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[in Japanese]

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To　assess　the　role　of　interleukin（lL) -33　and　ST2，　the　receptor　fbr　IL-33，in　the　pathogenesis　of　systemic juvenile　idiopathic　arthritis（s-JIA），　we　sequentially　measured　the　seruln　levels　of　IL-33　and　soluble　ST2（sST2） in　patients　with　s－JIA　and　deterlnined　their　correlation　with　measures　of disease　activity　and　severity．Twenty four 　patients　with　s－J工A，5“）’ith　rheumatoid　factor　positive　polyarticular　JIA（RF＋poly－JIA），　and　20　age－matched healthy　controls（HCs）were　analyzed、　IL-33　and　sST21evels　were　quantified　in　serum　by　enzyme-linked immunosorbent　assays．　Serum　IL-331evels　in　most　patients　with　active　s-JIA　were　below　the　lowest　detection limit．　Serum　IL-331evels　in　patients　with　RF+poly-JIA　were　much　higher　than　those　in　patients　with　s-JIA　and HCs．　Serum　sST2 1evels　in　patients　during　the　active　phase　of　s-JIA　were　mutch　higher　than　thosc　ill　patients with　poly-JIA　and　HCs．　Serum　sST2 1evels　in　patients　with　s-JIA　were　sjgnificantly　elevated　even　in　the　inactive phase，　when　other　clinical　parameters　were　normalized．　Serum　sST21evels　correlated　positively　with　the　clinical parameters　of　disease　activity．　These　findings　indicate　that　ST2　may　be　an　important　lnediator　in　s-JIA．　Serum sST2 1evels　in　patients　with　s-JIA　correlated　with　disease　activity，　suggesting　a　potential　role　as　a　promising indicator　of　disease　activity．

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　Microvascular　damage　is　closely　related　to　tlle　pathogenesis　of　systemic　sclerosis（SSc）or　juvenile dermatomyositis（JDM）．　Nailfold　Video　Capillaroscopy（NVC）is　a　convenient　and　noninvasive　method　to analyze　the　capillary　morphology　and　architecture．　Abnormal　NVC　findings　including　giant　capillaries　are specifically　observed　in　patients　with　SSc　and　JDM．　Early　in　the　disease，　thesc　findings　can　be　well　recognized　by NVC，which　is　reported　to　have　both　diagnostic　and　prognostic　values　in　SSc　and　JDM．　In　this　report，　we　report sequential　NVC　findings　in　one　patient　with　SSc　and　two　patients　with　JDM．NVC　patterns　were　correlated　with disease　activity　of these　diseases．　NVC　is　the　convenient　and　safe　method　to　analyze　microvascular　abnormalities and　is　useful　for　differential　diagnosis　and　evaluation　of　disease　activity　in　SSc　and　JDM．

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Patients　with　juvenile　fibromyalgia（JFM） have　been　treated　with　multidirectional　approach　during hospitalization　in　our　hospital.　Admission　isolates　JFM　patients　from　their　troublesome　environment　that　they cannot　handle　well　in　their　school　and/or　home．　The　patients　who　have　severe　physical　disability　and　come-up against　difficult　situation　were　suitable　for　hospitalization．　The　patients　are　in　scheduled　daily　life　and　enroll　in an　in-hospital　class　during　hospitalization．　Rehabilitation　is　started　for　improvement　of　patient's　physical disability　and　drug　therapy　is　helpful　for　relieving　pain． A total　of　32　children（aged　7.9-15.7　years）who　admitted　in　our　hospital　from　March　2001　to　December 2012 were　retrospectively　examined　by　medical　record．　Fifty-three　percent　of　the　patients　were　improved　in clinical　stage，and　only　one　patient　got　worse　in　activities　of　daily　living．　In　nine　out of　32　patients，tender　point counts　were　decreased，and　disappeared　in　6 out　of　32　patients．　Nine　out　of　25　patients　who　stopped　going　to school　came　to　go　to　school　at　three　months　after　leaving　hospital． 　　　We　suggest　that　multidirectional　approach　during　hospitalization　must　be　effective　therapy　for　severe JFM　patients　to　improve　their　physical　disability．

