The Journal of Clinical Pediatric Rheumatology
Online ISSN : 2434-608X
Print ISSN : 2435-1105
Volume 3 , Issue 2
Showing 1-13 articles out of 13 articles from the selected issue
  • Ken-ichi Yamagauchi, Masato Okada
    2010 Volume 3 Issue 2 Pages 43-46
    Published: 2010
    Released: October 30, 2020
    JOURNALS FREE ACCESS
  • Chiemi Spratt, Noriko Kinjo
    2010 Volume 3 Issue 2 Pages 47-52
    Published: 2010
    Released: October 30, 2020
    JOURNALS FREE ACCESS
    Juvenile dermatomyositis(JDM)is characterised by diffuse inflammation in muscle and non-infectious inflammation of skin by systemic small vessel vasculopathy. Polymyositis(PM)is rare in children. The clinical features of 13 JDM patients and 2 patients, which were 15children(8 boys and 7 girls)and aged between 2 months and 11 years and l l months, were analyzed retrospectively. There were 8 boys and 7 girls. The age at onset ranged from 2 month to 11 years and 11 month. Rash and muscle weakness were most common as initial symptoms. Initial treatment was a prednisoline therapy in 3 cases and a methlprednisolone pulse therapy in llcases.. Combination therapy with intravenous immunoglobulin or methotrexate were done in 5 cases. As complication, calcinosis in 3 cases, central nervous system symptoms in 1 case, endocrine dysfunction in 2 cases were shown. 1 case showed small intestine ulcer, so it was suspected severe type(Banker type)JDM.2 cases already showed interstitial lung disease with high levels of KL-6 at onset. On severe type JDM and early onset case(2 month), it were difficult to reduce dosage of prednisolone and control by combination therapy with some immunosuppressive drugs. The clinical features of JDM/PM exhibit broad spectrum of the severity. It is important to select proper treatment on a varied severity of JDM/PM.
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  • Nami Okamoto, Takuji Murata, Kousuke Shabana, Hirohumi Tamaki, Hiroshi ...
    2010 Volume 3 Issue 2 Pages 53-57
    Published: 2010
    Released: October 30, 2020
    JOURNALS FREE ACCESS
     We occasionally experience that healthy children show a transient high levels of anticoagulants after infection. Most of their features are subclinical or rarely hemorrhagic/thrombotic symptom, and they spontaneously recover from them. Thrombosis is caused by anticoagulants in patients with anti-phospholipid antibody syndrome, and the anticoagulant positive children tend to be easy bleeding. It was reported that low activity of complement and anticoagulant factor were complicated in acute phase, yet there was no certain tendency or fixed mechanism. We experienced three pediatric cases with hemorrhage and anticoagulants positive. Case l:2-y-o boy with lupus anticoagulant(LAC), anti-cardiolipin antibody(ACA)and low factor VIII,factor IX activity. Case 2:2-y-o girl with LAC, ACA and low factor IX activity. Case 3:4-y-o girl with LAC and low Factor IX activity. All cases showed low complement activity, instead of anti-nuclear antibody negative, and they clinically improved in a natural course. Case 2 and 3 also clinically and serologically improved. We concluded that both of the anti-phospholipid antibodies and the coagulation inhibitors influence the hemorrhagic symptoms during active phase.
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  • Masaki Shimizu, Yasuo Nakagishi, Kazuko Kasai, Yuichi Yamasaki, Mari M ...
    2010 Volume 3 Issue 2 Pages 58-62
    Published: 2010
    Released: October 30, 2020
    JOURNALS FREE ACCESS
    Tocilizumab(TCZ), a humanized anti-IL-6 receptor monoclonal antibody,is an effective cytokine inhibitor fbr the treatment of juvenile idiopathic arthritis(JIA). However, macrophage-activation syndrome (MAS)has been observed as a complication in some cases of systemic-JIA during TCZ therapy In this report, we described the clinical courses of 5 cases of MAS during TCZ therapy and demonstrated the need fbr monitoring serum interleukin(IL)-18concentrations. Clinical symptoms of patients with s-JIA receiving TCZ were apparently mild compared to those not receiving TCZ, Furthermore, serum CRP concentrations never increased during TCZ therapy, even in MAS. Serum IL-18concentrations extremely elevated over 10,000 pg/mL in MAS. Serum IL-18 concentrations increased persistently before the other measures of disease activity. The clinical symptoms of MAS and s-JIA could be masked during TCZ therapy;hence, monitoring serum concentrations of IL-18 is recommended for the evaluation of disease activity in s-JIA.
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  • Akane Hirose, Hisashi Kawashima, Eiko Sakai, Soken Go, Satoshi Sato, S ...
