リウマチ

Jais X et al. Immunosuppressive therapy in lupus- and mixed connective tissue disease-associated pulmonary arterial hypertension: a retrospective analysis of twenty-three cases.
Arthritis Rheum. 2008 Feb;58(2):521-31（フランス）.

　これまでの研究で強皮症合併肺高血圧が免疫抑制療法に反応しないが、SLEまたはMCTDに合併した肺高血圧の一部は免疫抑制療法に反応して改善することが知られている。
　この論文でJaisらは、SLEまたはMCTDに合併した肺高血圧16例をステロイド治療した場合、半数が改善する（軽症肺高血圧ほどよく反応する）。ステロイド治療に反応しなかた8例中6例で肺血管拡張薬（ボセンタンやエポプロステノールなど）に反応した。
ステロイドと免疫抑制薬（エンドキサン）を併用した7例中4例がレスポンダーであった。

BSR and BHPR guidelines for the management of adults with ANCA associated vasculitis.
Rheumatology (Oxford). 2007 Oct;46(10):1615-6

Treatment of antineutrophil cytoplasmic antibody associated vasculitis: a systematic review.
JAMA. 2007 Aug 8;298(6):655-69

Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis.
J Am Soc Nephrol. 2007 Jul;18(7):2180-8
(The investigators were permitted to use their local procedure with respect to (1) plasma filtration or centrifugation, (2) vascular access, (3) anticoagulation, and (4) daily or alternate-day exchanges. The following aspects of the procedure were mandated in the protocol: (1) A total of seven plasma exchanges within 14 d of study entry, (2) a plasma exchange volume of 60 ml/kg on each occasion, and (3) volume replacement with 5% albumin. The use of fresh frozen plasma at the end of the procedure to replenish coagulation factors was recommended but not mandated for patients who were at risk for hemorrhage, for example after kidney biopsy.)

（１）乾燥肌・皮脂欠乏性皮膚炎　asteatotic dermatitis・"senile dry skin type sjs."・Xerosisーーーputative?

（２）高ガンマグロブリン血症性紫斑

（３）環状紅斑（主に顔面、四肢も）表皮の変化に乏しい

◎　Seo P: Pregnancy and vasculitis. Rheum Dis Clin North Am. 2007 May;33(2):299-317（The Johns Hopkins University）.

・J Gynecol Obstet Biol Reprod (Paris). 1995;24(7):747-50.Links
[Association of Takayasu's arteritis, pregnancy and Still's disease]

　◎　妊娠中に発症したAOSDの2例（20週・22週：ともに除外診断後ステロイド治療）Pan VL, Haruyama AZ, Guberman C, Kitridou RC, Wing DA. Newly diagnosed adult-onset Still disease in pregnancy. Obstet Gynecol. 2003 May;101(5 Pt 2):1112-6（米国）
　◎　PREGNANCY AND RHEUMATOID ARTHRITIS. Rheumatic Diseases Clinics of North America - Volume 23, Issue 1 (February 1997)
　この1997年のレビューはRA合併妊娠に関するものであるが、AOSDについても触れている。To conclude, in all 21 pregnancies reported, no consistent pattern of the interaction between pregnancy and Still's disease emerged. Acute flares can occur during and after pregnancy, requiring high doses of salicylates or prednisone. RAのように妊娠中に軽快するという傾向はなさそうである。
　◎　 E. Liozon, K. Ly, Y. Aubard 1 and E. Vidal. Intravenous immunoglobulins for adult Still's disease and pregnancy. Rheumatology 1999; 38: 1024-1025（フランス）
　この1999年の報告（レター）時点で、著者等は成人スチル病に合併した33妊娠（24症例）の文献報告を引用している。AOSDの治療の主流はグルココルチコイドであり、妊娠合併AOSDにも有効で比較的安全に使えると記載している。彼らの症例はhepatic toxicity of salicylates・excessive weight gain early in pregnancy（a high risk of developing diabetes gestationis）からIVIGが選択された。

その他
・Polyserositis in adult Still's disease with onset during pregnancy(一例報告） Clin Rheumatol. 1994 Sep;13(3):513-7.
・Gerald H. Stein, Bernard Cantor, Richard S. Panush, MD: Adult still's disease associated with pregnancy. Arthritis & Rheumatism Volume 23, Issue 2 , Pages 248 - 250、1980
・Adult-onset Still's disease: A case with onset during pregnancy(一例報告） Arthritis & Rheumatism Volume 28, Issue 8 , Pages 957 - 957,1985

その他
(1) Mok MY, Lo Y, Leung PY, Lau CS（香港）：Pregnancy outcome in patients with adult onset Still's disease. J Rheumatol. 2004 Nov;31(11):2307-9.
Pregnancy outcome of patients with adult onset Still's disease (AOSD) has not been addressed. We report the maternal and fetal morbidity of 5 pregnancies from 3 Chinese patients with AOSD and review another 17 pregnancies from 14 patients reported in the English literature. Nine patients had their first manifestation in the 5th to 6th gestational month. Disease relapse in patients with known AOSDoccurred most commonly in the post-partum period. Most did not respond satisfactorily to nonsteroidal antiinflammatory agents alone, but responded to administration of corticosteroids, especially in high doses.

