メチルプレドニゾロンパルス療法が奏効した肺高血圧症合併overlap症候群の一例
症例は43歳女性. 1996年6月頃よりレイノー現象,手指の皮膚硬化を認め近医にて強皮症と診断された. 1997年4月,労作時の呼吸困難を自覚. Caチャンネルブロッカーおよびプロスタグランディン製剤の服用を開始,精査のため当院に入院した.血栓症や強度の間質性肺炎を認めなかったことから,血管障害にもとづく肺高血圧症が考えられた.さらに全身性エリテマトーデスの診断基準も満たしoverlap症候群と診断された.メチルプレドニゾロンのパルス療法にて自,他覚症状が改善した. Overlap症候群に合併した肺高血圧症の報告はきわめて稀である.膠原病に合併した肺高血圧症は予後が著しく悪い.本症例がステロイ...
Saved in:
| Published in | 日本臨床免疫学会会誌 Vol. 26; no. 1; pp. 28 - 33 |
|---|---|
| Main Authors | , , , |
| Format | Journal Article |
| Language | English |
| Published |
日本臨床免疫学会
2003
|
| Online Access | Get full text |
| ISSN | 0911-4300 1349-7413 |
| DOI | 10.2177/jsci.26.28 |
Cover
| Abstract | 症例は43歳女性. 1996年6月頃よりレイノー現象,手指の皮膚硬化を認め近医にて強皮症と診断された. 1997年4月,労作時の呼吸困難を自覚. Caチャンネルブロッカーおよびプロスタグランディン製剤の服用を開始,精査のため当院に入院した.血栓症や強度の間質性肺炎を認めなかったことから,血管障害にもとづく肺高血圧症が考えられた.さらに全身性エリテマトーデスの診断基準も満たしoverlap症候群と診断された.メチルプレドニゾロンのパルス療法にて自,他覚症状が改善した. Overlap症候群に合併した肺高血圧症の報告はきわめて稀である.膠原病に合併した肺高血圧症は予後が著しく悪い.本症例がステロイドパルス療法に反応したことから,ステロイドパルス療法は膠原病合併肺高血圧症に試みるべき一治療法と考えられ報告する. |
|---|---|
| AbstractList | 症例は43歳女性. 1996年6月頃よりレイノー現象,手指の皮膚硬化を認め近医にて強皮症と診断された. 1997年4月,労作時の呼吸困難を自覚. Caチャンネルブロッカーおよびプロスタグランディン製剤の服用を開始,精査のため当院に入院した.血栓症や強度の間質性肺炎を認めなかったことから,血管障害にもとづく肺高血圧症が考えられた.さらに全身性エリテマトーデスの診断基準も満たしoverlap症候群と診断された.メチルプレドニゾロンのパルス療法にて自,他覚症状が改善した. Overlap症候群に合併した肺高血圧症の報告はきわめて稀である.膠原病に合併した肺高血圧症は予後が著しく悪い.本症例がステロイドパルス療法に反応したことから,ステロイドパルス療法は膠原病合併肺高血圧症に試みるべき一治療法と考えられ報告する. |
| Author | 鎌谷, 直之 南家, 由紀 小竹, 茂 原, まさ子 |
| Author_xml | – sequence: 1 fullname: 南家, 由紀 organization: 東京女子医大膠原病リウマチ痛風センター – sequence: 1 fullname: 小竹, 茂 organization: 東京女子医大膠原病リウマチ痛風センター – sequence: 1 fullname: 原, まさ子 organization: 東京女子医大膠原病リウマチ痛風センター – sequence: 1 fullname: 鎌谷, 直之 organization: 東京女子医大膠原病リウマチ痛風センター |
| BookMark | eNo9kE1LAkEAhocwSK1L_2NtZmd2ZvYY0hcIXbwv4-5suZjKrgTd3J1DHwbVIUwIQig6WF0kyCD_zLhp_yIk6fI-vDzwHt4cyNQbdQnAOoIFEzG2EURutWDSgsmXQBZhYhuMIJwBWWgjZBAM4QrIRVEAIbYwI1kQaNXXKtZqoFVXqxetzrXq6GSs1atWQ61u5ioZTXvJ9_BWx5fp01V6MdJxV8cPs-TzZ3A367fT--dp9zS9Ppt8vTeOZVgTzXlv96bjRx2_TT7ak3FnFSz7ohbJtQXzoLy9VS7uGqX9nb3iZskITJNBgyJGPQIriNsWr2AuoeVLQiD2sA8R9CWreERKl3JqU-gywYkkNuTCZB51Ec6D4t9sELXEgXSaYfVIhCeOCFtVtyad-UXIZtwxqYMWwf-teyhCJxD4FxN7hwg |
| ContentType | Journal Article |
| Copyright | 日本臨床免疫学会 |
| Copyright_xml | – notice: 日本臨床免疫学会 |
| DOI | 10.2177/jsci.26.28 |
| DatabaseTitleList | |
| DeliveryMethod | fulltext_linktorsrc |
| Discipline | Biology |
| EISSN | 1349-7413 |
| EndPage | 33 |
| ExternalDocumentID | article_jsci1978_26_1_26_1_28_article_char_ja |
| GroupedDBID | ALMA_UNASSIGNED_HOLDINGS CS3 EBS EJD F5P JSF KQ8 MOJWN OK1 RJT |
| ID | FETCH-LOGICAL-j2270-6176d40b18958b38e05fe4403d3f010fe7bd4eec686960c7a84e4908a27d6c13 |
| ISSN | 0911-4300 |
| IngestDate | Wed Sep 03 06:29:09 EDT 2025 |
| IsDoiOpenAccess | true |
| IsOpenAccess | true |
| IsPeerReviewed | false |
| IsScholarly | false |
