Focused Issue of This Month Interstitial Lung Diseases in Collagen Vascular Diseases Sung Hwan Jeong, MD Department of Internal medicine, Gachon University of Medicine and Science E - mail : jsw@gilhospital.com J Korean Med Assoc 2009; 52(1): 30-40 Abstract Different types of interstitial lung diseases (ILDs) develop in collagen vascular diseases (CVDs) such as scleroderma, rheumatoid arthritis, systemic lupus erythematosus, dermatopolymyositis, Sjogren s syndrome, and mixed connective tissue disease. These CVDs represent various histological patterns, including usual interstitial pneumonia, desquamative interstitial pneumonia, nonspecific interstitial pneumonia, bronchiolitis obliterance organizing pneumonia, diffuse alveolar damage, and bronchiolitis. The clinical presentations, prognosis, and response to treatment vary depending on the underlying CVDs, as well as histological patterns of ILDs. In general, the prognosis and survival rate of ILDs in CVDs are better than idiopathic ILDs. Optimal treatment also varies depending on the type of CVDs and the presence of interstitial lung disease, although in many cases, a combination of corticosteroids and cytotoxic drugs are given. Keywords: Collagen vascular disease; Interstitial lung disease; Fibrosis 30
ILD in CVD Figure 1. Pulmonary hypertension in systemic sclerosis in a 46- year-old woman. Figure 2. Fibrosing alveolitis in systemic sclerosis, in a 46 -yearold woman. 31
Jeong SH 32
ILD in CVD Figure 3. RA with fibrosing alveolitis in a 47-year-old woman. CT scan shows honeycombing, irregular linear hyperattenuating areas in the basal areas of both lungs. Figure 4. RA with NSIP in a 48-year-woman. CT scan shows patchy distribution of irregular linear hyperattenuating areas and ground glass attenuation in both lungs. 33
Jeong SH Figure 5. RA with necrobiotic nodule in a 47-year-old woman. High-resolution computed tomography shows a rheumatoid nodules located in the both lungs. 34
ILD in CVD A B Figure 6. Systemic lupus erythematosus and acute lupus pneumonitis CT scans (3-mm collimation) obtained with lung window at the levels of the aortic arch (A) and lingular segmental bronchus (B) show airspace consolidation in the right upper lobe and groundglass attenuation in the left upper lobe. Bronchial dilatation is seen in the consolidation in the right upper lobe (RadioGraphics 2002; 22: S151-S165). Images by courtesy of Kyung Soo Lee, MD; Samsung Medical Center, Sungkyunkwan University School of Medicine. 35
Jeong SH Figure 7. SLE with NSIP in a 42-year-old woman. CT scan shows patchy distribution of irregular linear hyperattenuating areas and ground glass attenuation with traction bronchiectasis in both lungs. 36
ILD in CVD Figure 8. Dermatopolymyositis. High-resolution computed tomography at the level of the hila shows a bronchiolitis obliterans with organizing pneumonia pattern (ERJ 2001; 18: S32, 69s-80s). 37
Jeong SH A B C Figure 9. Dermatomyositis and usual interstitial pneumonia (A, B) CT scans obtained at the levels of the bronchus intermedius (A) and liver dome (B) show patchy areas of ground-glass attenuation and irregular linear hyperattenuating areas with random distribution in both lungs. (C) Photomicrograph (original magnification, x 12.5; H-E stain) shows irregular interstitial fibrosis (arrows) and mononuclear cell infiltration with subpleural predominance. Germinal center formation is also seen (arrowheads) (RadioGraphics 2002; 22: S151-S165). Images by courtesy of Kyung Soo Lee, MD; Samsung Medical Center, Sungkyunkwan University School