• 四川大學(xué)華西醫(yī)院風(fēng)濕免疫科（成都，610041）;

【摘要】　目的　 探討新診斷的多發(fā)性肌炎（PM）、皮肌炎（DM）和無肌病性皮肌炎（ADM）肺部病變發(fā)生率、臨床特點(diǎn)及相關(guān)因素。　方法　 回顧性分析2008年1月—2010年7月新確診的206例PM、DM、ADM臨床表現(xiàn)、肺部影像學(xué)、肺功能、超聲心動圖和實(shí)驗(yàn)室指標(biāo)。　結(jié)果　 206例患者中合并肺部病變156例，以肺間質(zhì)病變（ILD）最多見，占患者總數(shù)的51.46%。在性別、病程、是否吸煙方面合并ILD與無ILD患者相比差異無統(tǒng)計(jì)學(xué)意義，合并ILD患者年齡大于無ILD組。合并ILD患者呼吸困難、發(fā)熱、雷諾現(xiàn)象、關(guān)節(jié)炎或關(guān)節(jié)痛概率增加。合并ILD組白蛋白低于無ILD組，血沉和免疫球蛋白IgM高于無ILD組。急性ILD組中女性患者及出現(xiàn)雷諾現(xiàn)象的概率高于慢性組。206例患者中死亡13例，其中周圍型肺癌1例，特發(fā)性血小板減少并顱內(nèi)出血1例，嚴(yán)重肺部感染11例；死亡患者中10例伴肺間質(zhì)纖維化。合并急性ILD患者死亡率較慢性組高2倍。　結(jié)論　 PM、DM、ADM患者肺部病變發(fā)生率高，以ILD多見，發(fā)熱、年齡大、白蛋白降低、血沉升高、雷諾現(xiàn)象及關(guān)節(jié)炎或關(guān)節(jié)痛都是合并ILD的相關(guān)因素。合并急性ILD患者預(yù)后差，死亡者常合并肺部感染。
【Abstract】　Objective　 To explore the prevalence, clinical features, and predictive factors of pulmonary involvement in newly diagnosed polymyositis (PM), dermatomyositis (DM) and amyopathic dermatomyositis (ADM), in order to carry out early diagnosis and treatment, and improve the prognosis. 　Methods　 The clinical manifestations, chest imaging, pulmonary function test, ultrasonic cardiography and laboratory results of 206 inpatients with PM, DM and ADM in West China Hospital of Sichuan University from January 2008 to July 2010 were reviewed retrospectively. 　Results　 One hundred and fifty-six out of 206 patients developed PM/DM/ADM associated lung diseases, including 106 cases (51.46%) of interstitial lung disease (ILD). There was no significant difference in gender, disease duration, and smoking or not between the ILD and non-ILD group, but patients in the ILD group were significantly older than non-ILD group. The results also showed that patients with ILD were much more likely to have symptoms of breathing difficulties, fever, Raynaud phenomenon and arthritis/arthralgia. The patients with ILD had lower level of albumin but higher levels of ESR and IgM; In the group of acute ILD, female patients and the ratio of Raynaud phenomenon were higher than those in the chronicity group. Of the 206 patients, 13 patients died, including 1 death of peripheral lung cancer, 1 of essential thrombocytopenia and intracranial hemorrhages, and 11 of severe lung infection, and 10 in these patients developed ILD. Mortality in patients with acute ILD was 2 times higher than the chronicity group. 　Conclusion　 The prevalence of lung diseases is high in patients with PM, DM and ADM. ILD is the main pulmonary involvement, and fever, older age at onset, hypoalbuminemia, higher values of blood sedimentation, Raynaud phenomenon and arthritis or arthralgia were the predictive factors for developing ILD. Patients with acute ILD have poor prognosis. Death cases often have pulmonary infections.

引用本文： 李莉,謝其冰,劉怡欣,李昕怡,劉毅,劉鋼. 炎性肌病患者肺部病變特點(diǎn)分析. 華西醫(yī)學(xué), 2011, 26(5): 646-650. doi: 復(fù)制

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1. 　Marie I, Hatron PY, Hachulla E, et al. Pulmonary involvement in polymyositis and in dermatomyositis[J]. J Rheumatol, 1998, 25(7): 1336-1343.
2. 　Fathi M, Dastmalchi M, Rasmussen E, et al. Interstitial lung disease, a common manifestation of newly diagnosed polymyositis and dermatomyositis[J]. Ann Rheum Dis, 2004, 63(3): 297-301.
3. 　Marie I, Hachulla E, Chérin P, et al. Interstitial lung disease in polymyositis and dermatomyositis[J]. Arthritis Rheum, 2002, 47(6): 614-622.
4. 　Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts)[J]. N Engl J Med, 1975, 292(7): 344-347.