| Issue | 1 |
| Language | English |
| LinkModel | OpenURL |
| MergedId | FETCHMERGED-LOGICAL-j2270-6176d40b18958b38e05fe4403d3f010fe7bd4eec686960c7a84e4908a27d6c13 |
| OpenAccessLink | https://www.jstage.jst.go.jp/article/jsci1978/26/1/26_1_28/_article/-char/ja |
| PageCount | 6 |
| ParticipantIDs | jstage_primary_article_jsci1978_26_1_26_1_28_article_char_ja |
| PublicationCentury | 2000 |
| PublicationDate | 20030000 |
| PublicationDateYYYYMMDD | 2003-01-01 |
| PublicationDate_xml | – year: 2003 text: 20030000 |
| PublicationDecade | 2000 |
| PublicationTitle | 日本臨床免疫学会会誌 |
| PublicationTitleAlternate | 日臨免誌 |
| PublicationYear | 2003 |
| Publisher | 日本臨床免疫学会 |
| Publisher_xml | – name: 日本臨床免疫学会 |
| References | 1) Klings, E. S., et al.: Systemic sclerosis associated pulmonary hypertension: short-and long-term effects of epoprostenol (prostacyclin). Arthritis Rheum. 42: 2638-2645, 1999. 7) Rich, S., et al.: The effect of high doses of calcium channel blockers on survival in primary pulmonary hypertension. N. Engl. J. Med. 377: 76-81, 1992. 11) Badesch, D. B., et al.: Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann. Intern. Med. 132: 425-434, 2000. 21) Morelli, S., et al.: Plasma endothelin-1 levels, pulmonary hypertension, and lung fibrosis in patients with systemic sclerosis. Am. J. Med. 99: 255-260, 1995. 2) Morelli, S., et al.: Relationship between cutaneous and plumonary involvement in systemic sclerosis. J. Rheumatol. 24: 81-85, 1997. 14) Mata, J., et al.: Long-term iloprost infusion therapy for severe pulmonary hypertension in patients with connective tissue diseases. Arthritis Rheum. 10: 1528-1533, 1994. 18) Dahl, M., et al.: Ten year survival of a patient with advanced pulmonary hypertension and mixed connective tissue disease treated with immunosuppressive therapy. J. Rheumatol. 19: 1807-1809, 1992. 8) Seibold, J. R., et al.: Acute hemodynamic effects of ketanserin in pulmonary hypertension secondary to systemic sclerosis. J. Rheumatol. 14: 519-524, 1987. 13) Williamson, D. J., et al.: Acute hemodynamic responsed to inhaled nitric oxide in patients with limmited scleroderma and isoloted pulmonary hypertension. Circulation 3: 477-482, 1996. 10) Menon, N., et al.: The acute effects of prostacyclin on plumonary hemodynamics in patients with pulmonary hypertension secondary to systemic sclerosis. Arthritis Rheum. 41: 466-469, 1998. 16) Asherson, R. A., et al.: Pulmonary hypertension in a lupus clinic: experience with twenty-four patinets. J. Rheumatol. 17: 1292-1298, 1990. 19) Ihn, H.: Measurement of anticardiolipin antibody by ELISA using beta-2 GPI in systemic sclerosis. Clinical and Experimental Immunology 105: 475-479, 1996. 15) Anderson, G. N., Vasko, J.: Scleroderma renal crisis and concurrent isolated pulmonary hypertension in mixed coneective tissue disease and overlap syndrome: report of two cases. Clin. Rheumatol. 