Recent advances in the diagnosis and treatment of interstitial lung disease in systemic sclerosis (scleroderma): A review

Clinical and Experimental Rheumatology

Volumn 29, Issue 2 SUPPL. 65, 2011, Pages

  Tan, A ^a   Denton, C P ^a   Mikhailidis, D P ^a   Seifalian, Alexander M ^a  

^a UNIVERSITY COLLEGE LONDON   (United Kingdom)

Author keywords

Interstitial lung disease; Rheumatology; Scleroderma; Systemic sclerosis

Indexed keywords

CYCLOPHOSPHAMIDE; IMATINIB; METHYLPREDNISOLONE; MYCOPHENOLIC ACID 2 MORPHOLINOETHYL ESTER; NITRIC OXIDE; RITUXIMAB; AZATHIOPRINE; CORTICOSTEROID; MYCOPHENOLATE MOFETIL; PREDNISOLONE;

ALGORITHM; CELL THERAPY; COMPUTER ASSISTED TOMOGRAPHY; DEMOGRAPHY; DIAGNOSTIC ACCURACY; DIAGNOSTIC PROCEDURE; DISEASE ASSOCIATION; HUMAN; INTERSTITIAL LUNG DISEASE; NONHUMAN; PATHOGENESIS; PRACTICE GUIDELINE; PREVALENCE; PRIORITY JOURNAL; PROGNOSIS; RADIODIAGNOSIS; REVIEW; SCLERODERMA; SYSTEMIC SCLEROSIS; THORAX RADIOGRAPHY; COLLAGEN DEGRADATION; COMPUTER ASSISTED DIAGNOSIS; EXPIRED AIR; HIGH RESOLUTION COMPUTER TOMOGRAPHY; LUNG ALVEOLUS CELL TYPE 2; LUNG FIBROSIS; LUNG LAVAGE; LUNG TRANSPLANTATION; MESENCHYMAL STEM CELL; NEOPLASM; RHEUMATOID ARTHRITIS; STAGING;

ANTIRHEUMATIC AGENTS; AUTOIMMUNITY; DIAGNOSIS, COMPUTER-ASSISTED; DISEASE MANAGEMENT; EARLY DIAGNOSIS; FIBROSIS; HUMANS; IMMUNOSUPPRESSIVE AGENTS; LUNG; LUNG DISEASES, INTERSTITIAL; LUNG TRANSPLANTATION; SCLERODERMA, SYSTEMIC; SEVERITY OF ILLNESS INDEX; STEM CELL TRANSPLANTATION;

EID: 79960384725     PISSN: 0392856X     EISSN: 1593098X     Source Type: Journal    
DOI: None     Document Type: Review

References (90)

