The　first　version　of　guidance　for　the　diagnosis　and　primary　treatment　of juvenile　idiopathic　arthritis was　published in　2007，　since　its　primary　care　in　Japan　is　often　done　by　general　pediatricians　or　orthopedists whose　major　is　not　pediatric　rheumatology．　For　the　purpose　of　better　revision　make，　the　questionnaire　survey was　performed　by　working　group　on　revision　of　guidance　for　the　diagnosis　and　primary　treatment　of juvenile idiopathic　arthritis　in　Japan　College　of　Rheumatology．　We　sent　the　questionnaires　of　different　contents　to pediatric　specialists　and　pediatric　rheumatologists．　As　a　result，　both　groups　thought　that　the　guidance　like　a practical　manual　is　necessary，　which　contains　concise　information　for　diagnosis，　treatment　and　longitudinal management．　Moreover，　it　was　discovered　that　not　a　few　enthesitis　related　arthritis　patients　existed，　though　they had　been　thought　to　be　uncommon　in　Japan． 　　　Thus，　we　think　that　the　questionnaire　procedure　like　this　survey　is　useful　not　only　for　manifesting opinions　of both　users　and　promoters，　but　also　for　revealing　actualities　of the　disease　itself．

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Healthy　adults　have　lower　skin　temperature　of　the　knee　than　that　of tibia，and　it　is　called　'Cool　patella sign'．　Investigation　was　conducted　among　healthy　adults　and　children　to　confirm　the　'Cool　patella　sign' using the　surface　mode　of the　non-contact　infrared　thermometer．　The　skin　temperature　of the　tibia　and　patellar　among 20 healthy　adults　were　32．49 ± 0．94　℃，30．78± 0．83　℃ respectively．　The　skin　difference　of　temperature　was l.71 ± 0．82℃ and　recognized　significant　difference （P　value＜0．01），　The　skin　temperature　of the　tibia　and　patellar among 16 healthy　children　were　32.51 ± 0．92℃，32．06 ± 0．80 ℃ respectively．　The　difference　of　temperature　was 0.50 ± 0．51　degrees　Celsius （P value＝0．07），　When　there　was　the　case　that　the 'Cool　patella　sign' could　not confirm　because　the　subcutaneous　tissue　of the　patellar　part　was　thick　in　infants．　it　was　supposed　It　is　assumed that　there　are　cases　among　infants　where　the　sign　cannot　be　confirmed　due　to　its　thick　subcutaneous　tissue　of the　patellar　part．　Skin　temperature　of　knee　in　a　5　year-6　month-old　girl　diagnosed　with　polyarticular　juvenile idiopathic　arthritis　during　the　clinical　course　of　knee　joint　arthritis　was　also　measured．　Before　treatment，　the patient　was　under' Loss　of cool　patella　sign' state，　with　the　lower　skin　of the　knee （Right：34．3℃ Left：33，6°C） having　much　higher　temperature　than　that　of　the　thigh（Right：32．4℃ Left：32．6℃）and　the　lower　leg（Right： 31．3℃ Left：32．0℃）．　After　5　weeks　of　treatment，　as　knee　joint　arthritis　recovered，　the　'Cool　patella　sign' returned　and　was　confirmed　to　be　a　useful　measure in　examining　knee　joint　arthritis．

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A l4-year-old　girl　with　a　previous　history　of　rhinitis　was　admitted　to　our　hospital　because　of　respiratory distress，　fever，　and　purpura．　Laboratory　data　showed　reduction　of　hemoglobin　concentration．　elevated　C-reactive protein　level，　and　normal　serum　creatinine　level；there　was　no　proteinuria，　but　microscopic　hematuria　was　seen． Chest　radiograph　and　computed　tomography　showed　multiple　bilateral　infiltrates．　Biopsy　specimens　demonstrated granulomatous　vasculitis　in　the　skin and　fibrinoid　necrosis　in the　kidney．　Given　the　clinicopathologic　findings　and the　presence　of　circulating　C-ANCA，　we　arrived　at　a　diagnosis　of　granulomatosis　with　polyangiitis．　At　onset．　she was　treated　with　intravenous　methylprednisolone，　oral　prednisolone，　intravenous　immunoglobulin、　methotrexate， and　intravenous　cyclophosphalnide，　resulting　in　remission．　Five　months　after　the　initial　treatment，　she　had　relapse， with　an　increase　ill　serum　creatinine　and　C-ANCA　levels．　Four　weekly　administrations　of　rituximab（500　mg／ dose；total,　2　g／month）gradually　reduced　her　serum　C-ANCA　levels　and　inhibited　further　increase　in　serum creatinine．　The　dose　of　prednisolone　was　slowly　decreased　without　resulting　to　relapse．　Thereafter．　rituximab　was administered　every　6　months　based　on　serial　B　lymphocyte　counts　and　C-ANCA　levels．　To　date，　good　disease control　has　been　achieved　and　clinical　remission　has　been　maintained　for ＞48　months　without　side　effects．　We experienced　a　case　of　refractory　granulomatosis　with　polyangiitis　who　successfully　responded　to　rituximab　for both　remission　induction　and　maintenance．