21: 164-169, 2002. 4) Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Arthritis Rheum. 23: 581-590, 1989. 17) Goupill, P., et al.: Precapillary pulmonary hypertension dramatically improved with high doses of corticosteroids during systemic lupus erthematosus. J. Rheumatol. (letter). 1976-1977, 1994. 3) Stupi, A. M., et al.: Pulmonary hypertension in the cap syndrome variant of systemic sclerosis. Arthritis Rheum. 29: 515-524, 1986. 20) Stewart, D. J., et al.: Increased plasma endothelin-1 in pulomonary hypertension: Marder or mediator of disease? Ann Intern Med. 114: 464-469, 1991. 12) Humbert, M., et al.: Short-term and long-term epoprostenol (prostacyclin) therapy in pulmonary hypertension secondary to connective tissue diseases: results of a pilot study. Eur. Resir. J. 13: 1351-1356, 1999. 9) Ohar, J., et al.: The role of vasodilators in patients with progressive systemic sclerosis. Chest. 88: 263-265, 1985. 5) Nanke, Y., et al.: Raised plasma adrenomedullin in patients with systemic sclerosis complicated by pulmonary hypertension. Ann Rheum. Dis. 59: 493-494, 2000. 6) Tan, E. M., et al.: The 1982 revised criteria for the classification of systemic lupus erytematosus. Arthritis Rheum. 25: 1271-1277. 1982. |
| References_xml | – reference: 6) Tan, E. M., et al.: The 1982 revised criteria for the classification of systemic lupus erytematosus. Arthritis Rheum. 25: 1271-1277. 1982. – reference: 9) Ohar, J., et al.: The role of vasodilators in patients with progressive systemic sclerosis. Chest. 88: 263-265, 1985. – reference: 10) Menon, N., et al.: The acute effects of prostacyclin on plumonary hemodynamics in patients with pulmonary hypertension secondary to systemic sclerosis. Arthritis Rheum. 41: 466-469, 1998. – reference: 1) Klings, E. S., et al.: Systemic sclerosis associated pulmonary hypertension: short-and long-term effects of epoprostenol (prostacyclin). Arthritis Rheum. 42: 2638-2645, 1999. – reference: 11) Badesch, D. B., et al.: Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann. Intern. Med. 132: 425-434, 2000. – reference: 15) Anderson, G. N., Vasko, J.: Scleroderma renal crisis and concurrent isolated pulmonary hypertension in mixed coneective tissue disease and overlap syndrome: report of two cases. Clin. Rheumatol. 21: 164-169, 2002. – reference: 13) Williamson, D. J., et al.: Acute hemodynamic responsed to inhaled nitric oxide in patients with limmited scleroderma and isoloted pulmonary hypertension. Circulation 3: 477-482, 1996. – reference: 14) Mata, J., et al.: Long-term iloprost infusion therapy for severe pulmonary hypertension in patients with connective tissue diseases. Arthritis Rheum. 