全身性エリテマトーデス（systemic lupus erythematosus：SLE）に浮腫を伴う場合，ループス腎 炎に起因することが多い．また漿膜炎から心嚢および胸腹腔に腔水症がみられることも度々ある． 今回我々は，全身浮腫を伴う腔水症が顕著であり，心嚢および腹部ドレナージを含めた集中治療 を要したSLEの一例を経験した．症例は8歳女児．6歳時にSLEと診断され，ステロイドパルス療法 および免疫抑制療法が行われたが，寛解には至らなかった．経過中，発熱，呼吸不全にて2回入院（経 過①，②）となった．経過①では胸腹水による呼吸不全で非侵襲的陽圧換気を要し，経過②では呼 吸管理に加えて，腔水症の管理目的に心嚢および腹部ドレナージを必要とした．全身浮腫の所見 と同時期に認めた所見は，血小板減少，発熱，肝脾腫，全身のリンパ節腫脹，急速な腎不全の進 行であり，TAFRO症候群を想起させた．現在TAFRO症候群の定義ではSLEを含めたリウマチ性 疾患は除外基準に該当するが，SLEが背景にあってもTAFRO症候群を発症する可能性があると考 えられた．

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IgA vasculitis sometimes causes acute scrotum, but in many cases surgical treatment is not necessary. We report a case of unilateral orchiectomy because of acute scrotum due to IgA vasculitis. A 4-year-old boy was admitted to our hospital with a diagnosis of IgA vasculitis because he had abdominal pain, ankle pain, and purpura. These symptoms disappeared with administration of prednisolone（ PSL）. On the 8th day after onset while PSL was gradually being tapered, pain and swelling of the left scrotum suddenly appeared. His left testis became dark red and was diagnosed macroscopically as necrotic. Because we could not distinguish his symptoms from testicular torsion, an emergency orchiectomy was performed. On the 11th day, the patient experienced right scrotal pain. Since imaging findings showed normal blood flow to the right testis, the patient was treated conservatively. The scrotal symptoms disappeared, and the right testis was preserved. Pathological findings on the left testis showed vasculitis and hemorrhage without necrotizing changes. Many patients with acute scrotum associated with IgA vasculitis do not have damage to the testis, but in rare cases this disease causes serious vasculitis in the testis. Immediate examination by imaging and conservative treatment are important when acute scrotum develops during the course of IgA vasculitis, but surgical intervention should also be performed if the diagnosis is unclear.

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We report the case of Kawasaki disease （KD） complicating Immune thrombocytopenic purpura （ITP）. A 1-year-old girl with fever, rash, bilateral conjunctival injection, injected lips and pharynx, cervical lymphadenopathy, erythema of the palms or soles and swelling of hands and feet was referred to us with thrombocytopenia （1.6 × 104/μL） on the 3rd day after the onset of fever. The diagnosis of KD was made. Treatment with intravenous immunoglobulin was started. The symptoms related to KD were all improved but thrombocytopenia persisted. The level of platelet-associated immunoglobulin was elevated. Bone marrow aspiration revealed an increase of immature megakaryocytes. The diagnosis of ITP was made. Treatment with prednisolone was started and thrombocytopenia was gradually improved. Cardiac involvement associated with KD was not observed. Although most patients with KD showed thrombocytosis during the acute phase, some patients showed thrombocytopenia. The rate of thrombocytopenia association in KD has been reported as about 2 %. The cause of thrombocytopenia association in KD were disseminated intravascular coagulation, drug related thrombocytopenia, macrophage activation syndrome and so on. ITP should be considered as a cause of thrombocytopenia in patients with KD, although it might be rare.