    2010 Volume 3 Issue 2 Pages 63-66
    Published: 2010
    Released: October 30, 2020
    JOURNALS FREE ACCESS
       A surplus of cytokine production and an overreaction ofhistiocytes and macrophages that lead to necrosis are considered to be possibly associated with subacute necrotizing lymphadenitis(Kikuchi-Fujimoto disease), but descriptions in the pediatric literature are sparse. To better understand this disease in children we investigated their cytokine profiling and immunohistochemical staining. The serum levels of IL-6, IFN-γ, MIP-13 and TNF-α were significantly high compared with those of the controls. On immunohistochemical staining, cytotoxic T cells mainly infiltrated the necrotic area. Extremely high levels of IFN-γ suggest that chronic viral infection had occurred, and chemokines might have increased due to inflammation after infection. There was no significant difference in serum cytokines between in SLE and subacute necrotizing lymphadenitis. From these findings long-term -follow up is recommended.
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  • Yutaka Yoshimatsu, Tomowa Kanou, Fumika Miyajima, Haruka Suga, Katsumi ...
    2010 Volume 3 Issue 2 Pages 67-71
    Published: 2010
    Released: October 30, 2020
    JOURNALS FREE ACCESS
    We report a 16-year-old girl who developed neuromyelitis optica(NMO)associated with systemic juvenile idiopathic arthritis(s-JIA). She developed left optic neuritis at the age of 10. Since then she experienced recurrent attacks of optic neuritis and myelitis. She was diagnosed with NMO at the age of 14, and she was seropositive for NMO-IgG(antibody for aquaporin 4. She was hospitalized after she suffered joint pain of her bilateral fingers and left knee. At the same time, she experienced remittent fever. She suffered atypical erythema on her abdomen and femur. Laboratory examination showed that antinuclear antibodies were positive at l:320. MRI of her right fingers suggested arthritis. Based on the clinical symptoms and MRI findings, we confirmed a diagnosis of s-JIA. After admission, she developed macrophage activating syndrome and was treated with dexamethasone palmitate and cyclosporin A. She experienced a recurrent attack of s-JIA at the age of 16 and 9 months old. She had been treated with tocilizumab. We were unable to find a case report of NMO associated with s-JIA, and therefbre, we consider this as a rare case.
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  • Tomoko Kunishima, Noriko Kinjo
    2010 Volume 3 Issue 2 Pages 72-76
    Published: 2010
    Released: October 30, 2020
    JOURNALS FREE ACCESS
    A seven-year-old boy had had his right ankle sprained, while practicing a Japanese martial art,kendo. A PTB brace had been installed on the right fbot,but the symptom had worsened.Six months after the onset, he was diagnosed with oligoarticular juvenile idiopathic arthritis. The MRI was revealed a hyperplasia of synovium and a destruction of cartilage on his ankle. Also, the level of serum MMP-3 was high.Nine months after the onset,a low-dose methotrexate pulse therapy was initiated, but he was not able to walk without a crutch. The narrowing of the joint space progressively revealed by ankle x-ray image. The levels of MMP-3 and anti-CCP-antibody continued high.Nineteen months after the onset, he started tocilizumab therapy. Then, the levels of CRP, ESR, and MMP- 3 were reduced to normal, and the narrowing of the joint space remarkably improved.In conclusion,the tocilizumab therapy could be inhibited a destruction of bones and cartilages of oligoarticular JIA,and improved the radiological findings of articular damages of oligoarticular JIA.
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  • Kyoko Kuroiwa, Tomonari Shigemura, Norimoto Kobayashi, Kenichi Koike, ...
    2010 Volume 3 Issue 2 Pages 77-80
    Published: 2010
    Released: October 30, 2020
    JOURNALS FREE ACCESS
      Typical familial Mediterranean fever(FMF)shows severe stomachache or chest/back pain accompanied with fever. Some FMF patients show arthritis, however, the detail has been unclear. We here analyzed the prolonged arthritis accompanied by FMF.25-year-old woman showed fever with stomachache,chest pain, and arthritis in the knees and the fbot, which healed in a few days since her childhood. On the basis of MEFV gene analysis revealing M6941/M6941 mutation, we made a diagnosed of FMF. After having colchicine, she had had only mild attacks. She had pain and remarkable swelling in the right knee. Arthrocentesis revealed that the synovial fluid was yellow and quite muddy,mainly including neutrophils. MRI fndings of the knee were slight growth of synovial membrane,stagnation of synovial fluid,but no bone destruction.She had colchicine continuously,thereafter,she had an arthritis with erysipelas like rash in the foot joint.The arthritis disappeared after three months.We rarely see joint destruction in FMF.This may be attributed to the only activation of neutrophils,in contrast to RA accompanied by articular destruction,in which T cell abnormalities were recognized.
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  • Ken-ichi Yamaguchi
    2010 Volume 3 Issue 2 Pages 81-84
    Published: 2010
    Released: October 30, 2020
    JOURNALS FREE ACCESS
    ln Japan,it is considered juvenile psoriatic arthritis is rare,but that may have been underestimated. We had a case 14 year of age who took five years until diagnosed.We were examined with a discussion of the literature about the point that may be helpful in early diagnosis.
