A 13-year-old girl was referred to us with a 3-year history of stiffness of bilateral thighs and arthralgia of bilateral knees, and also 2-year history of stiffness of bilateral hands and bilateral shoulder pain. Musculoskeletal ultrasonography revealed polyenthesitis with edematous changes around tendons in upper and lower extremities. 18FFDG PET revealed hot spots in entheses of upper and lower extremities, tendons of bilateral hands and bilateral ischial tuberosities. Magnetic resonance image with fat-suppressed T2-weighted revealed high intensity lesions in entheses and tendons with FDG accumulation. The diagnosis of polyenthesitis was made. Ibuprofen was started but her symptoms were not improved. Oral prednisolone （PSL） was added and her symptoms were improved. However, PSL was discontinued because of steroid myopathy. Recurrence of enthesitis occurred after stopping PSL. Adalimumab was started and finally remission was achieved. Polyenthesitis without synovitis is very rare. Enthesitis has not been recognized as a cause of arthralgia yet. We should remain aware of the possibility of enthesitis in patients with arthralgia.

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Clinical features and outcome of 44 （5 men and 39 women） pediatric-onset collagen diseases were examined. Age at onset and age at final observation were 12.4±2.5 and 36.0±10.6 years old, respectively. Duration from onset to final observation was 11.9±8.9 years. Diagnoses（ there is some overlapping） were 26 of systemic lupus erythematosus（ SLE）, 8 of juvenile idiopathic arthritis, 6 of Sjögren’s syndrome, 5 of mixed connective tissue disease, 3 of polymyositis/dermatomyositis, 2 of Behçet disease, and 1 of systemic sclerosis. Most patients with SLE showed nonspecific manifestations compared with other diseases. SLE patients with facial erythema or proteinuria at disease onset tended to be diagnosed earlier than those with nonspecific symptoms and signs. Steroid pulse and immunosuppressant were frequently used in patients with SLE compared with other diseases. At final observation, 16 patients （37.7±11.1 years old） were visiting our hospital regularly, 17（ 32.5±8.5） were referred to other doctors, 6（ 42.5±10.0） were dead, and 5（ 35.4±9.6） were missing. All 6 fatal cases were SLE. Since pediatric-onset SLE patients are refractory and their outcome is poor, pediatricians and internists should cooperate to improve prognosis of SLE.

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The oral microbiome in a girl with childhood onset primary Sjögren Syndrome （SS） is the focus of this case report. An 8-year-old girl had severe tooth decay, beginning in infancy. She had intermittent fevers, general fatigue, and joint pain from 7 years of age. She was suspected to have SS based on anti- SS-A/Ro titers. A salivary gland disorder was confirmed by the Saxon test, salivary gland scintigraphy, and a lip biopsy. She was diagnosed with primary SS, and prednisolone and azathioprine were administered for extraglandular symptoms, such as fevers and arthralgias. Oral microbiology testing revealed a higher ratio of Streptococcus mutans-to-Streptcoccus spp., one of the known risk factors for caries development, when compared to her siblings, thus suggesting the need for intensive intraoral intervention.

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Antibodies against melanoma differentiation─associated gene 5（ MDA5） are frequently detected in dermatomyositis patients. These cases are often complicated by rapidly progressing interstitial lung disease （ILD） and arthritis/arthralgia. Here, we report a case of a 15-year-old girl with anti-MDA5-positive juvenile dermatomyositis （JDM） who developed polyarthralgia. The patient presented symmetrical polyarthralgia involving small joints of the hands and erythema, and was initially treated for polyarticular juvenile idiopathic arthritis（ JIA）. However, after the development of mild myalgia, she was diagnosed with JDM, complicated by ILD, without respiratory dysfunction. We treated the patient with immunosuppressive therapy including intravenous glucocorticoid pulse, oral cyclosporine, and intravenous cyclophosphamide （IVCY）；and her serum was positive for anti-MDA5 antibodies. Due to hypersensitivity to cyclophosphamide and/or mesna from the fourth infusion of IVCY, antihistamines and glucocorticoids were administered intravenously to reduce the allergy symptoms. Immunosuppressive therapy improved both arthralgia and ILD. In patients with multiple arthralgia/arthritis, anti-MDA5-positive JDM should be differentiated from polyarticular JIA.

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Lupus nephritis, a life-threating condition of systemic lupus erythematosus （SLE）, develops only rarely with acute renal failure presenting with anuria from onset. We treated a girl with SLE who progressed to acute renal failure at the beginning. Because renal failure progressed rapidly, aggressive treatment in addition to methylprednisolone pulse therapy was necessary to induce early remission. After commencement of double filtration plasmapheresis（ DFPP） and multi-target therapy using a combination of prednisolone, mycophenol mofetil, and tacrolimus administration, her proteinuria and serological abnormalities improved. Dose adjustment based on the trough levels of mycophenol mofetil and tacrolimus resulted in no critical complications associated with this therapy. In cases of SLE comorbided with rapidly progressive renal failure, aggressive treatment is essential for early induction and maintenance of remission. The combination of multi-target therapy and DFPP appears to be effective for induction treating severe lupus nephritis in both not only adults but also children.

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Erythropoietic protoporphyria（ EPP） is a congenital metabolic disorder which induces intense pain in the area exposed to sunlight. In some patients, their photosensitivity-like symptoms need to be differentiated from symptoms of rheumatic diseases. In this report, we present two EPP cases that we experienced. Case 1：A 13-year old girl complained of severe pain in the dorsum of hands after a school sport event. Since then, she had repeatedly developed pain in the area exposed to sunlight. At the first visit, the patient presented with brown pigmentation on her cheeks and slight redness on the skin of the dorsum of her finger joints. There was no other abnormality observed. A diagnosis of EPP was made based on the increased level of protoporphyrin in the red blood cells and FECH mutation, which already had been reported. Case 2：A 8-year old boy who had repeatedly complained of severe pain and swelling in the finger tips and nails after sun exposure since age 6 for unknown reasons. He developed juvenile idiopathic arthritis（ JIA） at the age of 8 and visited our clinic. Brown pigmentation and shallow scars were observed on his cheeks. The patient was diagnosed with EPP from the increased level of protoporphyrin in the red blood cells and FECH mutation. It is important to consider EPP as a differential diagnosis when we see a child complaining of severe pain in the area exposed to sunlight.

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Hereditary periodic fever syndromes are characterized by short and recurrent attacks of fever and severe localized inflammation that is caused by autoinflammation. These attacks are intermittent and selflimiting, therefore early detection is difficult, resulting in delay in the diagnosis. Here, we report the early diagnosis and treatment of familial Mediterranean fever（ FMF） in twin 5-year-old girls. The first twin had recurrent episodes of fever accompanied by pain in chest, stomach and back. Blood tests showed high CRP and IgG. A CT scan showed pleural effusion and ascites, which suggested serositis. Colchicine treatment was started 5 months after onset and was effective at preventing attacks, thus leading to the diagnosis of FMF. Two month later, the second twin was found to suffer from recurrent headache, arthralgia, abdominal pain and fatigue. Her symptoms responded to colchicine, resulting in diagnose of FMF. We analyzed the Mediterranean fever gene （MEFV） of the twin and detected previously reported mutations, E148Q/P369S/R408Q. Various phenotypes of FMF can occur with the same MEFV mutation within the same family. Interestingly, the onsets of FMF in the twins occurred around the same time and early diagnosis of the first twin led to earlier diagnosis of the second twin.

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