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DOI： 10.2169/internalmedicine.E004-21

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Objective To evaluate the effectiveness and drug retention rate of golimumab (GLM) for long-term use in daily practice for patients with rheumatoid arthritis (RA). Methods Patients with RA who started GLM therapy with a minimum follow-up period of 52 weeks were included. The patients were divided into a biologic-naïve group and switch group. The disease activity score (DAS) 28-erythrocyte sedimentation rate (ESR) (DAS28-ESR), grip power, and Japanese version of the health assessment questionnaire (J-HAQ) score were assessed. In addition, the treatment continuation rate was evaluated at the final follow-up. Patients Sixty-five patients (58 women and 7 men; median [range] age, 69 [61-74] years; median [range] disease duration, 9 [5-16] years) were included. Twenty-eight patients were biologic-naïve (naïve group), and 37 were switched to biologics (switch group). Results The median (range) follow-up period was 134 (58-162) weeks. The DAS28-ESR improved from a median (range) of 4.31 (3.52-5.25) to 2.65 (2.28-3.77) in the naïve group and from 4.27 (3.19-4.89) to 2.89 (2.49-3.88) in the switch group. The grip power improved in both groups (p<0.01); however, the J-HAQ score showed no marked improvement in either group. The continuation rates were 22/28 (78.6%) in the naïve group, and 26/37 (70.3%) in the switch group at the final follow-up. Conclusion We herein report for the first time that the long-term use of GLM improves the grip power. Improving the grip power may help prevent sarcopenia and frailty in the future. Given the efficacy and high continuation rate, we suggest that GLM would be a well-tolerated treatment option for RA.

DOI： 10.2169/internalmedicine.5381-20

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Objectives: The aim of this study was to identify the risk factors associated with severe infection in RA patients, with a particular focus on the association of the nutritional status.Methods: We retrospectively analyzed data from 74 patients with RA (male, n = 21; female, n = 53; age 74.2 ± 12.4) admitted to our hospital between 2016 and 2017 for infection (infection group). We also recruited control RA patients (n = 222) who were matched for age, gender and disease duration, with a match ratio of 1:3 (non-infection group). The nutritional condition was assessed based on controlling nutrition status (CONUT) score, and prognostic nutritional index (PNI). The data of the infection group were obtained from the most recent visit prior to the present admission, and non-infection group from the last regular visit in 2017.Results: The respiratory tract was the most frequent site of infection. The BMI and PNI were significantly lower and the CONUT score significantly higher in the infection group than in the non-infection group. A logistic regression analysis revealed that the CONUT score, underlying lung disease and use of prednisolone and biological disease-modifying anti-rheumatic drugs were independent and significant risk factors for serious infection.Conclusion: Poor nutritional status increases the risk of serious infection.

DOI： 10.1080/14397595.2019.1681653

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Language：English   Publisher：(一社)日本臨床リウマチ学会  

目的:関節リウマチ(RA)患者における関節オーバーユースの発症状況とリスク因子を調査する。方法:関節に負荷がかかるイベントの後に腫脹もしくは疼痛を生じた患者を関節オーバーユースと定義した。2016年8月から10月に当院外来を受診し関節オーバーユースと診断されたRA患者の患者背景、疾患活動性の変化を調査し、年齢、性別をマッチさせた123名のRA患者と比較しリスク因子を調査した。結果:41名が関節オーバーユースと診断された。Clinical disease activity indexの中央値はオーバーユースにより5.0から9.0に上昇し、オーバーユース後には4.80に低下した。オーバーユースを発症する患者の背景因子をロジスティック回帰分析で検索したところ、生物学的製剤の使用、Health assessment questionnaire disability index(HAQ-DI)が低値であること、Steinbrocker Stage III、IVであること(各々Odds ratio 3.10、95% confidence interval 1.24-7.76;0.27(0.09-0.80);5.90(2.28-15.2))が抽出された。結論:生物学的製剤使用者、HAQ-DI低値患者、Stage進行患者はオーバーユースを発症しやすく、患者教育が重要となる可能性がある。(著者抄録)

