Abstract
Objective
The aim of the study is to assess the natural course of systemic sclerosis (SSc) in patients with interstitial lung involvement and to evaluate the effects of treatment.
Materials and methods
Sixty SSc patients with interstitial lung involvement were included in the study and history and retrospective data records of the patients were reviewed.
Results
It was observed that 47 patients (78.3%) had Raynaud’s phenomenon, 7 patients (11.7%) had skin involvement, 5 patients (8.3%) had joint involvement, and 1 patient (1.7%) had gastrointestinal system involvement as the first manifestation of the disease. Lung involvement had developed on an average of 113±106 months after the first manifestation of the disease and was apparent within the first year in 10 patients (16.7%), between 1 and 2 years in 3 patients (5%), between 2 and 3 years in 2 patients (3.3%), between 3 and 4 years in 5 patients (8.3%), between 4 and 10 years in 21 patients (35%), and after 10 years in 19 patients (31.7%). When the symptoms of lung involvement appeared, 37 patients (61.7%) were not receiving treatment while 23 (38.3%) were using an immunosuppressive agent. The time interval of lung involvement for the treated patients was 131±95 months while it was 101±112 months in untreated patients (p>0.05).
Conclusion
In SSc patients with intersititial pulmonary involvement, the disease frequently starts with Raynaud’s phenomenon and pulmonary symptoms tend to appear at a mean of 7 years after the onset of disease. The first sign of the disease, the probability of interstitial pulmonary involvement, is highest during the first 15 years after. Although this probability decreases after 15 years, in up to 10% of the patients, interstitial pulmonary involvement can still occur even up to 40 years. Immunosuppressive treatment is not effective in preventing the development of pulmonary involvement. However, it delays the manifestation of pulmonary symptoms for nearly 4 years.
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References
Black CM, Stephens SC (1993) Systemic sclerosis (scleroderma) and related disorders. In: Maddison PJ, Isenberg DA, Woo P, Glass DN (eds) Oxford textbook of rheumatology. Oxford University Press, Oxford, pp 771–789
Medsger TA (1993) Systemic sclerosis (scleroderma), localized scleroderma, eosinophilic fasciitis and calcinosis. In: McCarty DJ, Koopman WJ (eds) Arthritis and allied conditions, 12th edn. Lea and Febiger, Philadelphia, pp 1113–1143
Seibold JR (1997) Scleroderma. In: Kelley WN, Harris ED, Ruddy S, Sledge CB (eds) Textbook of rheumatology, 5th edn. Saunders, Philadelphia, pp 1133–1163
Le Roy EC (1996) Systemic sclerosis (scleroderma). In: Bennet SC, Plumm F, Glass DN (eds) Cecil’ s textbook of medicine. Saunders, Philadelphia, pp 1183–1188
Wigley MF (1998) Systemic sclerosis. In: Klippel JK, Dieppe PA (eds) Rheumatology. Mosby, St Louis, pp 79.1–79.14
Veale DJ, Collidge TA, Belch JJ (1995) Increased prevalence of symptomatic macrovascular disease in systemic sclerosis. Ann Rheum Dis 54:853–855
Silman A, Jannini S, Symmons D et al (1988) An epidemiologic study of scleroderma in West Midlands. Br J Rheumatol 27(4):286–290
Geirsson AJ, Steinsson K, Guthmundsson S et al (1994) Systemic sclerosis in Iceland. A nationwide epidemiologic study. Ann Rheum Dis 53(8):502–505
Roberts-Thomson PJ, Jones M, Hakendorf P et al (2001) Scleroderma in South Australia: epidemiological observations of possible pathogenic significance. Intern Med J 31(4):220–229
Steen VD, Oddis CV, Conte CG et al (1997) Incidence of systemic sclerosis in Allegheny County, Pennsylvania. A twenty-year study of hospital-diagnosed cases, 1963–1982. Arthritis Rheum 40(3):441–445
Mayes M, Laing T, Gillespie B et al (1996) Prevalence, incidence and survival rates of systemic sclerosis in the Detroit Metropolitan Area. Arthritis Rheum 39:S150, (Suppl)
Rossa AD, Valentini G, Bombardieri S et al (2001) European multicentre study to define disease activity criteria for systemic sclerosis. I. Clinical and epidemiological features of 290 patients from 19 centres. Ann Rheum Dis 60:585–591
Coghlan JG, Mukerjee D (2001) The heart and pulmonary vasculature in scleroderma: clinical features and pathobiology. Curr Opin Rheumatol 13(6):495–499
Garber SJ, Wells AU, duBois RM et al (1992) Enlarged mediastinal lymph nodes in the fibrosing alveolitis of systemic sclerosis. Br J Radiol 65(779):983–986
Schurawitzki H, Stiglbauer R, Granibger W et al (1990) Interstitial lung disease in progressive systemic sclerosis: high-resolution CT versus radiography. Radiology 176(3):755–759
Griedinger EL, Flaherty KT, White B et al (1998) African American race and antibodies to topoisomerase I are associated with increased severity of scleroderma lung disease. Chest 114:801–807