1. KIM R, MEYER KC: Therapies for interstitial lung disease: past, present and future. Ther Adv Respir Dis 2008; 2: 319-38.
2. POCOCK G, RICHARDS CD: The respiratory system. Human Physiology: The Basis of Medicine, 3 ed: Oxford, 2006: 324.
3. BOGATKEVICH GS, LUDWICKA-BRADLEY A, SINGLETON CB, BETHARD JR, SILVER RM: Proteomic analysis of CTGF-activated lung fibroblasts: identification of IQGAP1 as a key player in lung fibroblast migration. Am J Physiol Lung Cell Mol Physiol 2008; 295: L603-611.
4. HIGHLAND KB, SILVER RM: New developments in scleroderma interstitial lung disease. Curr Opin Rheumatol 2005; 17: 737-45.
5. CAHOON L: Biomedical research. More than skin deep. Science 2008; 322: 667-9.
6. KHANNA D, SEIBOLD JR, WELLS A et al.: Systemic Sclerosis-Associated Interstitial Lung Disease: Lessons from Clinical Trials, Outcome Measures, and Future Study Design. Curr Rheumatol Rev 2010; 6: 138-44.
7. BOIN F, DE FANIS U, BARTLETT SJ, WIGLEY FM, ROSEN A, CASOLARO V: T cell polarization identifies distinct clinical phenotypes in scleroderma lung disease. Arthritis Rheum 2008; 58: 1165-74.
8. FRECH T, KHANNA D, MARKEWITZ B, MINEAU G, PIMENTEL R, SAWITZKE A: Heritability of vasculopathy, autoimmune disease, and fibrosis in systemic sclerosis: a population-based study. Arthritis Rheum 2010; 62: 2109-16.
9. 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.
10. FATHI N, FURST DE, CLEMENTS PJ: Management of interstitial lung disease in systemic sclerosis: lessons from SLS and FAST. Curr Rheumatol Rep 2007; 9: 144-50.
11. MATHAI SC, HUMMERS LK, CHAMPION HC et al.: Survival in pulmonary hypertension associated with the scleroderma spectrum of diseases: impact of interstitial lung disease. Arthritis Rheum 2009; 60: 569-77.
12. VONK MC, BROERS B, HEIJDRA YF et al.: Systemic sclerosis and its pulmonary complications in The Netherlands: an epidemiological study. Ann Rheum Dis 2009; 68: 961-5.
13. VAN DER KAMP R, TAK PP, JANSEN HM, BRESSER P: Interstitial lung disease as the first manifestation of systemic sclerosis. Neth J Med 2007; 65: 390-4.
14. ANTONIOU KM, WELLS AU: Scleroderma lung disease: evolving understanding in light of newer studies. Curr Opin Rheumatol 2008; 20: 686-91.
15. MAYBERRY JP, PRIMACK SL, MULLER NL: Thoracic manifestations of systemic autoimmune diseases: radiographic and high-resolution CT findings. Radiographics 2000; 20: 1623-35.
16. FINESCHI S, BONGIOVANNI M, DONATI Y et al.: In vivo investigations on anti-fibrotic potential of proteasome inhibition in lung and skin fibrosis. Am J Respir Cell Mol Biol 2008; 39: 458-65.
17. KOWAL-BIELECKA O, KOWAL K, ROJEWSKA J et al.: Cyclophosphamide reduces neutrophilic alveolitis in patients with scleroderma lung disease: a retrospective analysis of serial bronchoalveolar lavage investigations. Ann Rheum Dis 2005; 64: 1343-6.
18. CHRISTMANN RB, WELLS AU, CAPELOZZI VL, SILVER RM: Gastroesophageal Reflux Incites Interstitial Lung Disease in Systemic Sclerosis: Clinical, Radiologic, Histopathologic, and Treatment Evidence. Semin Arthritis Rheum 2010.
19. CARLO-STELLA N, BELLOLI L, BARBERA R et al.: Gastroesophageal reflux and lung disease in systemic sclerosis. Am J Respir Crit Care Med 2009; 179: 1167; author reply 1167-8.
20. GILSON M, ZERKAK D, WIPFF J et al.: Prognostic factors for lung function in systemic sclerosis: prospective study of 105 cases. Eur Respir J 2010; 35: 112-7.
21. BUCH MH, DENTON CP, FURST DE et al.: Submaximal exercise testing in the assessment of interstitial lung disease secondary to systemic sclerosis: reproducibility and correlations of the 6-min walk test. Ann Rheum Dis 2007; 66: 169-73.
22. THOMEER MJ, VANSTEENKISTE J, VERBEKEN EK, DEMEDTS M: Interstitial lung diseases: characteristics at diagnosis and mortality risk assessment. Respir Med 2004; 98: 567-73.