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Language：Japanese   Publisher：(一社)日本リウマチ学会  

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Hepcidin, a major regulator of iron metabolism and homeostasis, is regulated by inflammation. Recent studies have suggested that hepcidin and iron metabolism are involved in osteoporosis, and the aim of this study was to determine whether serum hepcidin levels are correlated with the degree of osteoporosis in patients with rheumatoid arthritis (RA). A total of 262 patients with RA (67.5 ± 11.4 years; 77.5% female) were enrolled. Serum iron, ferritin, and hepcidin levels were positively correlated each other. Multiple regression analyses revealed that the serum iron level was positively correlated with femoral T and Z scores, whereas the serum hepcidin level was not. Serum hepcidin level was correlated with the serum 25-hydroxy vitamin D level, which was in turn positively related to the femoral Z score. Serum hepcidin and serum iron were indirectly and directly related to osteoporosis in patients with RA.

DOI： 10.1038/s41598-020-66945-3

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Language：Japanese   Publisher：(一社)日本臨床リウマチ学会  

目的:高齢発症関節リウマチ(EORA)は発症時の疾患活動性が高く、内科的合併症も多いことから治療が困難となりやすい。セルトリズマブペゴル(CZP)はC-OPERA試験では発症早期症例に対する有用性が示されたが、比較的若年者(平均年齢49.4歳)での検討であり高齢発症例での検証はなされていない。EORAにおけるCZP早期治療の有用性を検討する。対象・方法:65歳以上のEORA患者で抗リウマチ薬開始から3ヵ月以内にCZP投与を開始した14例(男性9例、年齢77.5(71.5-83.0)歳)を対象とした。診療録を後方視的に調査し開始時、開始後4週、12週、24週における臨床項目を評価した。結果:CZP開始時の罹病期間は3(2-4)ヵ月、抗リウマチ薬開始からの期間は17.5(13.3-23.0)日だった。MTX併用は3例、PSL使用量は5.0(1.25-5.0)mg/日、併存症として間質性肺炎を2例、糖尿病を6例、慢性腎臓病を5例認めた。CZP治療開始前に比し、治療開始後4週で、DAS28-ESR(5.5(4.9-6.3)vs.3.4(2.5-4.1)、p=0.001)、HAQ-DI(1.3(0.7-2.0)vs.0.1(0-0.7)、p=0.001)は有意に低下した。24週以内に2例が二次無効のため他剤に変更となった。結論:CZPは高齢者においても発症早期における治療として疾患活動性の改善と生活機能の改善に有用であった。(著者抄録)

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Language：Japanese   Publisher：(一社)日本臨床リウマチ学会  

【目的】レミチェックQはインフリキシマブ(IFX)血中濃度を1.0μg/mL以上か未満であるか定性的に調べることができるキットである。関節リウマチ(RA)でIFXを投与している患者群でレミチェックQを使用しIFXの血中濃度を推定し、実臨床におけるIFX血中濃度と疾患活動性の関連について検討を行った。【対象・方法】2017年10月から2018年4月の期間で、新潟県立リウマチセンター通院中のRA患者でIFXを投与されレミチェックQの検査を施行された57例を対象とした。検査施行時の患者背景、疾患活動性、IFX投与量等を横断的に解析した。【結果】レミチェックQ陽性群が44例(77%)であった。陰性群では陽性群と比較しIFX投与量は有意に少なかった(6.8±2.6mg/kg vs.5.1±2.4mg/kg、p=0.032)。DAS28-ESR(2.67±1.13vs.2.48±0.44、p=0.775)では有意差はなかったがCDAI(6.6±6.5vs.3.1±2.7、p=0.049)、SDAI(6.9±6.8vs.3.2±2.7、p=0.048)は陰性群で有意に低かった。陰性の13例では全例でDAS28-CRPで寛解を達成し5例でBoolean寛解も達成していた。【結論】レミチェックQ陰性群では陽性群と比較し疾患活動性が良好な症例が集積していた。これは、レミチェックQ陰性群に、TNF製剤への反応性が良い症例が多く存在していた可能性が一因として考えられる。今後は、IFX開始早期に治療効果不十分な症例でレミチェックQを測定しその有用性を検討する必要がある。(著者抄録)