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  • Tomoko Shigemori, Yasuhiko Ito, Toru Igarashi, Kaoru Aki, Tsuyoshi Yan ...
    2010 Volume 3 Issue 2 Pages 85-88
    Published: 2010
    Released: October 30, 2020
    JOURNALS FREE ACCESS
    ubulointerstitial nephritis and uveitis(TINU)syndrome which complicates tubulointerstitial nephritis and uveitis was first described in 1975. Since then,200 or more cases with TINU syndrome have been reported in Japan. We report a case with TINU syndrome. Patient was a l5-year01d girl,who had complained of photophobia and conjunctival injection. Ophthalmologist made her a diagnosis of uveitis,and some renal diseases.We performed renal biopsy.An accumulation of inflammatory cells such as neutrophils and monocytes,and renal tubular atrophy were revealed.We diagnosed her as TINU syndrome because of the combination of serious tubulointerstitial nephritis and uveitis. Her photophobia and conjunctival injection had improved by conjunctival instillation of corticosteroid. However,the nephritis was so serious that we chose methylprednisolone pulse therapy of two courses, and after the courses,oral prednisolone 30mg/day was started and gradually tapered.After six months from the onset,the second renal biopsy was perfbrmed.In results,her interstitial nephritis was improved.  In the present case, her complaints came only from uveitis but not from interstitial nephritis. Nevertheless,her nephritis was serious、It is suggested that,even in cases with few renal symptoms、the level of nephritis varies,so that we have to select therapy carefully.Steroids are commonly effective for TINU syndrome. But uveitis and interstitial nephritis may occasionally worsen in the clinically course. We have to pay attention to follow-up even after the improvement.
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  • Tatsuo Kobayashi, Mitsumasa Kishimoto, Kazuki Yoshida, Hideto Oshikawa ...
    2010 Volume 3 Issue 2 Pages 89-92
    Published: 2010
    Released: October 30, 2020
    JOURNALS FREE ACCESS
     The patient was 16 year-old girl,whose chief complaint was polyarthralgia and low grade fever. She had polyarthritis when she was 12 years old and was diagnosed as JIA at age 13. She was treated with MTX and PSL. But her arthritis was fluctuated.We had been consulted about her arthritis when she was 16 years old. HLA-B51 was positive and recurrent oral ulcers lead us to a diagnosis of Behcet’s disease. We started with colchicines and naproxen.Four months later,abdominal pain and diarrhea were emerged.Colonoscopy showed multiple ulcers at the ileocecum. Consequently,she was diagnosed as having gastrointestinal Behcet’s disease. After PSL 20 mg/ day and mesalazine treatment, she remarkably became better.When we see polyarthritis in children,we should consider Behcet’s disease.
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  • Kazuhide Ohta, Yumi Senda, Mika Inoue, Kazunori Mizuno, Shinobu Sakazu ...
    2010 Volume 3 Issue 2 Pages 93-97
    Published: 2010
    Released: October 30, 2020
    JOURNALS FREE ACCESS
    This case involved a 15-year-old girl with critical systemic lupus erythematosus(SLE)from 11 years old. Her first renal histopathological finding was IV-G(A)according to the ISN/RPS classification. In a previous hospital, she had been treated using oral steroid,methylprednisolone pulse therapy,cyclophosphamide pulse therapy and other immunosuppressive drugs(mizoribine and cyclosporine). However, nephrotic state continued, and renal function showed a tendency toward slow exacerbation. She was referred to our hospital for the purpose of long-term management,including future dialysis,in October 2007.Her character at this time was taciturn and unsociable. After transfer to our hospital, we suspected non-compliance with the medication regime,based on the results of blood chemical testing.We only continued ambulatory treatment while suspecting non-compliance,as her clinical condition was stable. However, her SLE recurred from autumn 2008, while renal function suddenly worsened and progressed to chronic renal failure requiring hemodialysis.The etiology of sudden exacerbation was non-compliance with medication,as we had suspected. Compliance can be difficult to achieve in pubertal patients with SLE(particularly pubertal female patients),and we should have hospitalized her and introduced counseling earlier.
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  • Naoko Nakano
    2010 Volume 3 Issue 2 Pages 99-103
    Published: 2010
    Released: October 30, 2020
    JOURNALS FREE ACCESS
    Takayasu arteritis(TA)is a large vessel vasculitis. Young women are commonly affected. Pregnant patients with TA complicate with hypertension,heart failure,dissecting aneurysm of aorta, intracranial hemorrhage,spontaneous abortion and intrauterine growth retardation.However,it is limited to report on the optimal management fbr peripartum period in the patients.We experienced a 28-year-old pregnant woman with TA who had suffered from obstruction and narrowing of aortic arch for 12 years until the diagnosis.She became pregnant by permission under the stable condition.As we controlled her hypertension and the disease activity under the strict guidance,she was normally delivered and got healthy baby. An interdisciplinary collaboration among rheumatologists,nephrologists and obstetricians needs to manage for peripartum period.
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