-year-old girl presented with progressive weakness of proximal muscles, generalized rash, including heliotrope-type eyelid erythema, and bilateral visual disturbance. Laboratory data showed a markedly elevated serum creatine kinase and electromyography revealed a myogenic pathology. Funduscopic examination showed numerous cotton wool spots and macular edema. She developed massive rhabdomyolysis, generalized skin lesions, systemic edema, renal failure, and respiratory failure. After she received steroid pulse therapy, plasma exchange, and high-dose immunoglobulin, her general condition and visual symptoms improved. She remained well when we followed up her condition 5 years after the discharge. KEY
WORDS
Gen Med
: 2007
he causes
T
of retinal
of
systemic
ed with retinal and systemic using
retinal
may result
a 10-year-old plasma
immunoglobulin
systemic disease
Retinal
vasculi-
Since
the first
is rare.
retinopathy
However,
and may also precede
CASE
as Behcet's
lupus erythematosus.
with dermatomyositis
treated
; Retinal
by Bruce in 1938,' there have only been a
reports
dermatomyositis.2'3
experienced
include
such
with dermatomyositis
case was described case
vasculitis
diseases,
and systemic
; Retinopathy
vasculitis
in permanent
complications.4'5
girl of dermatomyositis vasculitis.
exchange
in addition
associated
with
in children vision
loss
We recently associat-
She was successfully
and intravenous
high-dose
to corticosteroid
administration.
girl presented
to the pediatric
DESCRIPTION
A 10-year-old
Vasculitis
; Rhabdomyolysis
; Plasma
Exchange
; 8 : 25-28
inflammatory
tis associated few
: Dermatomyositis
Japanese
department with 4 weeks history of progressive weakness and generalized rash. She described severe weakness on proximal muscles and could not stand up by herself so that she had to climb up and down stairs in her home in a squatting position. She had no joint pain and no mucosal lesions. Her past medical history was not significant. She denied use of any medications or oral supplements. On physical examination at admission, her vital signs were normal. She had no cardiac murmurs and her lungs were clear to auscultation. She had pathognomonic skin lesions for dermatomyositis, including heliotrope-type eyelid erythema and symmetric violaceous scaly papules on dorsal areas of metacarpophalangeal and proximal interphalangeal joints. Proximal-dominant symmetrical muscular weakness was noted at grade 3/5 in the rectus abdominis muscle, arms (deltoid, biceps and triceps muscles), and legs (quadriceps, iliopsoas and hamstring muscles).
Author for Correspondence : Yasuharu Tokuda, MD, MPH Clinical Practice Evaluation and Research Center St. Luke's Life Science Institute 9-1 Akashi-cho, Chuo-city, Tokyo 104-8560 Japan Phone & FAX+81-3-5550-2426 E-mail :
[email protected] Received for publication : April 8, 2007 Accepted for publication : September 18, 2007
26
General
Medicine.
Vol.
8. No.
Table
FIgure
1.
Photographs
1.
1. 2007
Admission
of numerous
laboratory
cotton-wool
data
spots
and
macular
edema
of fundus
Laboratory data on admission are shown in Table 1. In addition to mild leukopenia and thrombocytopenia, there was
day). However, during the course developed bilateral visual blurring
an elevated concentration of serum creatine kinase, alanine aminotransferase, aspartate aminotransferase, lactate dehy-
consultation was obtained. The visual acuity was 20/40 in both eyes. Examination of the fundus revealed numerous
drogenase, and alkaline phosphatase. Urinalysis and blood coagulation studies were normal. While she had positive
cotton wool spots around the fovea and macular edema in both eyes and these findings were consistent with retinal
antinuclear antibody of titer 160 : I, antibodies against Sm, DNA, RNP. and Jol were negative. Chest radiography and electrocardiography showed no abnormality.
vasculitis (Figure 1). Despite corticosteroid pulse therapy
Although muscle biopsy was not performed, electromyography showed a myogenic pattern of pathology. After she received a diagnosis of juvenile type dermatomyositis, she was given prednisolone therapy
(40 mg per
of her illness, she and ophthalmologic
(intravenous methyl-
prednisolone I g per day for 3 consecutive days), she developed fever, severe generalized muscular pain with rhabdomyolysis, massive edema, renal failure, and diffuse erythernatous skin lesions, requiring admission to intensive care unit and tracheal intubation for respiratory compromise.
Gushiken
FIgure
2.