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Language：Japanese   Publisher：中部リウマチ学会  

1991年の関節リウマチ429例と2018年の関節リウマチ368例を対象に、年齢や性を比較した。その結果、2018年に70歳代が4倍、80歳以上が15倍に増加し、男性が有意に増加していた。次に、2018年の368例の腎機能障害を調査した。65歳以上発症108例(A群)、65歳未満発症260例(B群)に分類し、更にB群で65歳未満発症で65歳以上の症例129例(C群)を抽出し、3群間で腎機能を比較した。その結果、発症時の血算や血清Cr、尿異常(血尿、蛋白尿等)は3群間に差がなかった。eGFR、抗CCP抗体はB群がA群、C群より有意に高かった。RFはB群がA群より有意に高かったが、力価に差はなかった。AAアミロイドーシスを全体の1.6%に認め、全例がC群であり、C群の4.7%を占めた。治療はステロイドの使用頻度、量ともに3群間に差はなかったが、メトトレキサートとbDMARDsの使用頻度、量はB群がA群、C群より有意に多かった。

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Language：English   Publisher：(一社)日本臨床リウマチ学会  

【目的】アログリプチンのステロイド糖尿病における有効性、自己免疫性疾患領域での安全性につき検討する。【方法】新潟県立リウマチセンターでプレドニソロン(PSL)使用中の自己免疫疾患患者で、2011〜2017年10月末までにアログリプチンを使用された患者を対象に後ろ向き観察研究を行った。欠損値はLOCF法で補完しWilcoxon signed-rank検定で解析した。【結果】74名が解析対象となり、アログリプチン開始から24週後にHbA1cは有意に低下していたがPSL投与量も有意に低下していた。PSL・糖尿病薬投与量が一定だった患者からなる群(n=57)でもHbA1cは24週後に有意に低下していた。PSL・抗リウマチ薬投与量が一定であった関節リウマチ(RA)患者からなる群(n=22)では、観察期間中の疾患活動性に有意差はなく、関節痛悪化による脱落はなかった。2例で関節炎悪化と主治医に判断されていたが、いずれもアログリプチンの中止には至っていなかった。【総括】関節リウマチ症例ではアログリプチン投与後の関節炎悪化を念頭に関節所見の追跡が重要と考えられた。(著者抄録)

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Language：Japanese   Publisher：(一社)日本臨床リウマチ学会  

【目的】イグラチモド(IGU)は2012年に薬価収載された新規抗リウマチ薬である。IGUの腎機能に与える影響を検討する。【対象・方法】新潟県立リウマチセンターで2015年から2016年の間にIGUを処方開始した201例(男性58例、女性143例、年齢68(60-77)歳)で開始前、開始後3ヵ月、6ヵ月の臨床データを2017年12月に診療録から後方視的に調査した。IGUが中止された症例では中止時と中止後3ヵ月のデータを調査した。【結果】開始後3ヵ月で関節リウマチの疾患活動性は有意に低下した(DAS28-ESR4.1(3.2-4.8)v.s.3.2(2.3-4.1)、p<0.001)。estimated glomerular filtration rate(eGFR)は開始前と比較して3ヵ月後で有意に低下した(eGFR77.3(63.9-91.2)、68.3(54.8-81.4)、p<0.001)。6ヵ月以内に51例がIGUを中止していたが、7例は腎機能低下が中止原因だった。中止例では中止時に比較して中止後3ヵ月でeGFR値は有意に上昇した(eGFR 70.7(57.6-79.7)v.s.75.7(63.4-88.7)、p=0.002)。多変量解析ではeGFR10ml/min以上低下に関わるリスク因子として非ステロイド性抗炎症薬(NSAIDs)の併用ありが抽出された。【結論】重篤な腎機能障害は少なかったが、IGU開始後、有意にeGFR値は低下した。IGU使用時には肝障害だけでなく腎障害にも留意する必要がある。しかし中止後は回復がみられており、可逆性の変化であることが示唆された。(著者抄録)

