Systemic sclerosis-associated interstitial lung disease - Proposed recommendations for future randomised clinical trials

Clinical and Experimental Rheumatology

Volumn 28, Issue 2 SUPPL. 58, 2010, Pages

  Khanna, Dinesh ^a   Brown, Kevin K ^b   Clements, Philip J ^a   Elashoff, Robert ^a   Furst, Daniel E ^a   Goldin, Jonathan ^a   Seibold, James R ^c   Silver, Richard M ^d   Tashkin, Donald P ^a   Wells, Athol U ^e  

^a UNIVERSITY OF CALIFORNIA   (United States)

^b DEPARTMENT OF MEDICINE   (United States)

^c UNIVERSITY OF CONNECTICUT HEALTH CENTER   (United States)

^d MEDICAL UNIVERSITY OF SOUTH CAROLINA   (United States)

^e ROYAL BROMPTON HOSPITAL   (United Kingdom)

Author keywords

Interstitial lung disease; Systemic sclerosis

Indexed keywords

BOSENTAN; CYCLOPHOSPHAMIDE; GLYCOPROTEIN; KREBS VON DEN LUNGEN 6; PLACEBO; SURFACTANT PROTEIN D; UNCLASSIFIED DRUG; AZATHIOPRINE; BIOLOGICAL MARKER; LEUKOCYTE ELASTASE; MYCOPHENOLATE MOFETIL; POLYMORPHONUCLEAR NEUTROPHILIC LEUKOCYTE ELASTASE;

COHORT ANALYSIS; COMPUTER ASSISTED TOMOGRAPHY; DISEASE CLASSIFICATION; DISEASE COURSE; DRUG RESPONSE; DYSPNEA; FOLLOW UP; HUMAN; INTERSTITIAL LUNG DISEASE; LUNG FUNCTION; PATHOPHYSIOLOGY; PATIENT SAFETY; PRIORITY JOURNAL; PROGRESSION FREE SURVIVAL; QUALITY CONTROL; RANDOMIZED CONTROLLED TRIAL (TOPIC); REVIEW; SURVIVAL; SYSTEMIC SCLEROSIS; THORAX RADIOGRAPHY; DISEASE SEVERITY; FORCED VITAL CAPACITY; LUNG DIFFUSION CAPACITY; LUNG FUNCTION TEST; MULTICENTER STUDY (TOPIC); SCORING SYSTEM; SLOW VITAL CAPACITY; STAGING; SYSTEMIC SCLEROSIS ASSOCIATED INTERSTITIAL LUNG DISEASE; TOTAL LUNG CAPACITY; BIOASSAY; HOSPITALIZATION; METHODOLOGY; MULTICENTER STUDY; RANDOMIZED CONTROLLED TRIAL; TREATMENT OUTCOME; VITAL CAPACITY;

ENDPOINT DETERMINATION; HUMANS; LUNG DISEASES, INTERSTITIAL; MULTICENTER STUDIES AS TOPIC; RANDOMIZED CONTROLLED TRIALS AS TOPIC; RESEARCH DESIGN; SCLERODERMA, SYSTEMIC; SEVERITY OF ILLNESS INDEX; TREATMENT OUTCOME; VITAL CAPACITY;

EID: 77956621760     PISSN: 0392856X     EISSN: 1593098X     Source Type: Journal    
DOI: None     Document Type: Review

References (46)