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A3-year-old　boy　was　admitted　to　our　hospital　because　of　a　prolonged　fever，red　lips，cervical lymphadenopathy，a　generalized　rash，edema　of　the　extremities　and　ambulation　difficulty．　A　physical　examination demonstrated　a　facial　erythematous　rash　with　edema　but　no　heliotrope　rash　or　Gottron's　papules．　His　ESR　and muscle　enzymes　such　as　CK　were　elevated　but　CRP　was　normal．　Echocardiography　revealed　a　normal　ejection fraction　and　no　coronary　artery　dilatation．　Clinical　findings　were　thought　to　be　consistent　with　the　diagnosis　of Kawasaki　disease（KD）．　He　was　given　2g/kg　of　intravenous　immunoglobulin　along　with　aspirin．　Although　he became　afebrile，　his　symmetric　proximal　muscle　weakness　persisted．　An　MRI　showed　hyperintensity　of his　thigh muscle　on　T2-weighted　images，　consistent　with　myopathy．　His　muscle　biopsy　specimens　demonstrated perifascisular　atrophy, necrosis　and　variation　in　fiber　size．　Given　the　results　of　clinica1-pathological　findings，in addition　to　the　imaging　pictures．　the　diagnosis　was　deterlnined　to　be consistent　with　juvenile　dermatomyositis （JDM）．　He　was　treated　with　pulse　steroids　of　3　courses．　All　clinical　and　laboratory　abnorlnalities　were　reversed． We　dcscribed，　to　the　best　of　our　knowledge．　the　first　reported　case　of　JDM　with　features　of　KD．　Clinically　it　is difficult　to　differentiate　between　these　two　vascuIitis　diseases，especially　when　KD　is　in　its　atypical　form．

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A l3-year-old　girl　visited　our　outpatient　clinic　complaining　of　cough　and　rhinorrhea．　She　had　butterfly erythema　on　the　face　and　several　itchy　papules　on　the　upper　limbs．　Proximal　muscle　weakness　was　evident． Laboratory　examination　revealed　proteinuria　and　an　elevated　serum　level　of　creatine　kinase（6,802 IU/L）． Abnormal　findings　on　the　muscles　in　the　front　of　both　the　thigh　in　magnetic　resonance　imaging（MRI）was suggestive　of　inflalllmato17y　myositis．　She　was　diagnosed　as　juvenile　dermatomyositis　and　was　initially　treated with　2　cycles　of methylprednisolone　pulse　therapy．　Although　the　initial　treatment　failed　to　achieve　full　remission， second　line　treatment　with　intravenous　cyclophosphamide　pulse　therapy（IVCY）was　effective　and　muscle weakness　was　markedly　improved．　However．　when　the　interval　of　IVCY　administration　was　prolonged，　the levels　of　muscle　enzymes　were　elevated　again．　She　was　subsequently　treated　with　mizoribine　in lieu　of cyclophosphamide．　This　decreased　the　levels　of　muscle　enzymes．which　allowed　us　to　reduce　the　dose　of　oral prednisolone．　As　total　dosage　of　cyclophosphamide　should　be　limited　because　of　its　side　effects，　it　is　impossible to　unboundedly　continue　IVCY.　The　present　case　suggests　the　effectiveness　of　mizoribine　for　refractory　juvenile dermatomyositis．

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A　1-month-old　girl　whose　mother　has　been　diagnosed　with　anti　U1RNP　antibody-positive　mixed connective　tissue　disease　came　to　our　hospital　because　of　oral　ulcer，poor　weight　gain　and　slight　erythema．　A week　later，oral　ulcer　improved　although　erythema　expanded　gradually．Shc　was　diagnosed　with　neonatal　lupus erythematosus（NLE）because　anti　U1RNPantibody　was　detected　from　herself.　Weight　gain　was　turned　well，　and erythema　improved　gradually，completely　disappeared　at　6　months　of　age．　No　electrocardiogram　abnormality was　observed．　NLE　is　an　autoimmune　syndrome　caused　by　acquired　autoantibody　through　the　placenta　from　the mother．　The　major　antibody　that　cause　NLE　are　anti-SS-A　antibody　and　anti-SS-B　antibody．　although　anti-UIRNP antibody　was　positive　in　this　presented　case．　This　is　an　interesting　case　of　female　NLE，　in　that first　symptom were　oral　ulcer　and　poor　weight　gain，and　erythema　exacerbated　later．