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Language：Japanese   Publisher：(一社)日本リウマチ学会  

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Language：Japanese   Publisher：(一社)日本リウマチ学会  

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OBJECTIVES: To clarify systemic effects of orthopedic surgical intervention in patients with rheumatoid arthritis (RA). METHODS: A prospective observational cohort study was performed in RA patients who were scheduled to have primary elective orthopedic surgeries. Assessments were performed at baseline, 6 and 12 months after surgery using J-HAQ, General Health, EQ-5D, BDI-II, DAS28-CRP(4) and CRP for all registered patients, DASH and grip power for patients with upper-extremity surgeries, TUG for patients with lower-extremity surgeries, and JSSF for patients with ankle and forefoot surgeries. RESULTS: There were 294 sites in 276 patients whose average age was 64 (19-89) years and average disease duration was 16 (1-60) years. Surgical site was shoulder in six patients, elbow in 26, wrist in 74, hand in 63, hip in 13, knee in 50, ankle in 12, and forefoot in 50. In total, physical function (J-HAQ, grip power, DASH, TUG, JSSF), quality of life (J-HAQ, General Health, EQ-5D) and depression (BDI-II) improved and disease activity (CRP, DAS28-CRP(4)) decreased significantly 6 and 12 months after surgery (p<.01), despite some differences in their outcomes by the preoperative disease activity and the surgical site. CONCLUSION: Overall benefits were provided by orthopedic surgical intervention generally in patients with RA.

DOI： 10.1080/14397595.2018.1457468

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Language：Japanese   Publisher：(一社)日本リウマチ学会  

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Objectives The present study was performed with the aim of analyzing the biological disease-modifying antirheumatic drug (bDMARD)-free (Bio-free) condition of adalimumab (ADA)-treated rheumatoid arthritis (RA) patients in a real-world setting. Methods ADA was used in the treatment of 130 (male, n=21; female, n=109 females) RA patients. Among them, 26 patients (20.0%) discontinued ADA due to a good response. We analyzed 20 patients who were followed up for more than 6 months after the discontinuation of ADA. The Disease Activity Score 28 based on C-reactive protein (DAS28-CRP) and modified health assessment questionnaires (mHAQs) were evaluated. Results The mean age of the patients was 53.4±11.1 years. The mean disease duration was 4.5±4.3 years. Sixteen patients were bDMARD-naïve, while 4 switched from bDMARDs to ADA. At 6 months after the discontinuation ADA, 19 patients had achieved a clinical remission, and 1 had achieved a low disease activity. The Bio-free period was 26.4±15.5 months. The dose of prednisolone was significantly reduced from baseline (3.45±3.17 mg/day) at 6 months after the discontinuation of ADA (2.63±2.78 mg/day). The dose of methotrexate was unchanged. The number of conventional synthetic DMARDs (csDMARDs) was significantly increased (0.8±0.6 to 1.4±1.06). The mHAQ values were significantly ameliorated by ADA and remained good in patients with a Bio-free condition. A multivariate analysis showed that the dose of methotrexate (MTX) was an important factor for achieving a Bio-free condition. Conclusion A sustainable Bio-free condition in a real clinical setting can be achieved and may be a suitable way of reducing medical costs. The dose of MTX and the additional administration of csDMARDs is therefore thought to be important for ensuring a good outcome in these patients.

DOI： 10.2169/internalmedicine.1332-18

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Language：Japanese   Publisher：(一社)日本内科学会  

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Language：Japanese   Publisher：新潟医学会  

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Other Link： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2018&ichushi_jid=J00990&link_issn=&doc_id=20200130080005&doc_link_id=%2Fdg3nigta%2F2018%2Fs13211%2F002%2F0397-0398%26dl%3D0&url=http%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fdg3nigta%2F2018%2Fs13211%2F002%2F0397-0398%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

