Scleroderma lung disease, variation in screening, diagnosis and treatment practices between rheumatologists and respiratory physicians

Internal Medicine Journal

Volumn 40, Issue 7, 2010, Pages 494-502

  Mangat, P ^a   Conron, M ^b   Gabbay, E ^c   Proudman, S M ^a,d  

^a ROYAL ADELAIDE HOSPITAL   (Australia)

^b ST VINCENT S HOSPITAL   (Australia)

^c ROYAL PERTH HOSPITAL   (Australia)

^d Australian Lung Foundation   (Australia)

Author keywords

diagnosis; hypertension; interstitial; lung diseases; pulmonary; scleroderma; systemic complications; therapeutics

Indexed keywords

BOSENTAN; BRAIN NATRIURETIC PEPTIDE; CALCIUM CHANNEL BLOCKING AGENT; CARBON MONOXIDE; CYCLOPHOSPHAMIDE; METHOTREXATE; PENTETIC ACID; PREDNISOLONE; WARFARIN;

ARTICLE; CLINICAL PRACTICE; DIAGNOSTIC TEST; DOPPLER ECHOCARDIOGRAPHY; E-MAIL; ECHOCARDIOGRAPHY; ELECTROCARDIOGRAM; HEART CATHETERIZATION; HIGH RESOLUTION COMPUTER TOMOGRAPHY; HUMAN; INTERSTITIAL LUNG DISEASE; LUNG DIFFUSION CAPACITY; LUNG FUNCTION TEST; LUNG LAVAGE; PHYSICIAN; PRIORITY JOURNAL; PULMONARY HYPERTENSION; RESPIRATORY PHYSICIAN; RHEUMATOLOGY; SCLERODERMA; SELF REPORT; THORAX RADIOGRAPHY;

CYCLOPHOSPHAMIDE; DATA COLLECTION; DIAGNOSIS, DIFFERENTIAL; DISEASE MANAGEMENT; HUMANS; HYPERTENSION, PULMONARY; LUNG DISEASES, INTERSTITIAL; MASS SCREENING; PHYSICIANS; RESPIRATION DISORDERS; RHEUMATOLOGY; SCLERODERMA, SYSTEMIC; TREATMENT OUTCOME; WARFARIN;

EID: 77954645111     PISSN: 14440903     EISSN: 14455994     Source Type: Journal    
DOI: 10.1111/j.1445-5994.2009.01990.x     Document Type: Article

References (38)

1. Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972-2002. Ann Rheum Dis 2007 66 : 940 944.
2. Mukerjee D, St George D, Coleiro B, Knight C, Denton CP, Davar J et al. Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis 2003 62 : 1088 1093.
3. Hachulla E, Gressin V, Loïc G, Carpentier P, Diot E, Sibilia J et al. Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum 2005 52 : 3792 3800.
4. Proudman SM, Stevens WM, Sahhar J, Celermajer D. Pulmonary arterial hypertension in systemic sclerosis: the need for early detection and treatment. Intern Med J 2007 37 : 485 494.
5. White B. Interstitial lung disease in scleroderma. Rheum Dis Clin North Am 2003 29 : 371 390.
6. Galie N, Rubin LJ, Hoeper MM, Jansa P, Kusic-Pajic A, Simonneau G. Abstract 1011: Bosentan improves hemodynamics and delays time to clinical worsening in patients with mildly symptomatic pulmonary arterial hypertension (PAH): results of the EARLY study. Eur Heart J 2007 28 (Abstract Supplement): 140.
7. Khanna D, Clements PJ, Furst DE, Park G, Merkel PA. Diagnostic and management preferences for lung disease in Scleroderma: results of survey of rheumatologists. Scleroderma Care and Research 2004 2 : 3 11.
8. Phung S, Strange G, Chung LP, Leong J, Dalton B, Roddy J et al. Prevalence of pulmonary arterial hypertension in an Australian scleroderma population: screening allows for earlier diagnosis. Intern Med J 2009 39 : 682 691.
9. National Pulmonary Hypertension Centres of the UK and Ireland. Consensus statement on the management of pulmonary hypertension in clinical practice in the UK and Ireland. Heart 2008 94 (Suppl 1 i1 41.
10. Rubin LJ, Badesch DB, Barst RJ, Galiè N, Black C, Keogh A et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002 346 : 896 903. (Pubitemid 34984638)
11. Mc Laughlin VV. Survival in patients with pulmonary arterial hypertension treated with first-line bosentan. Eur J Clin Invest 2006 36 (Suppl 3 10 15.
12. Denton CP, Pope JE, Peter HH, Gabrielli A, Boonstra A, van den Hoogen FH et al. Long-term effects of bosentan on quality of life, survival, safety and tolerability in pulmonary arterial hypertension related to connective tissue diseases. Ann Rheum Dis 2008 67 : 1222 1228.
13. Humbert M, Sitbon O, Chaouat A, Berticchi M, Habib G, Gressin V et al. Pulmonary arterial hypertension in France. Am J Respir Crit Care Med 2006 173 : 1023 1030.
14. Williams MH, Das C, Handler CE, Akram MR, Davar J, Denton CP et al. Systemic sclerosis associated pulmonary hypertension: improved survival in the current era. Heart 2006 92 : 926 932.
15. Rich S ed. Primary Pulmonary Hypertension: Executive Summary from the World Symposium on Primary Pulmonary Hypertension
16. Barst RJ, McGoon M, Torbicki A, Sitbon O, Krowka MJ, Olschewski H et al. Diagnosis and differential assessment of pulmonary arterial hypertension. J Am Coll Cardiol 2004 43 (12 Suppl 40S 47S. (Pubitemid 38759716)
17. McGoon M, Gutterman D, Steen V, Barst R, McCroroy DC, Fortin TA et al. Screening, early detection, and diagnosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004 126 (1 Suppl 14S 34S. (Pubitemid 38932340)
18. Steen V. Predictors of end stage lung disease in systemic sclerosis. Ann Rheum Dis 2003 62 : 97 9.
19. Borgeson DD, Seward JB, Miller FA Jr., Oh JK, Tajik AJ. Frequency of Doppler measurable pulmonary artery pressures. J Am Soc Echocardiogr 1996 9 : 832 837.
20. Mukerjee D, St George D, Knight C, Davar J, Wells AU, Du Bois RM et al. Echocardiography and pulmonary function as screening tests for pulmonary arterial hypertension in systemic sclerosis. Rheumatology (Oxford) 2004 43 : 461 466.
21. Rich S, Kaufmann E, Levy PS. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N Engl J Med 1992 327 : 76 81.
22. Walker UA, Tyndall A, Czirjak L, Denton C, Farge-Bancel D, Kowal-Bielecka O et al. Clinical risk assessment of organ manifestations in systemic sclerosis: a report from EULAR Scleroderma Trials and Research (EUSTAR) group database. Ann Rheum Dis 2007 66 : 754 763.
23. Sahhar J, Littlejohn G, Conron M. Fibrosing alveolitis in systemic sclerosis: the need for early screening and treatment. Intern Med J 2004 34 : 626 638.
24. Harrison NK, Glanville AR, Strickland B, Haslam PL, Corrin B, Addis BJ et al. Pulmonary involvement in systemic sclerosis: the detection of early changes by thin section CT scan, bronchoalveolar lavage and 99mTc-DTPA clearance. Respir Med 1989 83 : 403 414.
25. Warrick JH, Bhalla M, Schabel SI, Silver RM. High resolution computed tomography in early scleroderma lung disease. J Rheumatol 1991 18 : 1520 1528.
26. Buch MH, Denton CP, Guillevin L, Rubin LJ, Wells AU, Matucci-Cerinic M 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 : 169 173.
27. Villalba WO, Sampaio-Barros PD, Pereira MC, Cerqueira EMFP, Leme CA. Six minute walk test for the evaluation of pulmonary disease severity in scleroderma patients. Chest 2007 131 : 217 222.
28. Rossi GA, Bitterman PB, Rennard SI, Ferrans VJ, Crystal RG. Evidence for chronic inflammation as a component of the interstitial lung disease associated with progressive systemic sclerosis. Am Rev Respir Dis 1985 131 : 612 617.
29. Fuster V, Steele PM, Edwards WD, Gersh BJ, Mcgoon MD, Frye RL. Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation 1984 70 : 580 587.
30. Channick RN, Simonneau G, Sitbon O, Robbins IM, Frost A, Tapson VF et al. Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study. Lancet 2001 358 : 1119 1123. (Pubitemid 32971843)
31. 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 Resp Crit Care Med 2009 179 : 151 117.
32. Van Laar JM, Stolk J, Tyndall A. Scleroderma lung, pathogenesis, evaluation and current therapy. Drugs 2007 67 : 985 996.
33. Steen VD, Medsger TA Jr. Case-control study of corticosteroids and other drugs that either precipitate or protect from the development of scleroderma renal crisis. Arthritis Rheum 1998 41 : 1613 1619.
34. Tashkin DP, Elashoff R, Clements PJ, Goldin J, Roth MD, Furst DE et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med 2006 354 : 2655 2666.
35. Tashkin DP, Elashoff R, Clements PJ, Roth MD, Furst DE, Silver RM 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 1034.
36. Martinez FJ, McCune WJ. Cyclophosphamide for scleroderma lung disease. N Engl J Med 2006 354 : 2707.
37. Hoyles RK, Ellis RW, Wellsbury J, Lees B, Newlands P, Goh NS 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.
38. Van Den Hoogen FH, Boerbooms AM, Swaak AJ, Rasker JJ, van Lier HJ, van de Putte LB et al. Comparison of methotrexate with placebo in the treatment of systemic sclerosis: a 24 week randomized double-blind trial, followed by a 24 week observational trial. Br J Rheumatol 1996 35 : 364 372.