5. 　Bohan A, Peter JB. Polymyositis and dermatomyositis (second of two parts)[J]. N Engl J Med, 1975, 292(8): 403-407.
6. 　Euwer RL, Sontheimer RD. Amyopathic dermatomyositis: a review[J]. J Invest Dermatol, 1993, 100(1): 124S-127S.
7. 　Won Huh J, Soon Kim D, Keun Lee C, et al. Two distinct clinical types of interstitial lung disease associated with polymyositis-dermatomyositis[J]. Respir Med, 2007, 101(8): 1761-1769.
8. 　Schwarz MI. The lung in polymyositis[J]. Clin Chest Med, 1998, 19(4): 701-712.
9. 　Callen JP. Dermatomyositis[J]. Lancet, 2000, 355(9197): 53-57.
10. 　Marie I, Hachulla E, Hatron PY, et al. Polymyositis and dermatomyositis: short term and longterm outcome, and predictive factors of prognosis[J]. J Rheumatol, 2001, 28(10): 2230-2237.
11. 　Marie I, Hatron PY, Levesque H, et al. Influence of age on characteristics of polymyositis and dermatomyositis in adults[J]. Medicine (Baltimore), 1999, 78(3): 139-147.
12. 　Kang EH, Lee EB, Shin KC, et al. Interstitial lung disease in patients with polymyositis, dermatomyositis and amyopathic dermatomyositis[J]. Rheumatology (Oxford), 2005, 44(10): 1282-1286.
13. 　Mukae H, Ishimoto H, Sakamoto N, et al. Clinical differences between interstitial lung disease associated with clinically amyopathic dermatomyositis and classic dermatomyositis[J]. Chest, 2009, 136(5): 1341-1347.
14. 　Sontheimer RD, Miyagawa S. Potentially fatal interstitial lung disease can occur in clinically amyopathic dermatomyositis[J]. J Am Acad Dermatol, 2003, 48(5): 797-798.
15. 　Lee CS, Chen TL, Tzen CY, et al. Idiopathic inflammatory myopathy with diffuse alveolar damage[J]. Clin Rheumatol, 2002, 21(5): 391-396.
16. 　Ye S, Chen XX, Lu XY, et al. Adult clinically amyopathic dermatomyositis with rapid progressive interstitial lung disease: a retrospective cohort study[J]. Clin Rheumatol, 2007, 26(10): 1647-1654.
17. 　Ji SY, Zeng FQ, Guo Q, et al. Predictive factors and unfavourable prognostic factors of interstitial lung disease in patients with polymyositis or dermatomyositis: a retrospective study[J]. Chin Med J (Engl), 2010, 123(5): 517-522.
18. 　Hengstman GJ, van Engelen BG, van Venrooij WJ. Myositis specific autoantibodies: changing insights in pathophysiology and clinical associations[J]. Curr Opin Rheumatol, 2004, 16(6): 692-699.
19. 　Mimori T, Imura Y, Nakashima R, et al. Autoantibodies in idiopathic inflammatory myopathy: an update on clinical and pathophysiological significance[J]. Curr Opin Rheumatol, 2007, 19(6): 523-529.
20. 　Taggart AJ, Finch MB, Courtney PA, et al. Anti Jo-1 myositis. ’Mechanic’s hands’ and interstitial lung disease[J]. Ulster Med J, 2002, 71(1): 68-71.
21. 　Richards TJ, Eggebeen A, Gibson K, et al. Characterization and peripheral blood biomarker assessment of anti-Jo-1 antibody-positive interstitial lung disease[J]. Arthritis Rheum, 2009, 60(7): 2183-2192.
22. 　Schnabel A, Reuter M, Biederer J, et al. Interstitial lung disease in polymyositis and dermatomyositis: clinical course and response to treatment[J]. Semin Arthritis Rheum, 2003, 32(5): 273-284.
23. 　Chen IJ, Jan Wu YJ, Lin CW, et al. Interstitial lung disease in polymyositis and dermatomyositis[J]. Clin Rheumatol, 2009, 28(6): 639-646.
24. 　Dankó K, Ponyi A, Constantin T, et al. Long-term survival of patients with idiopathic inflammatory myopathies according to clinical features: a longitudinal study of 162 cases[J]. Medicine (Baltimore), 2004, 83(1): 35-42.
  1. 1. 　Marie I, Hatron PY, Hachulla E, et al. Pulmonary involvement in polymyositis and in dermatomyositis[J]. J Rheumatol, 1998, 25(7): 1336-1343.
  2. 2. 　Fathi M, Dastmalchi M, Rasmussen E, et al. Interstitial lung disease, a common manifestation of newly diagnosed polymyositis and dermatomyositis[J]. Ann Rheum Dis, 2004, 63(3): 297-301.