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Objective To investigate the efficacy of minodronate in the treatment of glucocorticoid-induced osteoporosis (GIO). Methods The study population included patients in whom the administration of minodronate (50 mg, once every 4 weeks) had been newly started for the treatment of GIO in Niigata Rheumatic Center from 2012 to 2015. Patients who were bisphosphonate-naïve and those who switched from other bisphosphonates were classified into the naïve and switch groups, respectively. The changes in the bone mineral density (BMD) and bone metabolic markers after one year of minodronate treatment were retrospectively evaluated. We also compared the BMD and bone turnover marker changes of minodronate-naïve patients with those in whom alendronate or risedronate had been prescribed as a first bisphosphonate (control group). Results Minodronate was prescribed to 142 patients, and data were successfully obtained from 120 patients. New vertebral fractures were observed in 5 of the 142 patients; 1 fracture occurred during the cessation of minodronate for dental treatment, and 3 patients already had multiple vertebral fractures before the initiation of minodronate. The patients' tartrate-resistant acid phosphatase 5b (TRACP-5b) (-27.0%, p<0.001) and bone alkaline phosphatase (BAP) (-15.7%, p<0.01) levels were decreased, but no patients showed a decrease to below the normal range. One year of treatment with minodronate significantly increased the lumbar BMD in the naïve (+3.9%, p<0.001) and switch (+2.3%, p<0.001) groups. Although the femoral BMD did not change to a significant extent overall, the patients with a low young adult mean (YAM) (<80%) at baseline showed a significant increase in their femoral BMD (+2.1%, p=0.034) values. Compared with the control group, the minodronate-naïve group showed a significant decrease in the TRACP-5b levels and a significant increase in the lumbar BMD. Conclusion The administration of minodronate appears to be an effective treatment for GIO.

DOI： 10.2169/internalmedicine.9885-17

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Language：Japanese   Publisher：新潟医学会  

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OBJECTIVES: The intensification of infliximab (IFX) treatment, involving escalation of the dose and shortening of interval, was approved in Japan in July 2009. We consider IFX intensification therapy to be preferable for patients with treatment-resistant active rheumatoid arthritis (RA). We retrospectively compared the efficacy of IFX with that of other bDMARDs in methotrexate (MTX)-resistant patients. METHODS: Patients who satisfied the following criteria were enrolled: (i) those who started bDMARDs between February 2011 and December 2016, and (ii) those who required bDMARDs after 180 d of MTX treatment. We compared 33 patients who had been treated with IFX (IFX group) and 146 who had received other bDMARDs treatment (non-IFX group). RESULTS: IFX was administered at a dose of 6.98 mg/kg/8-week equivalent at 52 weeks. Clinical disease activity index clinical remission (CDAI-CR) was achieved in 49 of the 179 patients at 52 weeks and 13 of these 49 patients received IFX. Logistic regression analysis showed that treatment with IFX was an important variable for the achievement of CDAI-CR at 52 weeks (odds ratio 2.69, 95% confidence interval 1.13-6.42). The severity and frequency of adverse events did not differ. CONCLUSION: Intensification of IFX was effective and well tolerated for MTX resistant patients.

DOI： 10.1080/14397595.2017.1380250

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Language：Japanese   Publisher：(一社)日本腎臓学会  

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Language：Japanese   Publisher：(一社)日本リウマチ学会  

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Language：Japanese   Publisher：(一社)日本リウマチ学会  

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Language：Japanese   Publisher：(一社)日本リウマチ学会  

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Language：Japanese   Publisher：(一社)日本リウマチ学会  

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Language：Japanese   Publisher：(一社)日本リウマチ学会  

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Language：Japanese   Publisher：(公社)日本整形外科学会  

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Language：Japanese   Publisher：(株)医薬情報研究所  