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　The　prevalence　of　rheumatic　fever　has　markedly　decreased　in　developed　countries；however，　it　is　an important　disease　in　terms　of　the　differential　diagnosis　of　fever　and　polyarthritis．　Moreover，　the　presence of　concomitant　carditis　has　a　great　effect　on　the　overall　prognosis．　We　reported　a　case　of　rheumatic　fever complicated　with　carditis　during　the　course　of　treatment．　The　patient　was　a　6-year-old　boy　who　visited　our hospital　for　fever，　sore　throat，　and　pain　and　swelling　in　the　multiple　joints，　After　admission，　the　patient　was administered　oral　non-steroidal　anti-inflammatory　drugs（NSAIDs）．　Elevated　C-reactive　protein（CRP）， erythrocyte　sedimentation　rate（ESR），　and　anti-streptolysin　O（ASO）levels　were　observed，　and　the　patient was　diagnosed　with　rheumatic　fever　according　to　Jones　criteria．　Although　arthritic　findings　and　CRP　and ESR　levels　immediately　improved　after　administering　NSAIDs，　echocardiography　findings　and　an　elevated brain　natriuretic　peptide（BNP）level　suggested　concomitant　carditis；therefore，　treatment　was　switched　to　oral steroids　and　high-dose　aspirin．　Thereafter，　echocardiography　findings　and　the　BNP　level　gradually　improved． On investigation，　we　found　that　inflammatory　cytokines　such　as　interleukin-6　and　interferon-γ decreased　after administering　NSAIDs，　whereas　vascular　endothelial　growth　factor（VEGF）increased．　VEGF　decreased　after administering　steroids，　suggesting　that　VEGF　may　be　useful　for　a　biomarker　of　carditis　as　a　complication　of rheumatic　fever．

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Prognosis　of juvenile　dermatomyositis（JDM）is　usually　better　than　that　of　adult　dermatomyositis because　of good　response　to　steroid　and　less　complications　such　as　malignant　tumor　and　interstitial　pneumonia． We　report　the　case　of　a　13-years-old　boy　treated　with　high-dose　intravenous　immunoglobulin（IVIG). He　was steroid-resistant，and　manifested　dysphasia　and　anarthria．　Clinical　symptoms　and　laboratory　findings　improved immediately　after　treatment　of　high-dose　IVIG　and　he　has　been　in　remission　for　2　years　so　far．　High-dose　IVIG may　be　one　of　the　treatment　strategies　for　refractory　JDM　with　dysphasia　and　anarthria．

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There are only few reports about macrophage activation syndrome (MAS) in the early phase of juvenile dermatomyositis (JDM). We describe an 8-year-old boy who was diagnosed as having JDM with the manifestation of MAS. He developed fever, blisters on his cheeks, and oral and lip aphtha. One month after from his first symptoms, his serum ferritin significantly elevated (2366ng/mL) without cytopenia. After a period of normal temperature of 2 weeks, remittent fever appeared to him again with skin rashes on his cheeks, femoral pain and leukopenia. There was no apparent muscle weakness but mild pressure pain at the posterior region of his thigh near the knees. Gadolinium-enhanced MRI of his thigh revealed fasciitis at the pressure pain region, and the skin pathology of the rash was consistent with JDM. We diagnosed him as having JDM with MAS caused by hypercytokinemia. Medications including methylprednisolone pulse therapy and oral methotrexate were administered, and since then he is stable without a relapse. MAS can develop not only in juvenile idiopathic arthritis but also in other juvenile rheumatic disorders. In this case, as the skin manifestation and muscle weakness was slight, skin pathology and gadolinium-enhanced MRI was useful for the diagnosis.

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