Photographs
of pan-retinal
photocoagulation
encircling
N. et al. Retinopathy
the
macula
in dermatomyositis
27
of fundus
Based on the diagnosis of retinal and systemic vasculitis associated with fulminant dermatomyositis, we performed courses of plasma exchange along with intravenous highdose immunoglobulin therapy. Multiple sessions of continuous hemodiafiltration were also provided for managing acute renal failure. Following these treatments, her general condition and visual acuity improved. Three months later. fundus examination revealed the resolution of cotton wool spots and retinal hemorrhage, although there was marked dilatation and tortuous deformity of the retinal
veins (Figure
2). Fluorescein
angiogram
showed an extensive non-perfusion area in the posterior fundus and leakage from optic disk neovascularization. Panretinal photocoagulation was performed for each eye to prevent retinal proliferation. The regression of neovascularization and the improvement of the fundus finding were noted
FIgure
3.
Course
of
illness
of
the
patient
during
hospitalization CPK=creatine sultation
phosphokinase
: SP=steroid
pulse
: OC=ophthalmologic therapy
: PE=plasma
conex-
change
after photocoagulation therapy. The course of her illness during hospitalization is shown in Figure 3. When we follow up her condition 5 years after the discharge, she remained well and her visual acuity was acceptable.
retinal vasculitis can be based on characteristic ophthalmologic findings along with systemic inflammatory signs. Differential diagnosis for clinical problems of our patient may include Behcet's disease, systemic lupus erythematosus,
DISCUSSION
Children with dermatomyositis may develop severe retinal and systemic vasculitis. Retinopathy is relatively rare in
and sarcoidosis, in addition to dermatornyositis. However, she had no mucosal and genital lesions for possible Behcet s disease, no joint involvement and serum antibodies to Sm
patients with dermatomyositis and cotton wool spots in fundoscopic examination are also unusual findings in these
and DNA antigens for possible systemic lupus erythematosus, and no lymphadenopathy and pulmonary granuloma for
patients.' However, there have been reports indicating cases of dermatomyositis with severe retinopathy, as well as
possible sarcoidosis. Further, she did present with pathognomonic skin lesions for dermatomyositis at admission, such as
rapidly progressive life-threatening systemic complications. Although diagnostic confirmation of vasculitis usually needs
eyelid erythema known as heliotrope rash and a scaly and
a histological proof of vascular inflammation,
diagnosis of
patchy violaceous erythema over the knuckles known as Gottron's sign. Moreover, her clinical findings met diagnostic
28
General
Medicine,
Vol. 8, No. 1, 2007
criteria proposed both by Japanese Health Ministry Research Group (1992) and Bohan et al (year 1972 criteria) for the diagnosis of dermatomyositis.6 Plasma exchange and intravenous high-dose immunoglobulin may be an effective treatment for patients who are refractory to steroid therapy.' For patients with dermatomyositis and visual disturbances, prompt and careful ophthalmologic evaluation should be considered, in addition to estimating and managing systemic complications of the disease. Aggressive treatments, including high dose corticosteroids, plasma exchange, and high dose immunoglobulin, may be needed for the successful treatment of severe vasculitis associated with fulminant dermatomyositis. One report suggested an additional use of cyclophosphamide for refractory cases.4 Together with pediatricians or generalist physicians, ophthalmologists should also have an important role in managing these patients.
References 1.
2.
Bruce
G.
Retinitis
Ophthalmol
Soc
De
S.
Vries
Ophthalmol 3.
Chu-Lee
Trans
Am
282-92. in
dermatomyositis.
Arch
432-5. G,
Jaffe
J Rheumatol
Yeo LM, Swaby visual
46:
Stroller
dermatomyositis. 4.
dermatomyositis..
36:
Retinopathy
1951; A,
in
1938;
IA.
Retinopathy
1995;
DS, Situnayake
loss in dermatomyositis.
22:
in
adult
2372-3.
RD, Murray
PL Irreversible
Br J Rheumatol
1995;
34:
1179-81. 5.
Backhouse O, Ophthalmic Dis
6.
1998;
Mastaglia thies:
7.
Griffiths
B,
manifestations 57:
Henderson
Emery
P.
Ann Rheum
447-9.
FL, Phillips
epidemiology,
BA. Idiopathic classification,
Rheum
Dis Clin North
Am 2002;
Cohen
BH,
LA,
dermatomyositis.
T,
of dermatomyositis.
Sedwick
inflammatory
myopa-
and diagnostic
criteria.
28:
Burde
J Clin Neuroophthalmol
723-41.
RM.
Retinopathy 1985;
5:
of 177-9.