10: 1528-1533, 1994. – reference: 3) Stupi, A. M., et al.: Pulmonary hypertension in the cap syndrome variant of systemic sclerosis. Arthritis Rheum. 29: 515-524, 1986. – reference: 5) Nanke, Y., et al.: Raised plasma adrenomedullin in patients with systemic sclerosis complicated by pulmonary hypertension. Ann Rheum. Dis. 59: 493-494, 2000. – reference: 4) Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Arthritis Rheum. 23: 581-590, 1989. – reference: 18) Dahl, M., et al.: Ten year survival of a patient with advanced pulmonary hypertension and mixed connective tissue disease treated with immunosuppressive therapy. J. Rheumatol. 19: 1807-1809, 1992. – reference: 16) Asherson, R. A., et al.: Pulmonary hypertension in a lupus clinic: experience with twenty-four patinets. J. Rheumatol. 17: 1292-1298, 1990. – reference: 2) Morelli, S., et al.: Relationship between cutaneous and plumonary involvement in systemic sclerosis. J. Rheumatol. 24: 81-85, 1997. – reference: 19) Ihn, H.: Measurement of anticardiolipin antibody by ELISA using beta-2 GPI in systemic sclerosis. Clinical and Experimental Immunology 105: 475-479, 1996. – reference: 7) Rich, S., et al.: The effect of high doses of calcium channel blockers on survival in primary pulmonary hypertension. N. Engl. J. Med. 377: 76-81, 1992. – reference: 20) Stewart, D. J., et al.: Increased plasma endothelin-1 in pulomonary hypertension: Marder or mediator of disease? Ann Intern Med. 114: 464-469, 1991. – reference: 21) Morelli, S., et al.: Plasma endothelin-1 levels, pulmonary hypertension, and lung fibrosis in patients with systemic sclerosis. Am. J. Med. 99: 255-260, 1995. – reference: 8) Seibold, J. R., et al.: Acute hemodynamic effects of ketanserin in pulmonary hypertension secondary to systemic sclerosis. J. Rheumatol. 14: 519-524, 1987. – reference: 12) Humbert, M., et al.: Short-term and long-term epoprostenol (prostacyclin) therapy in pulmonary hypertension secondary to connective tissue diseases: results of a pilot study. Eur. Resir. J. 13: 1351-1356, 1999. – reference: 17) Goupill, P., et al.: Precapillary pulmonary hypertension dramatically improved with high doses of corticosteroids during systemic lupus erthematosus. J. Rheumatol. (letter). 1976-1977, 1994. |
| SSID | ssj0035374 |
| Score | 1.3969939 |
| Snippet | 症例は43歳女性. 1996年6月頃よりレイノー現象,手指の皮膚硬化を認め近医にて強皮症と診断された. 1997年4月,労作時の呼吸困難を自覚.... |
| SourceID | jstage |
| SourceType | Publisher |
| StartPage | 28 |
| Title | メチルプレドニゾロンパルス療法が奏効した肺高血圧症合併overlap症候群の一例 |
| URI | https://www.jstage.jst.go.jp/article/jsci1978/26/1/26_1_28/_article/-char/ja |
| Volume | 26 |
| hasFullText | 1 |
| inHoldings | 1 |
| isFullTextHit | |
| isPrint | |
| ispartofPNX | 日本臨床免疫学会会誌, 2003/02/28, Vol.26(1), pp.28-33 |
