Bağ dokusu hastalıklarına bağlı gelişen interstisyel akciğer hastalığı: Tanı ve tedavi yaklaşımları
Bağ dokusu hastalıkları sıklıkla torasik değişikliklere yol açan immünolojik aracılı bir grup sistemik hastalıktır. Bağ dokusu hastalıklarında solunum sistemi tutulumu sıklığı ile ilgili yapılan epidemiyolojik çalışmalar arasında tespit ve seçilme yöntemlerine bağlı büyük farklılıklar vardır. Ancak bağ dokusu hastalıklarında pulmoner tutulum geliştiğinde mortalite ve morbidite artmaktadır. İnterstisyel akciğer hastalığı, bu anlamda önemli bir grubu oluşturmakta ve genel insidansın %15 olduğu tahmin edilmektedir. Günümüzde halen bağ dokusu hastalıklarına bağlı interstisyel akciğer hastalığının tanı, tedavi, takip ve prognozu ile ilgili klinik sorunlar yaşanmaktadır. Hastalığın tanısal yöntemleri klinik yaklaşım, solunum fonksiyon testleri, yüksek çözünürlüklü bilgisayarlı tomografi, bronkoalveoler lavaj ve cerrahi akciğer biyopsisini içermektedir. Tedavi ve ilaç seçiminde tartışmalar devam etmektedir. Bu derlemede her bir bağ dokusu hastalığı için ayrı ayrı tanı, solunum fonksiyon testi ve radyolojik özellikler, prognoz ve tedavi yaklaşımlarının değerlendirilmesi amaçlanmıştır.
Interstitial lung disease associated with connective tissue diseases: diagnosis and treatment approaches
Connective tissue diseases are a diverse group of immunologically mediated systemic disorders that often lead to thoracic changes. There are huge differences between epidemiological studies depending upon method of ascertainment and selec tion criteria regarding the incidence of respiratory involvement due to connective tissue diseases. However, increased mortality and morbidity develops pulmonary involvement due to connective tissue diseases. Interstitial lung disease constitute an important group in this respect and it is estimated that the overall incidence of 15%. Today, still diagnosis, treatment, follow-up, prognosis of IInterstitial lung disease due to Connective tissue diseases (CTD-ILD) related clinical problems have been experienced. The diagnostic methods of disease include clinical approach, pulmonary function tests, high-resolution computed tomography, bronchoalveolar lavage and surgical lung biopsy. Treatment and selection of drugs are still controversial. In this review, a CTD-ILD, separately for each diagnosis, pulmonary function tests and radiological features, prognosis and treatment approaches were evaluated.
___
- 1. Goh NSL, Desai SR, Veeraraghavan S, Hansell DM, Copley SJ, Maher TM, et al. Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med 2008; 177: 1248-54.
- 2. Behr J, Vogelmeier C, Beinert T, Meurer M, Krombach F, König G, et al. Bronchoalveolar lavage for evaluation and management of scleroderma disease of the lung. Am J Respir Crit Care Med 1996; 154: 400-6.
- 3. Goh NSL, Veeraraghavan S, Desai SR, Cramer D, Hansell DM, Denton CP, et al. Bronchoalveolar lavage cellular profiles in patients with systemic sclerosisassociated interstitial lung disease are not predictive of disease progression. Arthritis Rheum 2007; 56: 2005-12.
- 4. American Thoracic Society/European Respiratory and Society. International multidisciplinary consensus classification of the idiopathic interstitial pneumonia. Am J Respir Crit Care Med 2002; 165: 277-304.
- 5. Lee H, Kim DS, Yoo B, Seo JB, Rho J, Colby TV, et al. Histopathologic pattern and clinical features of rheumatoid arthritisassociated interstitial lung disease. Chest 2005; 127: 2019-27.
- 6. Tanaka N, Kim JS, Newell JD, Brown KK, Cool CD, Meehan R, et al. Rheumatoid arthritis-related lung diseases: CT findings. Radiology 2004; 232: 81-91.
- 7. Renzoni E, Rottoli P, Coviello G, Perari MG, Galeazzi M, Vagliasindi M. Clinical, laboratory and radiological findings in pulmonary fibrosis with and without connective tissue disease. Clin Rheumatol 1997; 16: 570-7.
- 8. Park JH, Kim DS, Park I, Jang SJ, Kitaichi M, Nicholson AG, et al. Prognosis of fibrotic interstitial pneumonia: idiopathic versus collagen vascular disease-related subtypes. Am J Respir Crit Care Med 2007; 175: 705-11.