1. STEEN VD. MEDSGER TA JR: Changes in causes of death in systemic sclerosis. Ann Rheum Dis 2007; 66: 940-4.
2. WHITE B, MOORE WC, WIGLEY FM et al.: Cyclophosphamide is associated with pulmonary function and survival benefit in patients with scleroderma and alveolitis. Ann Intern Med 2000; 132: 947-54.
3. TASHKIN DP, ELASHOFF R, CLEMENTS PJ et al.: Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med 2006; 354: 2655-66.
4. CLEMENTS PJ, ROTH MD, ELASHOFF R et al.: Scleroderma lung study (SLS): differences in the presentation and course of patients with limited versus diffuse systemic sclerosis. Ann Rheum Dis 2007; 66: 1641-7.
5. HOYLES RK, ELLIS RW, WELLSBURY J et al.: A multicenter, prospective, randomized, double-blind, 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.
6. BOUROS D, WELLS AU, NICHOLSON AG et al.: Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Crit Care Med 2002; 165: 1581-6.
7. KAZEROONI EA, MARTINEZ FJ, FLINT A et al.: Thin-section CT obtained at 10-mm increments versus limited three-level thin-section CT for idiopathic pulmonary fibrosis: correlation with pathologic scoring. AJR Am J Roentgenol 1997; 169: 977-83.
8. GOH NS, DESAI SR, VEERARAGHAVAN S et al.: Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med 2008; 177: 1248-54.
9. KIM HJ, LI G, GJERTSON D et al.: Classification of parenchymal abnormality in scleroderma lung using a novel approach to denoise images collected via a multicenter study. Acad Radiol 2008; 15: 1004-16.
10. GOLDIN J, ELASHOFF R, KIM HJ et al.: Treatment of scleroderma- interstitial lung disease with cyclophosphamide is associated with less progressive fibrosis on serial thoracic high-resolution CT scan than placebo: findings from the scleroderma lung study. Chest 2009; 136: 1333-40.
11. KIM H, GOLDIN J, TASHKIN D et al.: Cyclophosphamide Versus Placebo in Scleroderma Lung Study Using Quantitative Lung Fibrosis Score. 2009 International Conference of the Am. 2009: A3943.
12. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med 2002; 165: 277-304.
13. KEMERINK GJ, LAMERS RJ, THELISSEN GR et al.: CT densitometry of the lungs: scanner performance. j 1996; 20: 24-33.
14. KEMERINK GJ, KRUIZE HH, LAMERS RJ et al.: CT lung densitometry: dependence of CT number histograms on sample volume and consequences for scan protocol comparability. J Comput Assist Tomogr 1997; 21: 948-54.
15. AZIZ ZA, WELLS AU, HANSELL DM et al.: HRCT diagnosis of diffuse parenchymal lung disease: inter-observer variation. Thorax 2004; 59: 506-11.
16. HUNNINGHAKE GW, ZIMMERMAN MB, SCHWARTZ DA et al.: Utility of a lung biopsy for the diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2001; 164: 193-6.
17. MAYO JR, ALDRICH J, MULLER NL: Radiation exposure at chest CT: a statement of the Fleischner Society. Radiology 2003; 228: 15-21.
18. VAN DER BRUGGEN-BOGAARTS BA, BROERSE JJ, LAMMERS JW et al.: Radiation exposure in standard and high-resolution chest CT scans. Chest 1995; 107: 113-5.
19. TASHKIN DP, ELASHOFF R, CLEMENTS PJ et al.: Effects of 1-year treatment with cyclophosphamide on outcomes at 2 years in scleroderma lung disease. Am J Respir Crit Care Med 2007; 176: 1026-34.
20. PUNJABI NM, SHADE D, PATEL AM et al.: Measurement variability in single-breath diffusing capacity of the lung. Chest 2003; 123: 1082-9.
21. HATHAWAY EH, TASHKIN DP, SIMMONS MS: Intraindividual variability in serial measurements of DLCO and alveolar volume over one year in eight healthy subjects using three independent measuring systems. Am Rev Respir Dis 1989; 140: 1818-22.
22. PENNOCK BE, ROGERS RM, MCCAFFREE DR: Changes in measured spirometrie indices. What is significant? Chest 1981; 80: 97-9.
23. WANGER J, CLAUSEN JL, COATES A et al.: Standardisation of the measurement of lung volumes. Eur Respir J 2005; 26: 511-22.
24. MAClNTYRE N, CRAPO RO, VIEGI G et al.: Standardisation of the single-breath determination of carbon monoxide uptake in the lung. Eur Respir J 2005; 26: 720-35.