目的:膠原病・リウマチなどの患者におけるレイノー現象・冷え症に対する当帰四逆加呉茱萸生姜湯の検討 対象と方法:当帰四逆加呉茱萸生姜湯を7.5g/日、3回食前に投与されていた、レイノー現象・冷え症を有する39例(男性7例、女性32例、62.0±13.9歳)についてレトロスペクティブに検討した。原疾患は限局性強皮症が13例[うち原発性胆汁酸肝硬変合併2例、関節リウマチ(RA)合併1例、シェーグレン症候群(SS)合併1例]、全身性強皮症7例、RA6例(限局性強皮症合併例は限局性強皮症にカウント)、原発性SS3例、全身性エリテマトーデス、多発性筋炎、混合性結合組織病それぞれ1例ずつ、特定の膠原病の診断名確定せず7例であった。抗核抗体陽性(×40以上)は30例で、抗セントロメア抗体陽性13例、抗Scl-70抗体陽性7例、抗SS-A抗体陽性5例(うち2例は抗SS-B抗体陽性)、抗DNA抗体陽性、抗RNP抗体陽性が1例ずつであった。効果判定は、患者の自覚症状の改善、継続希望と主治医判断で行った。結果:25例(64.1%)が有効、3例(7.7%)がやや有効であった。4例(10.3%)が無効で中止し、さらに苦み、辛み、口の中がボロボロする、といった忍容性の問題で4例(10.3%)が、副作用(動悸、食思不振、嘔吐)で3例(7.7%)が内服を中止した。考察:抗セントロメア抗体や抗Scl-70抗体などが陽性の患者が対象であったが、他の薬剤の多剤使用が無効時に、当帰四逆加呉茱萸生姜湯は有効であると考えられた。本剤は安価であり、医療経済的にも優れていると考えられた。結語:膠原病・リウマチなどの患者のレイノー現象・冷え症に対し当帰四逆加呉茱萸生姜湯は有用であった。(著者抄録)

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Language：English   Publishing type：Research paper (scientific journal)  

Purpose TAFRO syndrome is a novel disorder manifesting as fever, anasarca, thrombocytopenia, renal insufficiency and organomegaly, and its etiology has not been clarified. The aim of this study was to elucidate similarities and differences between systemic lupus erythematosus (SLE) and TAFRO syndrome. Methods We examined 46 consecutive patients diagnosed with SLE and determined whether they meet the proposed diagnostic criteria for TAFRO syndrome (2015 version). Results Of the 46 patients with SLE, four (8.7%) also met the TAFRO syndrome criteria (TAFRO-like group). All patients in the TAFRO-like group were males, and their mean age was significantly higher than that of the non-TAFRO group (67.5 ± 8.7 vs. 39.3 ± 18.1 years, p = 0.004). C-reactive protein and γ-glutamyl transpeptidase levels were significantly higher, and frequencies of anti-dsDNA and anti-Sm antibodies were significantly lower in the TAFRO-like than non-TAFRO group. Elder cases (onset age ≥ 50 years) met significantly more categories of the diagnostic criteria for TAFRO syndrome than did those with younger cases. Conclusions Several patients with SLE, especially elder cases, showed features similar to those of TAFRO syndrome. Although exclusion of SLE is needed in the diagnostic criteria for TAFRO syndrome, TAFRO syndrome-like SLE should be considered.

DOI： 10.1177/0961203317725589

PubMed

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Language：Japanese   Publisher：(一社)日本臨床リウマチ学会  

目的:アダリムマブ皮下注製剤(ADAsc(0.8))は注射時痛が比較的強い製剤であったが、2016年11月に注射時痛軽減が期待できるADAsc(0.4)が発売された。今回ADAsc(0.4)を中心に、各種皮下注生物学的製剤の注射時痛の比較検討を行った。対象・方法:当院通院中の皮下注生物学的製剤を使用中の関節リウマチ患者のうち同意が得られた423例(ADAsc群72例、他製剤351例)に対し注射時痛のアンケート調査を行った。結果:ADAsc製剤変更前後で疾患活動性に統計学的有意な変化は認めなかった。注射時痛のShort-Form McGill Pain Questionnaire version 2(SF-MPQ-2)スコアは、4.50(1.00-19.0)から1.00(0.000-5.00)点(p<0.001)に改善した。穿刺時痛は55.4%、薬液注入時痛は78.6%の患者がADAsc(0.4)の方がとてもよいと回答した。重回帰分析で注射時痛が高値(SF-MPQ-2スコア3点以上)となる因子を検討したところ、ADAsc(0.4)と比較してADAsc(0.8)、アバタセプト、エタネルセプト、セルトリズマブペゴルでオッズ比が高かった。結論:注射時痛が改善したことにより、ADAscはさらに使用しやすい製剤となった。(著者抄録)