| journalDatabaseRights | – providerCode: PRVAFT databaseName: Open Access Digital Library customDbUrl: eissn: 1349-7413 dateEnd: 99991231 omitProxy: true ssIdentifier: ssj0035374 issn: 0911-4300 databaseCode: KQ8 dateStart: 19780101 isFulltext: true titleUrlDefault: http://grweb.coalliance.org/oadl/oadl.html providerName: Colorado Alliance of Research Libraries |
| link | http://utb.summon.serialssolutions.com/2.0.0/link/0/eLvHCXMwrR1Na9RANJSK4EX8xG96cI6pSSbJzKCXZHdLERSECr2FTTY5LFKLbA966m4OflRQD1ILghQUD1UvRbCC_TPTtfVf-N5LsmaLB6vCEt7OvO-32XkzeTMxjMt2nCjXi1PTb2eWiQ8MzbZlp6aMkyyBFDpzaFfajZv-7G33-rw3PzF5tVa1tNSLp5MHv91X8jdRhTaIK-6SPUBkR0yhAWCIL1whwnD9oxizFmeSs8AuAVkBQVgCSlQtjQpHVUCB47CwVeE0SyDkFfk-hoAM5IIphXDLR0zlUZfNJIjwWOAxOYOADAiZuko1AIAuSXwC1lLIVklsAROkhVSqwQJBIgSTglosJgHHZWGThR5WnN5pL45hYLEGqQWWBG4pKWgRjSS-LnbJsJ6Jo_bAAdRFoEEOksgwkMgT9AMDkDl8miTOJy946KbAJ54NpoIaAGYA1WjZmaibZLuH6oQ-_p6JFZDYpDBk8lYdP7TIewJFgfcIX6JnpTPGtoWepF4ytgyhTcEgDVWNrUJ8SfYB_1CM1AhRAdRfVc6p1n_46F79X36qrwnbtulyq9AwLUZE7ioT0k5eHzKLQw7G_hrK8U_WMqnihJP9Y7RTVAl0IcObdvzpimLszPPyjooQyVZCRo4f2eVFRlUv7kuMujA5OuTgYhxWZNwaPULkHi-OYK9sKs4mRulXfsmGvLMLs7CqgpOSyrljxtFyNjgVFKKOGxPpwgnjcPF-2Psnja7O13Xe1_mGzld1_kHnj3W-ogfbOv-o802dv8Cuwdbu2uD75kvdfzp892z4ZEv3V3X_zd7g64-NV3vry8PX73dXHw6fP9r59rm8ffD78tru9lvd_7TzZXlne-WUMTfTmmvMmuXLUcyu4whc8hF-x7ViWypPxlymlpelrmvxDs8s28pSEXfcNE186SvfSkRbuik-5G87ouMnNj9tTC7cXUjPGFNxR2a2k8R4cqgrHB4n0hOOlXkKOajsrHGt8FG0WByAEx0oPOf-jfy8cYTqZ2nV84Ix2bu3lF6EeUAvvkTx_gm_o92D |
| linkProvider | Colorado Alliance of Research Libraries |
| openUrl | ctx_ver=Z39.88-2004&ctx_enc=info%3Aofi%2Fenc%3AUTF-8&rfr_id=info%3Asid%2Fsummon.serialssolutions.com&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.atitle=%E3%83%A1%E3%83%81%E3%83%AB%E3%83%97%E3%83%AC%E3%83%89%E3%83%8B%E3%82%BE%E3%83%AD%E3%83%B3%E3%83%91%E3%83%AB%E3%82%B9%E7%99%82%E6%B3%95%E3%81%8C%E5%A5%8F%E5%8A%B9%E3%81%97%E3%81%9F%E8%82%BA%E9%AB%98%E8%A1%80%E5%9C%A7%E7%97%87%E5%90%88%E4%BD%B5overlap%E7%97%87%E5%80%99%E7%BE%A4%E3%81%AE%E4%B8%80%E4%BE%8B&rft.jtitle=%E6%97%A5%E6%9C%AC%E8%87%A8%E5%BA%8A%E5%85%8D%E7%96%AB%E5%AD%A6%E4%BC%9A%E4%BC%9A%E8%AA%8C&rft.au=%E5%8D%97%E5%AE%B6%2C+%E7%94%B1%E7%B4%80&rft.au=%E5%B0%8F%E7%AB%B9%2C+%E8%8C%82&rft.au=%E5%8E%9F%2C+%E3%81%BE%E3%81%95%E5%AD%90&rft.au=%E9%8E%8C%E8%B0%B7%2C+%E7%9B%B4%E4%B9%8B&rft.date=2003&rft.pub=%E6%97%A5%E6%9C%AC%E8%87%A8%E5%BA%8A%E5%85%8D%E7%96%AB%E5%AD%A6%E4%BC%9A&rft.issn=0911-4300&rft.eissn=1349-7413&rft.volume=26&rft.issue=1&rft.spage=28&rft.epage=33&rft_id=info:doi/10.2177%2Fjsci.26.28&rft.externalDocID=article_jsci1978_26_1_26_1_28_article_char_ja |
| thumbnail_l | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/lc.gif&issn=0911-4300&client=summon |
| thumbnail_m | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/mc.gif&issn=0911-4300&client=summon |
| thumbnail_s | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/sc.gif&issn=0911-4300&client=summon |