Steen VD, Conte C, Owens GR et al (1994) Severe restrictive lung disease in systemic sclerosis. Arthritis Rheum 37(9):1283–1289
Steen VD, Medsger JR TA (2000) Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum 43 (11):2437–2444
Shuford WH, Seaman WB, Goldman A (1953) Pulmonary manifestations of scleroderma. Arch Intern Med 92:85–97
Weaver AL, Divertie MB, Titus JL (1987) The lung in scleroderma. Mayo Clin Proc 42:754–766
Spain DM, Thomas AG (1950) The pulmonary manifestations of scleroderma: an anatomic–physiologic correlation. Ann Intern Med 32:152–161
Rodnan GP (1963) The natural history of progressive systemic sclerosis (diffuse scleroderma). Bull Rheum Dis 13:301–304
Antony TH, Lin MD, Philip J et al (2003) Update on disease-modifying antirheumatic drugs in the treatment of systemic sclerosis. Rheum Dis Clin North Am 29:409–426
White B (2003) Interstitial lung disease in scleroderma. Rheum Dis Clin North Am 29:371–390
Subcomittee for Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee (1980) Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 23(3):581–586
Bluestone R, Grahame R, Holloway V et al (1970) Treatment of systemic sclerosis with d-penisillamine. A new method of observing the effects of treatment. Ann Rheum Dis 29:153–158
Steen VD, Medsger TA, Rodnan GP (1982) d-Penisillamine therapy in progressive systemic sclerosis (scleroderma): a retrospective analysis. Ann Intern Med 97:652–659
Jimenez SA, Sigal SH (1991) A 15-year prospective study of treatment of rapidly progressive systemic sclerosis with d-penisillamine. J Rheumatol 18:1496–1503
Kang B, Veres-Thorner C, Heredia R et al (1982) Successful treatment of far-advanced progressive systemic sclerosis by d-penicillamine. J Allergy Clin Immunol 69:279–305
Steen VD, Owens GR, Redmond C et al (1985) The effect of d-penisillamine on pulmonary findings in progressive systemic sclerosis. Arthritis Rheum 28:882–888
DeClerck LS, Dequeker J, Francx L, Demedts M (1987) d-Penicillamine therapy and interstitial lung disease in scleroderma. Arthritis Rheum 30(6):643–650
Thomson J, Milne JA (1974) Two years of penicillamine for progressive syclerosis: a case report. Postgrad Med J 5:36–38
Smiley JD, Johnson RL Jr, Ziff M (1967) Effect of d-penicillamine on pulmonary function in patients with progressive systemic sclerosis (abstract). Arthritis Rheum 10:313–314
Clements PJ, Furst DE, Wong WK et al (1999) High-dose versus low-dose d-penisillamine in early diffuse systemic sclerosis analysis of a two-year, double-blind, randomized, controlled clinical trial. Arthritis Rheum 42:1194–1203
Rodnan GP, Black RL, Bollet AJ, Býnim JJ (1956) Observations on the use of prednisone in patients with progressive systemic sclerosis (diffuse scleroderma). Ann Intern Med 44:16–29
Van den Hoogen FH, Boerbooms AM, Van de Putte LB et al (1989) Methotrexate treatment in scleroderma. Am J Med 87:116–117
Van den Hoogen FH, Boerbooms AM, Van de Putte LB et al (1991) Low dose methotrexate treatment in systemic sclerosis [letter]. J Rheumatol 18:1763–1764
Van den Hoogen FH, Boerbooms AM, Swaak AJ et al (1996) 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 35:364–372
Pope JE, Bellamy N, Seibold JR et al (2001) A randomized, controlled trial of methotrexate versus placebo in early diffuse scleroderma. Arthritis Rheum 44:1351–1358
Silver RM, Warrick JH, Kinsella MB et al (1993) Cyclophosphamide and low-dose prednisone therapy in patients with systemic sclerosis (scleroderma) with interstitial lung disease. J Rheumatol 20:838–844
Akesson A, Scheja A, Lundin A et al (1994) Improved pulmonary function in systemic sclerosis after treatment with cyclophosphamide. Arthritis Rheum 37:729–735
Steen VD, Lanz KJ, Conte C et al (1994) Therapy for severe interstitial lung disease in systemic sclerosis. A retrospective study. Arthritis Rheum 37:1290–1296
Davas E, Peppas C, Maragou M et al (1999) Intravenous cyclophosphamide pulse therapy for the treatment of lung disease associated with scleroderma. Clin Rheumatol 18:458–461
Nadashkevich O, Davis P, Fritzler M, Kovalenko W (2006) Randomized unblinded trial of cyclophosphamide versus azathioprine in the treatment of systemic sclerosis. Clin Rheumatol 25(2):205–212
White B, Moore WC, Wigley FM et al (2000) Cyclophosphamide is associated with pulmonary function and survival benefit in patients with scleroderma and alveolitis. Ann Intern Med 132:947–954
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Benan, M., Hande, I. & Gul, O. The natural course of progressive systemic sclerosis patients with interstitial lung involvement. Clin Rheumatol 26, 349–354 (2007). https://doi.org/10.1007/s10067-006-0302-6
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DOI: https://doi.org/10.1007/s10067-006-0302-6