Currently, there are no specific diagnostic investigations for systemic juvenile idiopathic arthritis
（s-JIA）. Various investigations are considered when making a differential diagnosis. During the 5th
Pediatric Rheumatology Workshop held in 2017, we interviewed 17 Japanese pediatric rheumatology experts
about the investigations they conduct in the initial diagnostic process for s-JIA. Bone marrow aspiration was
preferred by 82％ of experts. Routine use of joint magnetic resonance imaging and measurement of serum
IL-18 for an initial diagnostic approach appeared to be controversial. Fluorodeoxyglucose-positron emission
tomography or Gallium scintigraphy were not frequently conducted. Although each investigation was known
to play a certain role in the diagnosis of s-JIA, they were often not conducted for reasons such as radiation
exposure, invasiveness, availability of the investigation in that hospital, and health insurance coverage. In
addition, investigations necessary for diagnosis should be selected on an individual basis because patients
with s-JIA show diverse initial manifestations. There was consensus among the experts that exclusion of
malignancy is important when diagnosing s-JIA； therefore, selected investigations should be performed
To clarify factors associating with transition readiness in youth/young adults with childhood-onset
rheumatic diseases（ coRD） and their parents/caregivers.
We enrolled 28 patients with coRD aged 10 years and older and their parents/caregivers. We evaluated
transition readiness both in youth/young adults with coRD and in their parents/caregivers by using
Transition readiness checklist. Mental disorders and overprotection of parents/caregivers were subjectively
evaluated by their attending doctors.
We did not find any association between achievement score of transition readiness in youth/young adults with
coRD and gender, primary diseases, age at the onset of primary disease, duration of primary disease, or age at
the enrollment. We found that both achievement score of transition readiness in youth/young adults with coRD
and that in parents/caregivers were significantly lower in subjects with overprotection of parents/caregivers.
Overprotection of parents/caregivers may be an obstructive factor for transition readiness in youth/young
adults with childhood-onset rheumatic diseases and their parents/caregivers.
Introduction : Methotrexate（ MTX） is the only disease modifying anti-rheumatic drugs（ DMARDs）
that is approved for juvenile idiopathic arthritis（ JIA） in Japan. Therefor, the patients who had an adverse
event for MTX have been taken into consideration of using biological agents.
Purpose : We evaluated retrospectively the association between dose of MTX, frequency of adverse
events, and introduction of biological agents in articulated JIA.
Results : Forty-six patients with JIA were included in this study. The mean age at evaluation was 17 years
and mean duration from illness onset was 10 years. Adverse events were observed in 23 patients（ 50%）, and the
most common symptom was nausea. The patients who experienced adverse events（ n = 23） received significant
higher dose of MTX compared to the patients who did not experienced adverse events（ n = 22）（ 2.6 vs. 7.4 mg/
m2/week ; p =0.0029）, and moreover the patients having MTX tolerance received more biological agents than
having MTX intolerance（ 83 vs. 50% ; p =0.0029）.
Conclusion: Although MTX is an effective for the patients with JIA, the patients frequently experienced
adverse events. There are no other DMARDs we could use instead of MTX in Japan. We expect that other
DMARDs will be approved for treatment of JIA, even in consideration of medical economy and appropriate
The present case report describes a 14-year-old girl with systemic lupus erythematosus（ SLE）. She
presented with butterfly-shaped erythema, oral ulcers, joint pain, proteinuria, pericarditis, leukopenia,
elevated anti-dsDNA-, anti-Sm-, and antiphospholipid antibodies, and low complement, and was antinuclear
antibody-positive. As she had a cough and bloody sputum on admission, alveolar hemorrhage syndrome was
suspected. Therefore, she underwent two courses of steroid pulse therapy, and her symptoms of SLE
International Society of Nephrology class Ⅲ （A/C） lupus nephritis was diagnosed according to
kidney biopsy results. Due to the possibility of diffuse alveolar hemorrhage, she underwent ovarian tissue
cryopreservation to preserve her fertility and cyclophosphamide pulse therapy was initiated four days after.
Young women with cancer undergo ovarian tissue cryopreservation when treated with alkylating
agent； however, this is the second report of cryopreservation of ovarian tissue for SLE in Japan.
SLE affects young women at a high rate； therefore, female patients with SLE should be presented
with the option of ovarian tissue cryopreservation before cyclophosphamide pulse therapy.
Patients with childhood-onset systemic lupus erythematosus （SLE） often follow a more acute and
severe clinical course compared with those with adult-onset SLE. Herein, we report a case of refractory SLE.
An 11-year-old boy was diagnosed with severe lupus nephritis （LN）, classified as IV-S（A） on histology
using the International Society of Nephrology/Renal Pathology Society classification. He was treated with
methylprednisolone pulse therapy （MPT）, mycophenolate mofetil （MMF）, and intravenous
cyclophosphamide for remission. Seven months after treatment initiation, the patient experienced a
recurrence of SLE, associated with deteriorated renal function. He was retreated with MPT, MMF,
tacrolimus, and hydroxychloroquine. A second renal biopsy was performed； he had progressed to class Ⅳ-G
（A/C） on histology with either crescent formation or segmental sclerosis or both in all glomerulus.