23. SILVER RM, WELLS AU: Histopathology and bronchoalveolar lavage. Rheumatology (Oxford) 2008; 47 (Suppl. 5): v62-64.
24. 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.
25. WELLS AU: The clinical utility of bronchoalveolar lavage in diffuse parenchymal lung disease. Eur Respir Rev 2010; 19: 237-41.
26. DAMJANOV N, OSTOJIC P, KALOUDI O et al.: Induced sputum in systemic sclerosis interstitial lung disease: comparison to healthy controls and bronchoalveolar lavage. Respiration 2009; 78: 56-62.
27. KUMANOVICS G, MINIER T, RADICS J, PALINKAS L, BERKI T, CZIRJAK L: Comprehensive investigation of novel serum markers of pulmonary fibrosis associated with systemic sclerosis and dermato/polymyositis. Clin Exp Rheumatol 2008; 26: 414-20.
28. TIEV KP, CABANE J, AUBOURG F et al.: Severity of scleroderma lung disease is related to alveolar concentration of nitric oxide. Eur Respir J 2007; 30: 26-30.
29. TIEV KP, LE-DONG NN, DUONG-QUY S, HUAHUY T, CABANE J, DINH-XUAN AT: Exhaled nitric oxide, but not serum nitrite and nitrate, is a marker of interstitial lung disease in systemic sclerosis. Nitric Oxide 2009; 20: 200-6.
30. TIEV KP, COSTE J, ZIANI M, AUBOURG F, CABANE J, DINH-XUAN AT: Diagnostic value of exhaled nitric oxide to detect interstitial lung disease in systemic sclerosis. Sarcoidosis Vasc Diffuse Lung Dis 2009; 26: 32-8.
31. MOUTHON L, BEREZNE A, BRAUNER M, KAMBOUCHNER M, GUILLEVIN L, VALEYRE D: (Interstitial lung disease in systemic sclerosis). Rev Mal Respir 2007; 24: 1035-46.
32. FISCHER A, MEEHAN RT, FEGHALI-BOSTWICK CA, WEST SG, BROWN KK: Unique characteristics of systemic sclerosis sine scleroderma-associated interstitial lung disease. Chest 2006; 130: 976-81.
33. STROLLO D, GOLDIN J: Imaging lung disease in systemic sclerosis. Curr Rheumatol Rep 2010; 12: 156-61.
34. GOLDIN JG, LYNCH DA, STROLLO DC et al.: High-resolution CT scan findings in patients with symptomatic scleroderma-related interstitial lung disease. Chest 2008; 134: 358-67.
35. FISCHER A, MISUMI S, CURRAN-EVERETT D et al.: Pericardial abnormalities predict the presence of echocardiographically defined pulmonary arterial hypertension in systemic sclerosis-related interstitial lung disease. Chest 2007; 131: 988-92.
36. 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.
37. 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.
38. MARTEN K, DICKEN V, KNEITZ C et al.: Interstitial lung disease associated with collagen vascular disorders: disease quantification using a computer-aided diagnosis tool. Eur Radiol 2009; 19: 324-32.
39. 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.
40. BOGATKEVICH GS, TOURKINA E, ABRAMS CS, HARLEY RA, SILVER RM, LUDWICKABRADLEYA: Contractile activity and smooth muscle alpha-actin organization in thrombininduced human lung myofibroblasts. Am J Physiol Lung Cell Mol Physiol 2003; 285: L334-343.
41. BAUGHMAN RP, RAGHU G: Bronchoalveolar cellular analysis in scleroderma lung disease: does Sutton's law hold? Am J Respir Crit Care Med 2008; 177: 2-3.
42. HARVEY RA, CHAMPE PC, FINKEL R: Anticancer drugs. In: Lippincott's Illustrated Review: Pharmacology. Lippincott Williams & Wilkins 2009: 482.
43. MCGONAGLE D, TAN AL, MADDEN J et al.: Successful treatment of resistant scleroderma-associated interstitial lung disease with rituximab. Rheumatology (Oxford) 2008; 47: 552-3.
44. YOO WH: Successful treatment of steroid and cyclophosphamide-resistant diffuse scleroderma-associated interstitial lung disease with rituximab. Rheumatol Int 2010.
45. LAFYATIS R, O'HARA C, FEGHALI-BOSTWICK CA, MATTESON E: B cell infiltration in systemic sclerosis-associated interstitial lung disease. Arthritis Rheum 2007; 56: 3167-8.
46. MANNO R, BOIN F: Immunotherapy of systemic sclerosis. Immunotherapy 2010; 2: 863-78.