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Other Link： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2017&ichushi_jid=J02269&link_issn=&doc_id=20180319110004&doc_link_id=10.14961%2Fcra.29.251&url=https%3A%2F%2Fdoi.org%2F10.14961%2Fcra.29.251&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_3.gif

Language：Japanese   Publisher：新潟医学会  

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Other Link： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2017&ichushi_jid=J00990&link_issn=&doc_id=20170707020018&doc_link_id=%2Fdg3nigta%2F2017%2Fs13102%2F012%2F0131-0131%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fdg3nigta%2F2017%2Fs13102%2F012%2F0131-0131%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

Language：English   Publishing type：Research paper (scientific journal)   Publisher：JAPAN SOC INTERNAL MEDICINE  

Chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory bone disorder that generally occurs in children and predominantly affects the long bones with marginal sclerosis. We herein report two cases of adult-onset CRMO involving the tibial diaphysis bilaterally, accompanied by polyarthritis. Magnetic resonance imaging (MRI) showed both tibial osteomyelitis and high intensity of the extensive lower leg muscles. Anti-interleukin-6 therapy with tocilizumab (TCZ) effectively controlled symptoms and inflammatory markers in both patients. High intensity of the lower leg muscles detected by MRI also improved. These cases demonstrate that CRMO should be included in the differential diagnosis of adult patients with bone pain, inflammation, and high intensity of the muscles detected by MRI. TCZ may therefore be an effective therapy for muscle inflammation of CRMO.

DOI： 10.2169/internalmedicine.8473-16

Web of Science

PubMed

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：TAYLOR & FRANCIS LTD  

The kidney is a major target organ for systemic amyloidosis, which results in proteinuria and an elevated serum creatinine level. The clinical manifestations and precursor proteins of amyloid A (AA) and light-chain (AL) amyloidosis are different, and the renal damage due to amyloid deposition also seems to differ. The purpose of this study was to clarify haw the difference in clinical features between AA and AL amyloidosis are explained by the difference in the amount and distribution of amyloid deposition in the renal tissues.
A total of 119 patients participated: 58 patients with an established diagnosis of AA amyloidosis (AA group) and 61 with AL amyloidosis (AL group). We retrospectively investigated the correlation between clinical data, pathological manifestations, and the area occupied by amyloid in renal biopsy specimens. In most of the renal specimens the percentage area occupied by amyloid was less than 10%. For statistical analyses, the percentage area of amyloid deposition was transformed to a common logarithmic value (Log(10)%amyloid). The results of sex-, age-, and Log(10)%amyloid-adjusted analyses showed that systolic blood pressure (SBP) was higher in the AA group. In terms of renal function parameters, serum creatinine, creatinine clearance (Ccr) and estimated glomerular filtration rate (eGFR) indicated significant renal impairment in the AA group, whereas urinary protein indicated significant renal impairment in the AL group. Pathological examinations revealed amyloid was predominantly deposited at glomerular basement membrane (GBM) and easily transferred to the mesangial area in the AA group, and it was predominantly deposited at in the AL group. The degree of amyloid deposition in the glomerular capillary was significantly more severe in AL group. The frequency of amyloid deposits in extraglomerular mesangium was not significantly different between the two groups, but in AA group, the degree amyloid deposition was significantly more severe, and the deposition pattern in the glomerulus was nodular. Nodular deposition in extraglomerular mesangium leads to renal impairment in AA group.
There are significant differences between AA and AL amyloidosis with regard to the renal function, especially in terms of Ccr, eGFR and urinary protein, even after Log10%amyloid was adjusted; showing that these inter-group differences in renal function would not be depend on the amount of renal amyloid deposits. These differences could be explained by the difference in distribution and morphological pattern of amyloid deposition in the renal tissue.

DOI： 10.1080/13506129.2017.1338565

Web of Science

PubMed

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