Management of interstitial lung disease in systemic sclerosis: Lessons from SLS and FAST

Current Rheumatology Reports

Volumn 9, Issue 2, 2007, Pages 144-150

  Fathi, Nihal ^b   Furst, Daniel E ^b   Clements, Philip J ^a  

^a UNIVERSITY OF CALIFORNIA   (United States)

^b NONE

Author keywords

[No Author keywords available]

Indexed keywords

AUTOANTIBODY; AZATHIOPRINE; CORTICOSTEROID; CYCLOPHOSPHAMIDE; METHOTREXATE; MYCOPHENOLIC ACID 2 MORPHOLINOETHYL ESTER; PENICILLAMINE; PLACEBO; PREDNISONE;

CLINICAL TRIAL; CONTROLLED CLINICAL TRIAL; DYSPNEA; ECHOCARDIOGRAPHY; FIBROSING ALVEOLITIS; HEMATURIA; HUMAN; INTERSTITIAL LUNG DISEASE; LEUKOPENIA; LUNG LAVAGE; NEUTROPENIA; PATHOGENESIS; PNEUMONIA; QUALITY OF LIFE; RANDOMIZED CONTROLLED TRIAL; REVIEW; SYSTEMIC SCLEROSIS;

CYCLOPHOSPHAMIDE; DISABILITY EVALUATION; HUMANS; IMMUNOSUPPRESSIVE AGENTS; LUNG; LUNG DISEASES, INTERSTITIAL; RANDOMIZED CONTROLLED TRIALS; RESPIRATORY FUNCTION TESTS; SCLERODERMA, SYSTEMIC;

EID: 34250679440     PISSN: 15233774     EISSN: 15346307     Source Type: Journal    
DOI: 10.1007/s11926-007-0009-y     Document Type: Review

References (17)

1. Mayes MD, Reveille JD: Epidemiology, demographics, and genetics. In Systemic Sclerosis, 2nd edn. Edited by Clements PJ, Furst DE. New York: Lippincott Williams & Wilkins; 2004:1-15.
2. Steen VD: Changes in causes of death in systemic sclerosis over the past 30 years. Arthritis Rheum 2004, 50:S422.
3. •• Tashkin DP, Elashoff R, Clements PJ, et al.: Oral cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med 2006, 354:2655-2666. The SLS trial showed significant but modest slowing of loss of FVC and TLC in the CYC group compared to placebo. More clinically and statistically significant improvement were also noted in dyspnea, function, quality of life, and skin thickening in the CYC group.
4. Steen VD, Conte C, Owens GR, Medsger TA Jr: Severe restrictive lung disease in systemic sclerosis. Arthritis Rheum 1994, 37:1283-1289.
5. Steen VD: Autoantibodies in systemic sclerosis. Semin Arthritis Rheum 2005, 35:35-42.
6. Charles C, Clements P, Furst DE: Systemic sclerosis: hypothesis-driven treatment strategies. Lancet 2006, 367:1683-1691.
7. Latsi PI, Wells AU: Evaluation and management of alveolitis and interstitial lung disease in scleroderma. Curr Opin Rheumatol 2003, 15:748-755.
8. Atamas SP, White B: The role of chemokines in the pathogenesis of scleroderma. Curr Opin Rheumatol 2003, 15:772-777.
9. Silver RM, Miller KS, Kinsella MB, et al.: Evaluation and management of scleroderma lung disease using bronchoalveolar lavage. Am J Med 1990, 88:470-476.
10. Silver RM, Warrick JH, Kinsella MB, et al.: Cyclophosphamide and low-dose prednisone therapy in patients with systemic sclerosis (scleroderma) with interstitial lung disease. J Rheumatol 1993, 20:838-844.
11. 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, 132:947-954.
12. Khanna D, Furst DE, Hays RD, et al.: Minimally important difference in diffuse systemic sclerosis: results from the D-penicillamine study. Ann Rheum Dis 2006, 65:1325-1329.
13. 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, in press.
14. •• Clements PJ, Furst DE, Silver RM, et al.: Outcomes at 24-months are better for patients who received one-year of cyclophosphamide (CYC) therapy for SSc alveolitis than in those who received one-year of placebo (PBO) in the Scleroderma Lung Study. Arthritis Rheum 2006, 54:S522. The 24-month results of the SLS (when patients had not taken study medication for 12 months) showed that the beneficial effects of CYC on FVC were lost at 24 months.
15. •• 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, 54:3962-3970. Although FAST was very underpowered, the trial confirmed the beneficial effects of CYC on FVC that had been shown in the SLS trial.
16. 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, 130:30-36.
17. Liossis SN, Bounas A, Andonopoulos AP: Mycophenolate mofetil as first-line treatment improves clinically evident early scleroderma lung disease. Rheumatology 2006, 45:1005-1008.