25. MILLER MR, HANKINSON J, BRUSASCO V et al.: Standardisation of spirometry. Eur Respir J 2005; 26: 319-38.
26. AU K, KHANNA D, CLEMENTS PJ et al.: Current concepts in disease-modifying therapy for systemic sclerosis-associated interstitial lung disease: lessons from clinical trials. Curr Rheumatol Rep 2009; 11: 111-9.
27. BOERS M, BROOKS P, STRAND CV et al.: The OMERACT filter for Outcome Measures in Rheumatology. J Rheumatol 1998; 25: 198-9.
28. KHANNA D, CLEMENTS PJ, FURST DE et al.: Correlation of the degree of dyspnea with health-related quality of life, functional abilities, and diffusing capacity for carbon monoxide in patients with systemic sclerosis and active alveolitis: results from the Scleroderma Lung Study. Arthritis Rheum 2005; 52: 592-600.
29. KHANNA D, YAN X, TASHKIN DP et al.: Impact of oral cyclophosphamide on health-related quality of life in patients with active scleroderma lung disease: results from the scleroderma lung study. Arthritis Rheum 2007; 56: 1676-84.
30. KHANNA D, TSENG CH, FURST DE et al.: Minimally important differences in the Mahler's Transition Dyspnoea Index in a large randomized controlled trial - results from the Scleroderma Lung Study. Rheumatology (Oxford) 2009; 48: 1547-40.
31. CHARLES C, CLEMENTS P, FURST DE: Systemic sclerosis: hypothesis-driven treatment strategies. Lancet 2006; 367: 1683-91.
32. FLEMING JN, NASH RA, McLEOD DO et al.: Capillary regeneration in scleroderma: stem cell therapy reverses phenotype? PLoS One 2008; 3: e1452.
33. YAMANE K, IHN H, KUBO M et al.: Serum levels of KL-6 as a useful marker for evaluating pulmonary fibrosis in patients with systemic sclerosis. J Rheumatol 2000; 27: 930-4.
34. MERKEL PA, SILLIMAN N, CLEMENTS P et al.: Patterns and Predictors of Change in Outcome Measures in Clinical Trials in Scleroderma. 2005.
35. AMERICAN THORACIC SOCIETY: Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS). Am J Respir Crit Care Med 2000; 161 (Pt. 1): 646-64.
36. SEIBOLD J, DENTON CP, GUILLEVIN L et al.: Randomized, prospective, placebo-controlled trial of bosentan in interstitial lung disease secondary to systemic sclerosis. 2010 (in press).
37. STRANGE C, BOLSTER MB, ROTH MD et al.: Bronchoalveolar lavage and response to cyclophosphamide in scleroderma interstitial lung disease. Am J Respir Crit Care Med 2008; 177: 91-8.
38. WELLS AU, LATSI P, MCCUNE WJ: Daily cyclophosphamide for scleroderma: are patients with the most to gain underrepresented in this trial? Am J Respir Crit Care Med 2007; 176: 952-3.
39. STEEN VD, CONTE C, OWENS GR et al.: Severe restrictive lung disease in systemic sclerosis. Arthritis Rheum 1994; 37: 1283-9.
40. FLAHERTY KR, MUMFORD JA, MURRAY S et al.: Prognostic implications of physiologic and radiographic changes in idiopathic interstitial pneumonia. Am J Respir Crit Care Med 2003; 168: 543-8.
41. COLLARD HR, KING TE JR, BARTELSON BB et al.: Changes in clinical and physiologic variables predict survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2003; 168: 538-42.
42. LATSI PI, DU BOIS RM, NICHOLSON AG et al.: Fibrotic idiopathic interstitial pneumonia: the prognostic value of longitudinal functional trends. Am J Respir Crit Care Med 2003; 168: 531-7.
43. YAN X, ELASHOFF R: Multivariate latent variable analysis with non-ignorable missing data (In Press). Computational statistics and data analysis 2010.
44. HANT FN, LUDWICKA-BRADLEY A, WANG HJ et al.: Surfactant protein D and KL-6 as serum biomarkers of interstitial lung disease in patients with scleroderma. J Rheumatol 2009; 36: 773-80.
45. ASANO Y, IHN H, YAMANE K et al.: Clinical significance of surfactant protein D as a serum marker for evaluating pulmonary fibrosis in patients with systemic sclerosis. Arthritis Rheum 2001; 44: 1363-9.
46. SATO S, NAGAOKA T, HASEGAWA M et al.: Elevated serum KL-6 levels in patients with systemic sclerosis: association with the severity of pulmonary fibrosis. Dermatology 2000; 200: 196-201.