of Medicine. A Figure 10. Sjögren s syndrome and lymphoid interstitial pneumonia (A) CT scan shows centrilobular nodules and branching linear structures (straight arrow) in the right lung. Many thin-walled cysts (curved arrows) were seen in both lungs. (B) Photomicrograph (original magnification, x 20; H-E stain) shows diffuse lymphocyte infiltration in the peribronchovascular interstitium and surrounding alveolar septa (arrows) (RadioGraphics 2002; 22: S151-S165). Images by courtesy of Kyung Soo Lee, MD; Samsung Medical Center, Sungkyunkwan University School of Medicine. B 38
ILD in CVD Figure 11. Mixed connective tissue disease and usual interstitial pneumonia. CT scan shows subpleural areas of groundglass attenuation, irregular linear hyperattenuating areas, and traction bronchiectasis and bronchiolectasis (arrows) (RadioGraphics 2002; 22: S151-S165). Images by courtesy of Kyung Soo Lee, MD; Samsung Medical Center, Sungkyunkwan University School of Medicine. 11. Marten K, Dicken V, Kneitz C, Hohmann M, Kenn W, Hahn D, Engelke C, Intrstitial lung disease associated with collagen vascular disorders:disease quantification using a computeraided diagnosis tool. Eur Radiol 2008; 11: 1. 12. Kim DS, Yoo B, Lee JS, Kim EK, Lim CM, Lee SD, Koh Y, Kim WS, Kim WD, Colby TV, Kitiaichi M. The major histopathologic pattern of pulmonary fibrosis in scleroderma is nonspecific interstitial pneumonia, Sarcoidosis Vasc Diffuse Lung Dis 2002; 19: 121-127. 13. Kim EA, Lee KS, Johkoh T, Kim TS, Suh GY, Kuon OJ, Han J. Interstitial Lung Diseases Associated with Collagen Vascular Diseases: Radiologic and Histopathologic Findings. Radio Graphics 2002; 22: S151-S165. 14. Oguz Uzun, Tekin Akpolat, Levent Erkan, Pulmonary Vasculitis in Behcet disease: A Cumulative Analysis 2005; 127: 2243-2253. 15. Parambil JG, Myers JL, Ryu JH. Diffuse Alveolar Damage: Uncommon Manifestation of Pulmonary Involvement in Patients With Connective Tissue Diseases. 2006; 130: 553-558. 16. Kang EH, Lee EB, Shin KC, Im CH, Chung DH, Han SK, Song YW. Interstitial lung disease in patients with plymyositis, dermatomyositis and amyopathic dermatomyositis, Rheumatology 2005; 44: 1282-1286. 17. F.Figen Ayhan-Ardie, Oznur Oken, Z.Rezan Yorgancioglu, Nilgun Ustun, F.Dilek Gokharman, Pulmonary involvement in lifelong non-somking patients with rheumatoid arthritis and ankylosing spondylitis without respiratory symtoms, Clin Rheumatol 2006; 25: 213-218. 18. Lamblin C, Bergoin C, Saelens T, Wallaert B. Interstitial lung diseases in collagen vascular diseases, Eur Respir J 2001; 18: S32, 69s-80s. 19. Suda T, Fujisawa T, Enomoto N, Nakamura Y, Inui N, Naito T, Hashimoto D, Sato J, Toyoshima M, Hashizume H, Chida K. Interstitial lung diseases associated with amyopathic dermatomyositis, Eur Respir J 2006; 28: 1005-1012. 10. Bodolay E, Szekanecz Z, Dévényi K, Galuska L, Csípo I, Vègh J, Garai I, Szegedi G. Rheumatology. Evaluation of interstitial lung disease in mixed connective tissue disease (MCTD), Rheutology 2005; 44: 656-661. 11. Wells AU, Hansell DM, Rubens MB, King AD, Cramer D, Black CM, du Bois RM. Fibrosing alveolitis in systemic sclerosis: indices of lung function in relation to extent of disease on computed tomography. Arthritis Rheum 1997; 40: 1229-1236. 12. Harrison NK, Glanville AR, Strickland B, Haslam PL, Corrin B, Addis BJ, Lawrence R, Millar AB, Black CM, Turner-Warwick M. Pulmonary involvement in systemic sclerosis: the detection of early changes by thin section CT scan, bronchoalveolar 39
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