[in Japanese]

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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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　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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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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　　　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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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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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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　　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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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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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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　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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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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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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