鈥濷rphan diseases鈥?in der Rheumatologie

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• 作者：Prof. Dr. A. Rubbert-Roth (1)
  
• 关键词：Orphan disease ; Panarteriitis nodosa ; Diagnosekriterien ; Therapie ; Hepatitis B ; Orphan disease ; Panarteritis nodosa ; Diagnostic criteria ; Treatment ; Hepatitis B
• 刊名：Zeitschrift f篓鹿r Rheumatologie
• 出版年：2012
• 出版时间：February 2012
• 年：2012
• 卷：71
• 期：2
• 页码：119-121
• 全文大小：463KB
• 参考文献：1. Kussmaul A, Maier R (1866) Ueber eine bisher nicht beshriebene eigen-thumliche Arterienerkrankung (Periarteritis nodosa), die mit Morbus Brightii und rapid fortschreibtender allgemeiner Muskellahmung einhergeht. Dtsch Arch Klin Med 1:484鈥?17
  2. Valente RM, Conn DL (o J) Polyarteritis-Polyarteritis nodosa and microscopic angiitis. In: Klippel JH, Dieppe PA (Hrsg) Rheumatology, 2. Aufl. Mosby, St. Louis London, S聽20.1鈥?0.10
  3. Scott DGI, Watts RA (1994) Classification and epidemiology of systemic vasculitis. Br J Rheumatol 33:897鈥?00 CrossRef
  4. Ozen S, Ruperto N, Dillon MJ et al (2006) EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis 65:936鈥?41 CrossRef
  5. Lightfoot RW, Michel BA, Bloch DA et al (1990) The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum 33:1088鈥?093 CrossRef
  6. De Kort SW, Rossum MA van, ten Cate R (2006) Infliximab in a child with therapy-resistant systemic vasculitis. Clin Rheumatol 25:769鈥?71 CrossRef
  7. Kroiss MM, Hohenleutner U, Gruss C et al (2001) Transient and partial effect of high-dose intravenous immunoglobulin in polyarteritis nodosa. Dermatology 203:188鈥?89 CrossRef
  8. Guillevin L, Cohen P, Mahr A et al (2003) Treatment of polyarteritis nodosa and microscopic polyangiitis with poor prognosis factors; a prospective trial comparing glucocorticoids and six or twelve cyclophosphamide pulses in sixty five patients. Arthritis Rheum 15:93鈥?00 CrossRef
  9. Guillevin L, Mahr A, Callard P et al (2005) French Vasculitis Study Group. Hepatitis B virus-associated polyarteritis nodosa: clinical charac- teristics, outcome and impact of treatment in 115 patients. Medicine (Baltimore) 84:313鈥?22
  
• 作者单位：Prof. Dr. A. Rubbert-Roth (1)
  
  1. Klinik I f眉r Innere Medizin, Uniklinik K枚ln, Kerpener Str. 62, 50937, K枚ln, Deutschland
  

文摘

The term orphan disease is used for those diseases which are diagnosed in less than 5 out of 10,000 persons. An example of an orphan disease in rheumatology is panarteritis nodosa (PAN), the clinical manifestation of which ranges from mild forms to life-threatening situations. The scientific publications on the diagnosis and therapy of PAN are usually limited to casuistics and small case studies. Treatment of this disease is usually carried out analogue to other forms of vasculitis but the therapeutics used are not formally approved for PAN. With this in mind it is much more realistic to treat such patients in specialized centers or at least to allow an evidence-based medical treatment in, for example network associations, using the targeted documentation of patients.