Therefore, we introduced immuno-adsorption plasmapheresis, as well as repeated rituximab treatment. It
took approximately 10 months to achieve normal urinary findings after the recurrence, despite a persistently
high level of serum anti-double-stranded DNA antibody. Male sex, childhood onset, and associated LN are
adverse prognostic factors of SLE. Thus, remission induction in the early stage of the disease should include
treatment with multi-target therapy, immuno-adsorption plasmapheresis and B-cell depletion in male
patients with childhood-onset SLE complicated with severe LN.
Juvenile dermatomyositis（ JDM） is an autoimmune disease presenting with predominantly proximal
muscle weakness and characteristic dermatological symptoms. Patients generally have a good prognosis, and
the majority of cases are responsive to steroid therapy. However, a fulminant subtype is particularly
dangerous, with rapid deterioration of muscle tissue and release of myoglobin into the bloodstream, causing
significant myoglobinemia and myoglobinuria, which can trigger acute renal failure.
An 8─year─old boy diagnosed with JDM by his previous physician had no symptomatic improvement
following intravenous methylprednisolone （IVMP） and methotrexate （MTX）. He was further diagnosed
with steroid─resistant JDM, and referred to our hospital for further treatment. The boy was started on
intravenous cyclophosphamide upon admission. Subsequent dramatic decline in muscle strength, severe
muscle pain, and dysphagia led to a diagnosis of fulminant JDM. Tacrolimus （TAC） and intravenous
immunoglobulin （IVIG） were added to his treatment regimen, but his condition still did not improve.
Concerned about his risk of renal failure, we decided to perform therapeutic plasma exchange （TPE） to
induce remission. Our efforts were successful.
Our experience suggests that TPE in combination with immunosuppressants is an effective treatment
for acute presentations of treatment─resistant fulminant JDM.
Juvenile dermatomyositis （JDM） is a rare autoimmune disorder characterized by dermal and
muscular symptoms and defined as dermatomyositis that develops under the age of 16. Its prognosis is not
always benign especially if the intervention is delayed. Therefore, we should have high index of suspicion for
JDM in children having prolonged dermal and muscular symptoms for early diagnosis of JDM.
We encountered a 4-year-old girl with JDM who presented with butterfly rash and skin eruptions
lasting for more than 3 months. She responded well to the intervention consisting of steroid and
In order to clarify the clinical features for the diagnosis of JDM in early infancy, we reviewed the
reported cases of JDM which developed younger than 5 years of age including ours. As a result, persistent
dermal symptoms were observed dominantly, while signs and symptoms due to myositis were rare in
patients with JDM younger than 5 years of age. Therefore, in infants with prolonged dermal signs and
symptoms, we should suspect the existence of JDM behind and perform MRI to detect the myositis in
Pyoderma gangrenosum （PG） is a sterile pyoderma that initially forms small pustules and papules
predominantly on the lower extremities, and rapidly evolves to ulcers. It is often associated with
inflammatory bowel disease, arthritis or vasculitis. A 2-year-old girl presented with a two-week history of
fever, tonsillitis, mild abdominal pain and a skin ulcer on her left lower leg. Initially, a small pustule formed
on her leg, for which oral antibiotics were not effective. Subsequently, the skin lesion ulcerated. The ulcer
improved with cleaning and the application of an ointment （Sulfadiazine silver）, but she showed pathergy
and sterile abscesses developed on her finger and lips. Because of the absence of HLA-A26 and B51, major
symptoms of Behçet’s disease（ eg. genital ulcer or uveitis）, and vasculitis, we diagnosed Sweet’s syndrome at
the first medical examination. After starting prednisolone, all of her symptoms improved. However during
the tapering of prednisolone, her symptoms, especially abdominal pain, recurred. Multiple ulcers on her colon
were observed during gastrointestinal endoscopy, and we revised the diagnosis to intestinal Behçet’s disease.
As the features of skin pathologies of Behçet’s disease, Sweet’s syndrome and PG are similar to each other,
attention should be given to the changes in symptoms and diagnosis and treatment should be reviewed.
We describe the case of linear scleroderma with arthritis of interphalangeal joints of hand as an initial
symptom. An 8-year-old girl was referred to us with a 4-month history of swelling and arthralgia of right
third metacarpophalangeal （MP） joint and a 3-month history of arthralgia of left second proximal
interphalangeal joint. Laboratory examination showed no significant findings. Contrast magnetic resonance
imaging （MRI） revealed peritendinitis around the left third MP joint with longitudinal enhancement of
flexor tendon, bone marrow edema of the third metacarpal bone and peritendinitis around the right second
PIP joint. Linear lesions of scleroderma became gradually clear several months later. The diagnosis of linear
scleroderma was made. Cyclosporine was started and abnormal findings at the site of linear lesion on MRI
were improved after 1 year treatment with cyclosporine. This patient presented diagnostic difficulties
because she had no typical skin lesion of scleroderma. MRI might be useful to diagnose linear scleroderma.
Patients with linear scleroderma with arthritis should be treated aggressively with immunosuppressants
including cyclosporine to prevent the development of joint contracture leading to permanent dysability.