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A 4-year-old boy with Kawasaki disease（ KD） was treated with two cycles of intravenous immunoglobulin therapy（ IVIG）, but was resistant to IVIG. Administration of prednisolone（ PSL） led to the resolution of fever and other KD symptoms. After tapering off PSL in 15 days, his fever returned and, new blood tests showed hemocytopenia and the elevation of ferritin levels. A diagnosis of macrophage activation syndrome（ MAS） was made, and intravenous methylprednisolone pulse（ mPSL） therapy was started. We conducted cytokine profile analysis from previous blood samples, and found the serum IL-18 has sustained high level since his disease onset. Based on this finding, systemic juvenile idiopathic arthritis （sJIA） was a speculated complication with KD. We suspected that PSL improved the symptoms of KD and CRP level, but was not effective enough to lower the serum IL-18 levels. Therefore, after tapering off PSL, an IL-18 related inflammation was activated and caused MAS. After shifting to post-treatment of mPSL pulse therapy, we tapered the PSL dosage over several months while monitoring the serum IL-18 levels, and succeeded to end without relapses. sJIA is often difficult to distinguished from KD, especially cases without arthritis. In such situations, monitoring of cytokine profiles may be helpful to diagnose, evaluate patients’ pathophysiology, and select the appropriate treatment strategies.

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Intestinal Behçet’s disease and Crohn’s disease share a clinical presentation of intestinal ulcers, as well as extra-intestinal manifestations such as oral aphthous ulcers, nodular erythema, and arthritis. We present a case who was difficult to distinguish between the two diseases. In addition, we discussed the differences of clinical features of them. The patient is 13-year-old girl. She initially had presented with fever, diarrhea, oral aphthous ulcers, nodular erythema of legs, and knee joint swelling. Gastrointestinal endoscopy revealed multiple ulcers, and histopathological examination showed nonspecific inflammatory cell infiltration. HLA-B51 testing had showed a positive result, and she had been diagnosed with intestinal Behçet’s disease. Although the symptoms improved after treatment with prednisolone, she developed a relapse accompanied with endoscopic histopathological findings of longitudinal ulcers, a cobblestone appearance of the mucosa, and non-caseating granuloma leading to a diagnosis of Crohn’s disease. We could not exclude the possibility that an allergic reaction to mesalazine altered her treatment course. We speculated that she initially had been diagnosed as intestinal Behçet’s disease for the evidences of no specific endoscopic findings of Crohn’s disease, extra-intestinal manifestations like as Behçet’s disease, HLA-B51 positive, and failure to reduce temperature by nutritional therapy. We suppose that Crohn’s disease seemed like as intestinal Behçet’s disease from the confusing clinical features.

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Lymphocytic infundibulo-neurohypophysitis（LINH） is a rare disease that exhibits central diabetes insipidus（CDI） through autoimmune mechanisms. Importantly, LINH should be always differentiated from neoplasia such as Langerhans cell histiocytosis and germinoma. A 6-year-old boy presented with polyuria and polydipsia. Endocrinological investigations proved CDI. Hypophysis MRI showed enlargement of the stalk and diffuse Gadrinium-enhancement, suggesting the diagnosis of LINH. Hypophysis biopsy that is indispensable for the definitive diagnosis of LINH was not performed in our patient because of its invasiveness. However, the follow up MRI revealed the improvement of the stalk swelling in the time course, and this finding not only supported the diagnosis but also ruled out neoplastic diseases. He was treated with DDAVP alone, and his CDI promptly dissolved. Since he lacked headache and visual disorders, glucocorticoid treatment was not needed. Recently, serum anti-Rabphylin-3A antibody has been reported as a useful diagnostic tool for LINH. Our patient also possessed this antibody in the serum, strongly supporting the diagnosis.

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We experienced a case of chronic recurrent multifocal osteomyelitis（ CRMO） characterized by discordance between clinical symptoms and serum inflammatory markers（ e.g., C-reactive protein, serum amyloid A, and erythrocyte sedimentation rate） 3 years after disease onset. A 9-year-old girl presented with recurrent pain in multiple bones. A blood examination showed an inflammatory reaction, and an imaging study indicated osteomyelitis. Neither bacteria nor malignancy was detected from a bone biopsy. She was diagnosed with CRMO based on these findings, and remission was maintained with nonsteroidal anti-inflammatory drugs and steroids. After the onset of a stress fracture, she presented with pain in the right tibia and difficulty walking. A blood examination showed no inflammatory reaction, but magnetic resonance imaging and bone scintigraphy confirmed osteomyelitis in the right tibia. The patient was therefore diagnosed with inflammatory reaction- negative CRMO. Despite additional treatment with bisphosphonate, her symptoms did not improve. Infliximab was then started with in-hospital ethics review board permission, and she achieved remission and a return to normal life.

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