47. RANG HP, DALE MM, RITTER JM, FLOWER R: Anti-inflammatory and immunosuppressant drugs. In: Rang & Dale's Pharmacology. Churchill Livingstone Elsevier 2008: 243.
48. SWIGRIS JJ, OLSON AL, FISCHER A 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.
49. GERBINO AJ, GOSS CH, MOLITOR JA: Effect of mycophenolate mofetil on pulmonary function in scleroderma-associated interstitial lung disease. Chest 2008; 133: 455-60.
50. ZAMORA AC, WOLTERS PJ, COLLARD HR et al.: Use of mycophenolate mofetil to treat scleroderma-associated interstitial lung disease. Respir Med 2008; 102: 150-5.
51. SAKETKOO LA, ESPINOZA LR: Rheumatoid arthritis interstitial lung disease: mycophenolate mofetil as an antifibrotic and diseasemodifying antirheumatic drug. Arch Intern Med 2008; 168: 1718-9.
52. PLASTIRAS SC, VLACHOYIANNOPOULOS PG, TZELEPIS GE: Mycophenolate mofetil for interstitial lung disease in scleroderma. Rheumatology (Oxford) 2006; 45: 1572.
53. VANTHUYNE M, BLOCKMANS D, WESTHOVENS R et al.: A pilot study of mycophenolate mofetil combined to intravenous methylprednisolone pulses and oral low-dose glucocorticoids in severe early systemic sclerosis. Clin Exp Rheumatol 2007; 25: 287-92.
54. NIHTYANOVA SI. BROUGH GM, BLACK CM, DENTON CP: Mycophenolate mofetil in diffuse cutaneous systemic sclerosis - a retrospective analysis. Rheumatology (Oxford) 2007; 46: 442-5.
55. ClinicalTrials.gov. Comparison of therapeutic regimens for scleroderma interstitial lung disease (The Scleroderma Lung Study II) (SLS II) [Online]. http://clinicaltrials.gov/ct2/show/NCT00883129?term=scleroderma+lung+study& rank=1 Updated December 2010. Accessed December 2010.
56. PHUMETHUM V, JAMAL S, JOHNSON SR: Biologic Therapy for Systemic Sclerosis: A Systematic Review. J Rheumatol 2010.
57. GORDON JK, SPIERA RF: Targeting tyrosine kinases: a novel therapeutic strategy for systemic sclerosis. Curr Opin Rheumatol 2010; 22: 690-5.
58. RANG HP, DALE MM, RITTER JM, FLOWER R: Cancer chemotherapy. In: Rang & Dale's Pharmacology. Churchill Livingstone Elsevier 2008: 731.
59. DISTLER JH, DISTLER O: Imatinib as a novel therapeutic approach for fibrotic disorders. Rheumatology (Oxford) 2009; 48: 2-4.
60. SABNANI I, ZUCKER MJ, ROSENSTEIN ED et al.: A novel therapeutic approach to the treatment of scleroderma-associated pulmonary complications: safety and efficacy of combination therapy with imatinib and cyclophosphamide. Rheumatology (Oxford) 2009; 48: 49-52.
61. YIANNOPOULOS G, PASTROMAS V, ANTONOPOULOS I et al.: Combination of intravenous pulses of cyclophosphamide and methylprednizolone in patients with systemic sclerosis and interstitial lung disease. Rheumatol Int 2007; 27: 357-61.
62. GRIFFITHS B, MILES S, MOSS H, ROBERTSON R, VEALE D, EMERY P: Systemic sclerosis and interstitial lung disease: a pilot study using pulse intravenous methylprednisolone and cyclophosphamide to assess the effect on high resolution computed tomography scan and lung function. J Rheumatol 2002; 29: 2371-8.
63. PHILLIPS K, BYRNE-DUGAN C, BATTERSON E, SEIBOLD JR: Rapidly progressive fatal interstitial lung disease in a patient with systemic sclerosis. Nat Rev Rheumatol 2009; 5: 225-8.
64. RANG HP, DALE MM, RITTER JM, FLOWER R: Anti-inflammatory and immunosuppressant drugs. In: Rang & Dale's Pharmacology. Churchill Livingstone Elsevier 2008: 240.
65. HARVEY RA, CHAMPEPC, FINKEL R: Anticancer drugs. In: Lippincott's Illustrated Review: Pharmacology. Lippincott Williams & Wilkins 2009: 473.
66. RANG HP, DALE MM, RITTER JM, FLOWER R: Cancer chemotherapy. In: Rang & Dale's Pharmacology. Churchill Livingstone Elsevier 2008: 724.
67. TASHKIN DP, ELASHOFF R, CLEMENTS PJ et al.: Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med 2006; 354: 2655-66.
68. RANG HP, DALE MM, RITTER JM, FLOWER R: General Principles. In: Rang & Dale's Pharmacology. Churchill Livingstone Elsevier 2008: 95-7.