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lt　is　a　case　of　the　6-month-old　girl　introduced　to　our　hospital　for　fever　of　undetermined　origin．　She　had fever　and　exanthema，her　serum　CRP　level　was　high　and　her　echocardiography　revealed　a　coronary　aneurysm．　At the　time, we　diagnosed　that　she　was　Kawasaki　disease．　However，enhanced　CT　performed　at　day　36　demonstrated vasculitis．Therefore，we　decided　her　diagnosis　as　Takayasu　arteritis，and　treated　with　metylpredorlisolone （mPSL） pulses　with　anticoagulant　therapy．　The　coronary　arteries　were　extended　although　inflammation　was　an improvement　tendency，so　the　treatment　of　mPSL　pulses　was　canceled，and　a　treatment　of　cyclophosphamide pulses　was　started． 　　　Her　clinical　and　biochemical　parameters　improved　with　cyclophospharnide．　Although　a　total　of　six courses　of　cyclophosphamide　pulse　therapies　was　enforced　using　azathioprine （AZP）together　after　that，advance of the　narrowing　artery　of the　coronary　arteries　and　others　is　not　accepted　by　the　present．　It　was　the　case　that　the differential　diagnosis　of　Kawasaki　disease　and　Takayasu　arteritis　was　difficult, and　the　selection　of　the　treatment was　not　easy　to　us．

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We　report　a　12-year-old　boy　with　juvenile　intestinal　Behcet’s　disease．　He　presented　with　fever　and　oral aphtha　repeatedly　from　early　childhood　and his　blood　examination　often　showed　an　inflammatory　reaction．　But no　bacteria　were　found　in　any　bacteriological　examination．CT，　MRI　and　scintigram　of　the　whole　body　were normal．his　bone　marrow　chromosomal　test　revealed　trisomy　8（47XY，＋8［19］/46XY［1］），raising　suspicion　of the　presence　of　intestinal　Behcet's　disease．　Endoscopy　revealed　several　circular　ulcers　in　his　distal　esophagus and　ileocecal　region．　He　was　diagnosed　as　intestinal　Behcet's　disease　with　accompanying　trisomy　8． Myelodysplastic　syndrome（MDS） which　often　complicates　trisomy　8　was　not　observed　in　his　bone　marrow． 　　　This　was　a　rare　case　in　which　the　marrow　chromosome　abnormalities　led　to　the　diagnosis　of the　cause　of an　unknown　fever．　In　addition　it　is　very　interesting　case　in　terms　of　studying　the　pathogenic　mechanism　of intestinal　Behcet’s　disease．

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Although　most　fevers　ill　children　are　acute　and　are　due　to　common　infections，　we　sometimes　encounter patients　with　persistent　fever　of　unknown　origin（FUO）．　Recent　discovery　of　autoinflammatory　syndromes further　provides　diagnostic　advances，　Nevertheless，　it　remains　difficult　to　make　a　definitive　diagnosis　in　some cases of FUO．　A　fourteen-year-old　girl　visited　our　clinic，　complaining　recurrent　fever　over　38．0°C　with　headache． nausea，　vomiting，　anorexia，　and　general　malaise．Although　infections，　tumors，　drug-induced　fever，　autoimmune disorders、　and　autoinflammatory　syndromes　were　considered　as　differential　diagnosis，　we　failed　to　obtain laboratory　results　consistent　with　these　conditions．　Repeated　blood　examinations　did　not　show　any　positive inflammatory　markers．　A　gallium　scintigraphy　performed　ill　a　febrile　condition　was　negative．　The　levels　of inflammatory　cytokines　were　within　normal　ranges．　Taken　together，we　reached　a　diagnosis　of　psychogenic　fever． As　persistent　high　body　temperature　is　not　commonly　seen　in　psychogenic　fever，　it　was　difficult　for　us　to　yield　a correct　diagnosis．Our　case　suggests　that　when　we　see　a　patient　with　persistent　fever　without　source　consideration should　be　given　to　psychogenic　fever．

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