The Journal of Clinical Pediatric Rheumatology
Online ISSN : 2434-608X
Print ISSN : 2435-1105
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Showing 1-10 articles out of 10 articles from the selected issue
  • Akira Hokonohara
    2011 Volume 4 Issue 1 Pages 3-7
    Published: 2011
    Released: October 29, 2020
    JOURNALS FREE ACCESS
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  • Yoshiaki Shikama, Gen Sasaki, Akihiko Shimizu, Jun-ichi Ishikawa, Kuni ...
    2011 Volume 4 Issue 1 Pages 8-12
    Published: 2011
    Released: October 29, 2020
    JOURNALS FREE ACCESS
    We report a case of a five-year-old girl suffering from systemic-onset juvenile idiopathic arthritis (S-JIA). Her joint symptoms and hypercytokinemia persisted despite treatment with tocilizumab plus cyclosporine A or tacrolimus. In order to decrease the steroid dose, we introduced thalidomide in her treatment. Some foreign reports have indicated the effectiveness of 23 mg/kg of thalidomide, but in our case, even 5 mg /kg of thalidomide was not sufficiently effective to decrease the steroid dose, though serum IL-18 did decrease slightly. Subtle constipation was noticed during thalidomide treatment, but no other side effects, including peripheral neuropathy, were evident. When the efficacy of tocilizumab for S-JIA is not enough, we must grope for another means of treatment, and it appears that thalidomide, with meticulous attention to side effects, can be an option, although it is not clear why the drug was not significantly effective in our case.
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  • Kazuyuki Ueno, Masaki Shimizu, Akihiro Yachie
    2011 Volume 4 Issue 1 Pages 13-15
    Published: 2011
    Released: October 29, 2020
    JOURNALS FREE ACCESS
    Influenza infection is an important exacerbating factor in systemic juvenile idiopathic arthritis(s-JIA). Influenza vaccination is encouraged in s-JIA patients to prevent primary influenza infection. Previous report showed there is no increase in disease activity after vaccination and patients with s-JIA can undergo infiuenza vaccination. We experienced the case of a patient with(s-JIA)receiving tocilizumab(TCZ)who experienced relapses of s-JIA after receiving influenza vaccination. A 3-year-old girl with steroid dependent s-JIA was treated with TCZ and long-term remission was achieved. She subsequently received a subcutaneous injection of commercially available inactivated influenza vaccine in Japan. Seven days later, she had a relapse of arthritis of her left shoulder. She responded to additional therapy with methylprednisolone pulse therapy and methotrexate. Four weeks after the first vaccination, she received another subcutaneous injection of inactivated influenza vaccine. Seven days later, she had a relapse of arthritis of her left ankle. The serum CRP level remained normal during both relapses, but the serum levels of IL-6 and IL-18were extremely elevated. Systemic symptoms of s-JIA could be masked during TCZ therapy. Carefu1 observation with the monitoring of serum interleukin (IL)-18 and IL-6 levels may be useful.
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  • Yuko Tokuhisa, Masaki Shimizu, Yasuhisa Sakakibara, Natsumi Inoue, Say ...
    2011 Volume 4 Issue 1 Pages 16-19
    Published: 2011
    Released: October 29, 2020
    JOURNALS FREE ACCESS
       Macrophage activation syndrome(MAS)has been observed in patients with systemic lupus erythematosus( SLE).Recognition of MAS in patients with SLE may be particularly challenging because it may mimic the clinical features of the underlying disease or be confused with an infectious complication. We present a case of SLE associated MAS(SLE-MAS). The patient showed the distinct cytokine profile of SLE-MAS compared to systemic juvenile idiopathic arthritis associated MAS. The observed TNF-α dominant increase appears to be characteristic of SLE-MAS.IgM type antilymphocyte antibody(ALAB)was detected on the surface of lymphocytes during the acute phase and disappeared when the patient was in remission. The patient had a heterozygous P369S-R408Q mutation in the MEFV gene. Our results suggest that ALAB and a MEFV mutation might play important roles in the pathogenesis of SLE-MAS. Furthermore,the cytokine profile of SLE-MAS differs from that of S-JIA-MAS:the TNF-α dominant increase appears to be characteristic.
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  • Akiko Sasaki, Gen Sasaki, Yoshiaki Shikama, Eihiko Takahashi, Kunihiko ...