  3. 3. 　Marie I, Hachulla E, Chérin P, et al. Interstitial lung disease in polymyositis and dermatomyositis[J]. Arthritis Rheum, 2002, 47(6): 614-622.
  4. 4. 　Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts)[J]. N Engl J Med, 1975, 292(7): 344-347.
  5. 5. 　Bohan A, Peter JB. Polymyositis and dermatomyositis (second of two parts)[J]. N Engl J Med, 1975, 292(8): 403-407.
  6. 6. 　Euwer RL, Sontheimer RD. Amyopathic dermatomyositis: a review[J]. J Invest Dermatol, 1993, 100(1): 124S-127S.
  7. 7. 　Won Huh J, Soon Kim D, Keun Lee C, et al. Two distinct clinical types of interstitial lung disease associated with polymyositis-dermatomyositis[J]. Respir Med, 2007, 101(8): 1761-1769.
  8. 8. 　Schwarz MI. The lung in polymyositis[J]. Clin Chest Med, 1998, 19(4): 701-712.
  9. 9. 　Callen JP. Dermatomyositis[J]. Lancet, 2000, 355(9197): 53-57.
  10. 10. 　Marie I, Hachulla E, Hatron PY, et al. Polymyositis and dermatomyositis: short term and longterm outcome, and predictive factors of prognosis[J]. J Rheumatol, 2001, 28(10): 2230-2237.
  11. 11. 　Marie I, Hatron PY, Levesque H, et al. Influence of age on characteristics of polymyositis and dermatomyositis in adults[J]. Medicine (Baltimore), 1999, 78(3): 139-147.
  12. 12. 　Kang EH, Lee EB, Shin KC, et al. Interstitial lung disease in patients with polymyositis, dermatomyositis and amyopathic dermatomyositis[J]. Rheumatology (Oxford), 2005, 44(10): 1282-1286.
  13. 13. 　Mukae H, Ishimoto H, Sakamoto N, et al. Clinical differences between interstitial lung disease associated with clinically amyopathic dermatomyositis and classic dermatomyositis[J]. Chest, 2009, 136(5): 1341-1347.
  14. 14. 　Sontheimer RD, Miyagawa S. Potentially fatal interstitial lung disease can occur in clinically amyopathic dermatomyositis[J]. J Am Acad Dermatol, 2003, 48(5): 797-798.
  15. 15. 　Lee CS, Chen TL, Tzen CY, et al. Idiopathic inflammatory myopathy with diffuse alveolar damage[J]. Clin Rheumatol, 2002, 21(5): 391-396.
  16. 16. 　Ye S, Chen XX, Lu XY, et al. Adult clinically amyopathic dermatomyositis with rapid progressive interstitial lung disease: a retrospective cohort study[J]. Clin Rheumatol, 2007, 26(10): 1647-1654.
  17. 17. 　Ji SY, Zeng FQ, Guo Q, et al. Predictive factors and unfavourable prognostic factors of interstitial lung disease in patients with polymyositis or dermatomyositis: a retrospective study[J]. Chin Med J (Engl), 2010, 123(5): 517-522.
  18. 18. 　Hengstman GJ, van Engelen BG, van Venrooij WJ. Myositis specific autoantibodies: changing insights in pathophysiology and clinical associations[J]. Curr Opin Rheumatol, 2004, 16(6): 692-699.
  19. 19. 　Mimori T, Imura Y, Nakashima R, et al. Autoantibodies in idiopathic inflammatory myopathy: an update on clinical and pathophysiological significance[J]. Curr Opin Rheumatol, 2007, 19(6): 523-529.
  20. 20. 　Taggart AJ, Finch MB, Courtney PA, et al. Anti Jo-1 myositis. ’Mechanic’s hands’ and interstitial lung disease[J]. Ulster Med J, 2002, 71(1): 68-71.
  21. 21. 　Richards TJ, Eggebeen A, Gibson K, et al. Characterization and peripheral blood biomarker assessment of anti-Jo-1 antibody-positive interstitial lung disease[J]. Arthritis Rheum, 2009, 60(7): 2183-2192.
  22. 22. 　Schnabel A, Reuter M, Biederer J, et al. Interstitial lung disease in polymyositis and dermatomyositis: clinical course and response to treatment[J]. Semin Arthritis Rheum, 2003, 32(5): 273-284.
  23. 23. 　Chen IJ, Jan Wu YJ, Lin CW, et al. Interstitial lung disease in polymyositis and dermatomyositis[J]. Clin Rheumatol, 2009, 28(6): 639-646.
  24. 24. 　Dankó K, Ponyi A, Constantin T, et al. Long-term survival of patients with idiopathic inflammatory myopathies according to clinical features: a longitudinal study of 162 cases[J]. Medicine (Baltimore), 2004, 83(1): 35-42.