- 9. 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 1980; 23: 581-90.
- 10. Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972-2002. Ann Rheum Dis 2007; 66: 940-4.
- 11. Reveille JD, Solomon DH. Evidence-based guidelines for the use of immunologic tests: anticentromere, Scl-70, and nucleolar antibodies. Arthritis Rheum 2003; 49: 399-412.
- 12. Steen VD, Medsger TA Jr. Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum 2000; 43: 2437-44.
- 13. Strange C, Bolster MB, Roth MD, Silver RM, Theodore A, Goldin J, et al; Scleroderma Lung Study Research Group. Bronchoalveolar lavage and response to cyclophosphamide in scleroderma interstitial lung disease. Am J Respir Crit Care Med 2008; 177: 91-8.
- 14. Tashkin DP, Elashoff R, Clements PJ, Goldin J, Roth MD, Furst DE, et al; Scleroderma Lung Study Research Group. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med 2006; 354: 2655-66.
- 15. Hoyles RK, Ellis RW, Wellsbury J, Lees B, Newlands P, Goh NS, et al. A multicenter, prospective, randomized, doubleblind, placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma. Arthritis Rheum 2006; 54: 3962-70.
- 16. Nadashkevich O, Davis P, Fritzler M, Kovalenko W. A randomized unblinded trial of cyclophosphamide versus azathioprine in the treatment of systemic sclerosis. Clin Rheumatol 2006; 25: 205-12.
- 17. Nannini C, West CP, Erwin PJ, Matteson EL. Effects of cyclophosphamide on pulmonary function in patients with scleroderma and interstitial lung disease: a systematic review and meta-analysis of randomized controlled trials and observational prospective cohort studies. Arthritis Res Ther 2008; 10: R124.
- 18. Condliffe R, Kiely DG, Peacock AJ, Corris PA, Gibbs JS, Vrapi F, et al. Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med 2009; 179: 151-7.
- 19. McLaughlin V, Humbert M, Coghlan G, Nash P, Steen V. Pulmonary arterial hypertension: the most devastating vascular complication of systemic sclerosis. Rheumatology (Oxford) 2009; 48(Suppl 3): iii25-31.
- 20. Dawson JK, Fewins HE, Desmond J, Lynch MP, Graham DR. Predictors of progression of HRCT diagnosed fibrosing alveolitis in patients with rheumatoid arthritis. Ann Rheum Dis 2002; 61: 517-21.
- 21. Hamblin MJ, Horton MR. Rheumatoid arthritis-associated interstitial lung disease: diagnostic dilemma. Pulm Med 2011; 2011: 872120.
- 22. Tsuchiya Y, Takayanagi N, Sugiura H, Miyahara Y, Tokunaga D, Kawabata Y, et al. Lung diseases directly associated with rheumatoid arthritis and their relationship to outcome. Eur Respir J 2011; 37: 1411-7.
- 23. Park JH, Kim DS, Park IN, Jang SJ, Kitaichi M, Nicholson AG, et al. Prognosis of fibrotic interstitial pneumonia: idiopathic versus collagen vascular disease-related subtypes. Am J Respir Crit Care Med 2007; 175: 705-11. 24. Cheema GS, Quismorio FPJ. Interstitial lung disease in systemic lupus erythematosus. Curr Opin Pulm Med 2000; 6: 424- 9.
- 25. Weinrib L, Sharma OP, Quismorio FPJ. A longterm study of interstitial lung disease in systemic lupus erythematosus. Semin Arthritis Rheum 1990; 20: 48-56.
- 26. Swigris JJ, Olson AL, Fischer A, Lynch DA, Cosgrove GP, Frankel SK, et al. Mycophenolate mofetil is safe, well tolerated, and preserves lung function in patients with connective tissue disease related interstitial lung disease Chest 2006; 130: 30-6.
- 27. Parambil JG, Myers JL, Lindell RM, Matteson EL, Ryu JH. Interstitial lung disease in primary Sjögren syndrome. Chest 2006; 130: 1489-95 28. Manoussakis MN, Moutsopoulos HM Sjögrens syndrome: current concepts. Adv Intern Med 2001; 47: 191-217.
- 29. Chen IJ, Jan Wu YJ, Lin CW, Fan KW, Luo SF, Ho HH, et al. Interstitial lung disease in polymyositis and dermatomyositis. Clin Rheumatol 2009; 28: 639-46.