    2011 Volume 4 Issue 1 Pages 20-25
    Published: 2011
    Released: October 29, 2020
    JOURNALS FREE ACCESS
    The symptom and change along with time and new other symptoms occasionally develop in a clinical course of mixed connective tissue disease(MCTD).    We describe a 17-year-old girl with 8-year history of MCTD whose clinical course was unique. She suffered from common symptoms such as Raynaud’s phenomenon and high titers of anti-Ul-RNP antibodies, SLE-like findings(with low titers of anti-Sm antibodies), polymyositis-like findings and JIA-like findings at the first visit. A diagnosis of MCTD was established. HLA typing was DR2 positive. Other manifestation included asymptomatic Sjogren syndrome, but anti-Ro/La antibodies were negative. Inflammatory myositis was markedly diminished and anti-Sm antibodies disappeared following corticosteroid/methotrexate treatment. Four years later, hypocomplementemia persisted and anti-Sm antibodies reappeared. Renal biopsy showed diffuse proliferative glomerulonephritis. During corticosteroid/azathioprine treatment,hypocomplementemia reappeared and anti-dsDNA antibodies developed. The combination of anti-Sm antibodies, HLA-DR2 and anti dsDNA  antibodies would be associated with refractory diffuse proliferative glomenllonephritis. ln addition, anti- Ro antibodies developed and increase in level of anti-Ro antibodies was associated with clinical exacerbations of photosensitivity rash.    Since change of symptom and sign is the nature of the disease,thorough and frequent evaluation of children with MCTD and HLA typing is important to detect organ involvement.
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  • Masaki Shimizu, Yoshihito Kasahara, Akira Sato, Akiko Toga, Kazuyuki U ...
    2011 Volume 4 Issue 1 Pages 26-30
    Published: 2011
    Released: October 29, 2020
    JOURNALS FREE ACCESS
    Juvenile dermatomyositis(JDM)is a chronic multisystem pediatric inflammatory myopathy primarily affecting the muscles and skin. Corticosteroids are the first-line therapy, however, long-term use of corticosteroids is associated with many side effects. Recently, the B cell-depleting anti-CD20 monoclonal antibody rituximab(RTX)has been successfully used in patients with refractory JDM to improve muscle strength and/or skin manifestations. We describe a case of refractory JDM successfully treated with RTX, which led to long-term remission. RTX can be effective in treating refractory JDM. Prospective randomized trials are required to evaluate the optimal therapeutic dose schedule of RTX and to determine as a proper maintenance immunosuppressive therapy.
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  • Kosuke Shabana, Nami Okamoto, Takuji Murata, Hiroshi Tamai
    2011 Volume 4 Issue 1 Pages 31-36
    Published: 2011
    Released: October 29, 2020
    JOURNALS FREE ACCESS
    We experienced 3 cases of refractory juvenile dermatomyositis that intravenous immune globulin (IVIG)has responded. Case l is 5-y-o boy. He was treated with intravenous methylprednisolone(IVMP), oral corticosteroid and methotrexate at the onset,and he had maintained remission. After 10 months, he had a relapse. We underwent intravenous cyclophosphamide(IVCYC)in addition to the change from methotrexate to azathioprine, but he did not respond. Therefore,we performed IVIG, added methotrexate and changed from azathioprine to cyclosporin A,and he was in remission.After that he relapsed,we underwent IVIG again and changed from cyclosporin A to tacrolimus. Case 2 is 11-y-o girl. Her symptoms were improved IVMP and oral corticosteroid,but worsened according as decreasing corticosteroid after 5 months from the start of treatment. Despite increasing corticosteroid and administrating methotrexate she was not improved.Accordingly,we performed IVIG and added cyclosporin A,and she was in remission. After 4 months,on account of relapse she was administered IVIG again. Since then,she has maintained in remission(she has on no medication after 4 years and 5 months after last IVIG). Case 3 is 15-y-o boy. He was complicated by interstitial pneumonia. We treated him with IVMP,oral corticosteroid and azathioprine.We changed from azathioprine to cyclosporin A,performed IVCYC(8 times)and replaced cyclosporine A with tacrolimus after IVCYC, for interstitial pneumonia. His myositis relapse as decreasing corticosteroid,and we performed IVIG, increased corticosteroid and added methotrexate. Then he has been in remission.     Their symptoms of myositis were in effect of IVIG immediately and also observed in the effects of cutaneous symptoms as well.
