67-第67章 多发性肌炎和皮肌炎

可乐加水

随着免疫抑制治疗的出现，特发性炎性肌病的预后不断改善。皮质激素出现前成人特发性炎性肌病的5年生存率为60%,1947一1968年为 68%，近几年为 80 。用通信方式对1986一1998年的59例特发性炎性肌病病人进行了随访，一年生存率为90%,2-5年生存率为80%。前5年共死亡12例，其中死于肺内感染5例，肺纤维化4例，肿瘤3例。发病前3年病情较重，激素用量大，激素减量或停用时病情易复发;发病5年后病情趋于稳定，有三分之一的病人脱离激素，病情仍保持稳定。我们的病人多数较重，病例也较少，因此，这些统计数字与实际情况可能会有差异，它只是在一定程度上也反映了一种趋向。除使用免疫抑制剂外，早诊断早治疗，以及有效控制并发症也有助于预后的改善。
8 _+ X1 C: J+ z9 S5 k                                                                                                                (吴东海)
      参考文献：

       马丽，王丽英，侯广庆，等 皮肌炎多发性肌炎59例随访.中华风湿病学杂志，5:59一61   

: F$ I* B3 |# D, X6 n; l7 F( j      Askanas V, Engel W N. Sporadic inclusion-body myositisand hereditary inclusion body myopathies: current concepts of di-agnosis and pathogenesis. Curr Opin Rheumatol, 1998.10:530  

$ z" d0 u% T5 c4 I      Ausem M G E M, Lochman P, van Diggelen O P, et al. Adiagnostic test for adult-onset glycogen storage disease type 11.Neurology, 1999.52:851  

      Behrens L, Bender A, Johnson M A, et al. Cytotoxic mech-anisms in inflammatory myopathied co-expression of Fas and pro-tective Bcl-2 in muscle fibers and inflammatory cells. Brain,1997. 120:929

( U1 z) p0 B- u5 T4 n- o& i8 H- j      Dalakas M C,Dambrosia J M. A controlled trial of high-doseintravenous immuneglobulin infusions as treatment for dermato-myositis. N Engl J Med, 1993. 329:1993一2000

: P3 Z4 r' }4 k, x      Fafalak R G, Peterson M G, Kagen L J. The strength inpolymyositis and dermatomyositis: best outcome in patients treat-ed early. J Rheumatol, 1994.21:643一648
% Y0 J% K& Y" Y: |6 y% G' z. H* ~( R
$ N6 q2 w8 U* {' L/ [      Fyher I-M, Moslemi A-R, Mosavia A, et al. Oligoexpres-sion in muscle infiltrating T cells in inclusion body myositis. JNeuroimmunol 1997.79:185

4 K- J) V# E6 I- M% \6 K7 F6 J      Garlepp M J, Mastaglia F L. Inclusion body myositis. JNeurol Neurosurg Psychiatry, 1996. 60:251一258
6 t8 l  X3 \' c4 j$ A0 M0 w      Garton M J, Isenberg D A. Clinical features of lupus myosi-tis versus idiopathic myositis: A review of 30 cases.  Br JRheumatol, 1997.36:1067

6 k" m: @; V5 w  R' {4 u9 m      Goebels N, Michaelis D, Engelhardt M, et al. Differentialexpression of perforin in muscle-infiltrating T cells in polymyositisand dermatomyositis. J Clin Invest, 1996.97:2905  
7 f7 a& r# X/ i$ t5 W8 m
0 j6 A' g# t3 w, i7 c      Gonzalez-Lopez L, Gamez-Nava JI, Sanchez L, et al. Car-diac manifestations in dermato-polymyositis. Clin Exp Rheumatol1996. 14:373

      Mantegazza R, Bernasconi P, Confalonieri P, et al. Inflam-matory myopathies and systemic disorders: a review of im-munopathogenetic mechanisms and clinical features. J Neurol,1997. 244:277

: c( A; I" {' U4 ?8 y% j8 e/ i: K      Marie 1, Horton P-Y, Hachulla E, et al. Pulmonary in-volvement in polymyositis and in dermatomyositis. J Rheumatol,1998. 25:1336

' n& e! A1 v' s, f      Naparstek Y, Plotz P H. The role of autoantibodies in au-toimmune disease. Ann Rev Immunol, 1993.11:79一104   

      Pascuzzi R M. Drugs and toxins associated with myopathies.Curr Opin Rheumatol, 1998.10:511   
5 a' M/ n1 p6 n% P( L2 i
* f# w( ?5 L( [9 b$ @7 g! H8 R9 o      Pickering M C, Walport M J. Eosinophilic myopathic syn-dromes. Curr Opin Rheumatol,1998. 10:504
# U( p# q* y3 c$ X
; ?. l' Y5 u1 m' P* h: U5 P3 `      Plotz P H, Rider L G，Targoff I N, et al. NIH conference.Myositis:  immunologic contributions to understanding cause,pathogenesis and therapy. Ann Intern Med, 1995-122: 715一724
; [  q- H/ _# A+ U
' y- h+ u% D2 Z; M/ o" @      Robert L. Wortmann. Inflammatory Diseases of Muscle andOther Myopathies. Kelley’s Textbook of Rheumatology, 6thEd，W. B. Saunders Company. 2001  
7 D* e! d+ B% i! P& _
& ~0 g9 g( ^5 x      Sieb J P, Ries F, Traber, et al. Recurrent focal myositis.Muscle Nerve, 1997. 20:1205
7 H8 P  l8 o; E& T+ |7 o
      Targoff I N, Miller F W, Medsger T A Jr, et al. Classifica-tion criteria for the idiopathic inflammatory myopathies. CurrOpin Rheumatol, 1997.9:527  

& l9 F6 \# p; _" h7 a      Villalba L, Hicks J E, Adams E M, et al. Treatment of re-fractory myositis. Arthritis Rheum 1998. 41:392  

      Whitemore S E, Watson R, Rosenshein N B, et al. Der-matomyositis sine myositis:  Association with malignancy.  JRheumatol, 1996.23:1010
$ \5 R# J0 v' \0 G" j" B
) {+ I! d# a3 ]/ U; \1 N      Zieglschmid M E, Pandya A G, Cohen S B, et al. Treat-ment of dermatomyositis with methotrexate. J Am Acad Derma-tol, 1995.32:754一757