(2) Le Loët X, Daragon A, Duval C, Thomine E, Lauret P, Humbert G.（フランス）: Adult onset Still's disease and pregnancy. J Rheumatol. 1993 Jul;20(7):1158-61.
The interactions between pregnancy and adult onset Still's disease (AOSD) are unclear. Between 1983 and 1990, 5 pregnancies occurred in 4 women with AOSD. AOSD occurred at the 5th and 6th month of the 1st pregnancy in 2 patients; a 2nd pregnancy occurring in one of them was not associated with a flare. In the other 2 patients pregnancy occurred 36 and 44 months after the AOSD; during gestation only transient arthralgia were observed in both cases. Pregnancy seems to have no effect on AOSD, and conversely, in our experience, AOSD has no influence on pregnancy, fetal growth or infant health.

(3) 成人スチル病合併妊娠の1症例 日本産科婦人科學會雜誌 Vol.48, No.6(19960601) pp. 431-434

Drug Approved. Is Disease Real? By ALEX BERENSON
NY TIMES Published: January 14, 2008

・Lyrica (Pfizer's drug pregabalin which is already a seller for seizures and diabetic nerve damages; Approved by FDA for FM in 2007 June; In November, Pfizer began a television ad campaign for Lyrica) and two other drugs (= Eli Lilly's Cymbalta and Forest Laboratories' milnacipran, both of which were originally developed as antidepressants) that may be approved this year

・Controversial; ACR, FDA and insurers recognize FM as a diagnosable disease. But doctors who are skeptical of FM think that vague complaints of chronic pain do not add up to a disease

Feng H, Shuda M, Chang Y, Moore PS. Clonal Integration of a Polyomavirus (=the seventh virus linked to human cancers) in Human Merkel Cell Carcinoma. Science 2008

They detected a fusion transcript between a previously undescribed virus T antigen and a human receptor tyrosine phosphatase in 80% of MCC vs 8-16% of controls.

Drug Has No Benefit in Trial, Makers Say By ALEX BERENSON
NY TIMES Published: January 14, 2008

New Questions on Treating Cholesterol By ALEX BERENSON
NY TIMES Published: January 17, 2008

・http://www.ach.or.jp/departments/ja/houshasen/topics.html
・http://www.luke.or.jp/shinryo/28_housyasen_2.html
・http://www3.kmu.ac.jp/radiol/pvp/index.html
・http://www.marianna-u.ac.jp/hospital/daigakubyouin/housyasenka.html
・経皮的椎体形成術についての第Ⅰ/Ⅱ相臨床試験 (JIVROSG-0202)症例登録終了(2003/2/3-2006/3/8)
参加施設2006.8月：http://jivrosg.umin.jp/institutions.htm
・http://www.innervision.co.jp/042info/2008/042info08_02_02_2.html

・J Clin Rheumatol. 2007 Dec;13(6):313-6.
Protein-losing enteropathy in systemic lupus erythematosus: analysis of the clinical features of fifteen patients（Zheng WJ et al. Peking Union Medical College）.
Zheng（中国）らの自検例15例の検討では、ほとんどの症例で抗DNA抗体は陰性。

・Intern Med. 2001 May;40(5):449-53. Protein-losing enteropathy exacerbated with the appearance of symptoms of systemic lupus erythematosus (Yoshida M, Miyata M, Saka M, Sasajima T, Sato H, Takiguchi J, Shishido H, Saito A, Kokubun M, Nishimaki T, Kasukawa R).
一例報告だが、RNP/SS-A/Sm (+)だが抗DNA抗体は陰性

・ Rheumatology (Oxford). 2006 Apr;45(4):425-9.
Outcome of protein-losing gastroenteropathy in systemic lupus erythematosus treated with prednisolone and azathioprine　（Mok CC, Ying KY, Mak A, To CH, Szeto ML）.
この報告では抗DNA抗体は75%で陽性。下痢は50%。プレドニンとイムラン併用が有効。

・ヨンセイのParkらは11才の小児SLEに合併したPLEを報告している（Yonsei Med J. 2004;45(5):923-6）。この症例は抗DNA抗体陽性だが、Discussionで文献的にSLEに合併したPLEの血清学的特徴として、抗核抗体陽性(Specled）、抗DNA抗体陰性、抗RNP抗体陽性、低補体を挙げている。
　しかし、一方でSLEに伴うPLEに対するアザチオプリンの有用性を示したMokの16症例においては、抗DNA抗体は75%で陽性であったと記載されている（Rheumatology 2006;45(4) :425-9）

・MCTDに伴うPLEとしては、Nosho Kらの報告があるA case of protein-losing gastroenteropathy in association with mixed connective tissue disease which was successfully treated with cyclophosphamide pulse therapy（4回）. Ryumachi (1998) 38 : pp 818-824.