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  • Yasuhiro Ueda, Yasuhiro Yamazaki, Syunichiro Takezaki, Masafumi Yamada ...
    2011 Volume 4 Issue 1 Pages 37-40
    Published: 2011
    Released: October 29, 2020
    JOURNALS FREE ACCESS
    Chronic nonbacterial osteitis/osteomyelitis(CNO)is a rare and possibly autoinflammatory disease characterized by chronic and recurrent bone pain often associated with low grade fever or general malaise. We report an eight-year-old girl with CNO presenting with recurrent spiking fever and bone pain. Her febrile episodes were associated with elevated erythrocyte sedimentation rate, serum C-reactive protein and IL- 181evels but not with serum levels of IFN-γ, IL-6,or TNF-α. Positron emission tomography demonstrated fluorodeoxyglucose accumulation in epiphyses of bilateral distal femurs and tarsal bones. Bone edema-1ike lesions with high intensity in short-tau inversion recovery image and low in Tl weighted image were documented in magnetic resonance imaging of lower extremities. Bacterial osteomyelitis and bone neoplasm were excluded by bone biopsy of distal femur. All of these findings were consistent with the diagnosis of CNO. Her fever and bone pain responded to a short course of corticosteroid therapy but not to non-steroidal anti-inflammatory drugs. We suggest that CNO should be considered as a diagnosis of high grade fever particularly associated with bone pain,and that innate immune mechanisms are involved in the pathogenesis.
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  • Takashi Ishige, Maiko Tatsuki, Takeshi Tomomasa, Takumi Takizawa, Reik ...
    2011 Volume 4 Issue 1 Pages 41-45
    Published: 2011
    Released: October 29, 2020
    JOURNALS FREE ACCESS
    We present a patient diagnosed with inflammatory bowel disease type unclassified(IBDU)who had multiple whitish papules consistent with those of malignant atrophic papulosis(Degos disease),which appeared during a flare of her disease. Twelve-year-old girl developed recurrent abdominal pain, hematochezia,and diagnosed as ulcerative colitis as a result of colonoscopy.Mesalazine treatment relieved her symptoms,whereas upper gastrointestinal involvement developed after 2 weeks of treatment. After treatment with prednisolone, she developed multiple papules on both upper and lower extremities. The lesions of malignant atrophic papulosis are said to be pathognomonic;nevertheless, various diseases with similar clinical lesions have been described. In present case, elevated PR3-ANCA and arthritis assumed complication of vasculitis, which is involved in both IBDU and Degos disease.
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  • Tomomi Sato, Tomo Nozawa, Taichi Kanetaka, Masako Kikuchi, Kazuko Yama ...
    2011 Volume 4 Issue 1 Pages 46-49
    Published: 2011
    Released: October 29, 2020
    JOURNALS FREE ACCESS
    The subject was a 5-year old girl in whom prednisolone and methotrexate administered fbr oligo-articular  juvenile idiopathic arthritis in the right knee-joint. The girl was referred to our department at the age of 2 years and 10 months. Although pain disappeared after administering etanercept,the joint mobility continued to decrease. Etanercept was replaced with tocilizumab,after which the pain in the right knee-joint disappeared, and images showed that the inflammation had disappeared. Contracture of the right knee-joint was nearly stabilized by continuous physiotherapy. Further, because of the improvement in the range of mobility,invasive mobilization surgery and synovectomy were performed. During surgery, fibrous adhesion and fibrillization of the synovial membrane were discovered.After surgery,the range of mobility in the right knee-joint improved;subsequent continuous physiotherapy enabled marked improvement in the restriction of mobility,thereby allowing the subject to walk normally.In recent years,the popularization of immunosuppressants and biopharmaceuticals has reduced the number of surgical opportunities in cases of juvenile idiopathic arthritis. For children in their growth period,however,maintaining the mobility,particularly in the joints,is vital. Therefbre, synovectomies and invasive mobilization surgery should be actively encouraged in patients who are unresponsive to medical treatment and who have fibrous adhesion or fibrillization of the synovial membranes,after the inflammation has been resolved.
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