69. KUSCHNER WG: Cyclophosphamide in scleroderma lung disease. N Engl J Med 2006; 355: 1173; author reply 1174.
70. 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 2001; 176: 1026-34.
71. AIRO P, DANIELI E, ROSSI M et al.: Intravenous cyclophosphamide for interstitial lung disease associated to systemic sclerosis: results with an 18-month long protocol including a maintenance phase. Clin Exp Rheumatol 2007; 25: 293-6.
72. KHANNA D, FURST DE, CLEMENTS PJ, TASHKIN DP, ECKMAN MH: Oral cyclophosphamide for active scleroderma lung disease: a decision analysis. Med Decis Making 2008; 28: 926-37.
73. 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.
74. DOMICIANO DS, BONFA E, BORGES CT et al.: A long-term prospective randomized controlled study of non-specific interstitial pneumonia (NSIP) treatment in scleroderma. Clin Rheumatol 2010.
75. WANCHU A, SURYANARYANA BS, SHARMA S, SHARMA A, BAMBERY P: High-dose prednisolone and bolus cyclophosphamide in interstitial lung disease associated with systemic sclerosis: a prospective open study. Int J Rheum Dis 2009; 12: 239-42.
76. PESHIN R, O'NEILL L, BROWNE P, O'CONNELL P, KEARNS G: Early aggressive intra-venous pulse cyclophosphamide therapy for interstitial lung disease in a patient with systemic sclerosis. A case report. Clin Rheumatol 2009; 28 (Suppl. 1): S31-34.
77. BEREZNE A, RANQUE B, VALEYRE D et al.: Therapeutic strategy combining intravenous cyclophosphamide followed by oral azathioprine to treat worsening interstitial lung disease associated with systemic sclerosis: a retrospective multicenter open-label study. J Rheumatol 2008; 35: 1064-72.
78. 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.
79. KOWAL-BIELECKA O, LANDEWÉ R, AVOUAC J et al.: EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR). Ann Rheum Dis 2009; 68: 620-8.
80. SCHACHNA L, MEDSGER TA JR., DAUBER JH et al.: Lung transplantation in scleroderma compared with idiopathic pulmonary fibrosis and idiopathic pulmonary arterial hypertension. Arthritis Rheum 2006; 54: 3954-61.
81. SHITRIT D, AMITAL A, PELED N et al.: Lung transplantation in patients with scleroderma: case series, review of the literature, and criteria for transplantation. Clin Transplant 2009; 23: 178-83.
82. ROJAS M, XU J, WOODS CR et al.: Bone marrow-derived mesenchymal stem cells in repair of the injured lung. Am J Respir Cell Mol Biol 2005; 33: 145-52.
83. ZHAO F, ZHANG YF, LIU YG et al.: Therapeutic effects of bone marrow-derived mesenchymal stem cells engraftment on bleomycin-induced lung injury in rats. Transplant Proc 2008; 40: 1700-5.
84. MARINI FC: The complex love-hate relationship between mesenchymal stromal cells and tumors. Cytotherapy 2009; 11: 375-6.
85. SERRANO-MOLLAR A, NACHER M, GAYJORDI G, CLOSA D, XAUBET A, BULBENA O: Intratracheal transplantation of alveolar type II cells reverses bleomycin-induced lung fibrosis. Am J Respir Crit Care Med 2007; 176: 1261-8.
86. MANGAT P, CONRON M, GABBAY E, PROUDMAN S: Scleroderma Lung Disease; Variation in Screening, Diagnosis and Treatment Practices between Rheumatologists and Respiratory Physicians. Intern Med J 2009.
87. HIGGINS JPT, GREEN S: Cochrane Handbook for Systematic Reviews of Interventions. [Online]. http://www.cochrane-handbook.org/ Updated September 2009. Accessed September 2010.
88. TAKAHASHI K, TANABE K, OHNUKI M et al.: Induction of pluripotent stem cells from adult human fibroblasts by defined factors. Cell 2007; 131: 861-72.
89. CHIEN KR: Regenerative medicine and human models of human disease. Nature 2008; 453: 302-5.
90. LAUNAY D, MOUTHON L, HACHULLA E et al.: Prevalence and characteristics of moderate to severe pulmonary hypertension in systemic sclerosis with and without interstitial lung disease. J Rheumatol 2007; 34: 1005-11.