Добавил:
kiopkiopkiop18@yandex.ru Вовсе не секретарь, но почту проверяю Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз: Предмет: Файл:
5 курс / Пульмонология и фтизиатрия / Orphan_Lung_Diseases_A_Clinical_Guide_to_Rare.pdf
Скачиваний:
2
Добавлен:
24.03.2024
Размер:
74.03 Mб
Скачать

12  Systemic Sclerosis and the Lung

203

 

 

a 50

 

 

 

 

 

Echo normal

 

 

DLco

40

 

 

 

 

Mean PAP 35

Mean PAP 35

 

 

R heart failure

 

 

PVR 560 (form 800)

 

predicted

 

 

30

 

6MWD 130m to 190m

 

 

 

 

 

 

 

 

Petcentage

20

 

 

 

 

Sildenafil

 

 

10

Prostanoids (increasing doses, warfarin)

 

 

 

 

 

 

 

 

 

 

 

Oxygen dependency

 

 

0

 

 

 

 

0

24

48

72

Time (months)

b

75

Echo normal

 

 

 

Kco

60

Mean PAP 35

R heart failure

Mean PAP 35

 

 

 

PVR 560 (form 800)

 

 

 

 

 

 

 

 

6MWD 130m to 190m

 

predicted

45

 

 

 

 

 

 

 

 

 

Petcentage

30

 

Sildenafil

 

 

 

 

 

 

 

Prostanoids (increasing doses, warfarin)

 

 

15

 

Oxygen dependency

 

 

 

 

 

 

 

 

 

 

 

0

 

 

 

 

 

0

24

 

48

72

Time (months)

c

12.5

 

 

Mean PAP 35

 

 

PVR 560 (form 800)

(kpa)

10

6MWD 130m to 190m

 

 

R heart failure

gradient

 

7.5

Echo

 

Mean PAP 35

Aa

 

normal

 

 

pO2,

5

 

 

 

Sildenafil

2.5Prostanoids (increasing doses, warfarin)

Oxygen dependency

0

0

24

48

72

Time (months)

Fig. 12.5  Serial measures of gas exchange and gas transfer throughout follow-up are shown, including DLco (a), Kco (b) and pO2 and calculated alveolar-arterial oxygen gradient on air (c). Serial trends are shown in relation to relevant therapies and ndings at echocardiography and right heart catheterization. Serial Kco trends were most discriminatory as pulmonary hypertension developed, whereas gas exchange trends were more predictive of right heart decompensation

References

1.\Bairkdar M, Rossides M, Westerlind H, et al. Incidence and prevalence of systemic sclerosis globally: a comprehensive systematic review and meta-analysis. Rheumatology (Oxford). 2021;60:3121–33.

2.\American Thoracic Society/European Respiratory Society. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classi cation of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med. 2002;165:277–304.

3.\Tyndal AJ, Banert B, Vonk M, et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010;69:1809–15.

4.\Steen VD, Conte C, Owens GR, Medsger TA Jr. Severe restrictive lung disease in systemic sclerosis. Arthritis Rheum. 1994;37:1283–9.

5.\Elhai M, Meune C, Boubaya M, et al. Mapping and predicting mortality from systemic sclerosis. Ann Rheum Dis. 2017;76:1897–905.

6.\Li X, Qian Y-Q, Liu N, et al. Survival rate, causes of death, and risk factors in systemic sclerosis: a large cohort study. Clin Rheumatol. 2018;37:3051–6.

7.\Mayes MD, Lacey JV Jr, Beebe-Dimmer J, et al. Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum. 2003;48:2246–55.

8.\Reveille JD. Ethnicity and race and systemic sclerosis: how it affects susceptibility, severity, antibody genetics, and clinical manifestations. Curr Rheumatol Rev. 2003;5:160–7.

9.\D’Angelo WA, Fries JF, Masi AT, Shulman LE. Pathologic observations in systemic sclerosis (scleroderma). A study of fty-­ eight autopsy cases and fty-eight matched controls. Am J Med. 1969;46:428–40.

10.\Tansey D, Wells AU, Colby TV, et al. Variations in histological patterns of interstitial pneumonia between connective tissue disorders and their relationship to prognosis. Histopathology. 2004;44:585–96.

11.\Bouros D, Wells AU, Nicholson AG, et al. Histopathologic subsets of brosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Crit Care Med. 2002;165:1581–6.

12.\Fischer A, Swigris JJ, Groshong SD, et al. Clinically signi cant interstitial lung disease in limited scleroderma: histopathology, clinical features, and survival. Chest. 2008;134:601–5.

13.\Launay D, Remy-Jardin M, Michon-Pasturel U, et al. High resolution computed tomography in brosing alveolitis associated with systemic sclerosis. J Rheumatol. 2006;33:1789–801.

14.\Fischer A, Strek ME, Cottin V, et al. Proceedings of the American College of Rheumatology/Association of Physicians of Great Britain and Ireland Connective Tissue Disease–Associated Interstitial Lung Disease Summit: a multidisciplinary approach to address challenges and opportunities. Arthritis Rheum. 2019;71:182–95.

15.\Desai SR, Veeraraghavan S, Hansell DM, et al. CT features of lung disease in patients with systemic sclerosis: comparison with idiopathic pulmonary brosis and nonspeci c interstitial pneumonia. Radiology. 2004;232:560–7.

16.\Wells AU, Cullinan P, Hansell DM, et al. Fibrosing alveolitis associated with systemic sclerosis has a better prognosis than lone cryptogenic brosing alveolitis. Am J Respir Crit Care Med. 1994;149:1583–90.

17.\Kocheril SV, Appleton BE, Somers EC, et al. Comparison of disease progression and mortality of connective tissue disease-related interstitial lung disease and idiopathic interstitial pneumonia. Arthritis Rheum. 2005;53:549–57.

18.\Tashkin DP, Elashoff R, Clements PJ, et al. Scleroderma Lung Study Research Group. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med. 2006;354:2655–66.

Данная книга находится в списке для перевода на русский язык сайта https://meduniver.com/

204

A. U. Wells et al.

 

 

19.\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.

20.\Nihtyanova SI, Sari A, Harvey JC, et al. Using autoantibodies and cutaneous subset to develop outcome-based disease classi cation in systemic sclerosis. Arthritis Rheum. 2020;72:465–76.

21.\Walker UA, Tyndall A, Czirják L, et al. Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials And Research group database. Ann Rheum Dis. 2007;66:754–63.

22.\Briggs DC, Vaughan RW, Welsh KI, et al. Immunogenetic prediction of pulmonary brosis in systemic sclerosis. Lancet. 1991;338:661–2.

23.\Gilchrist FC, Bunn C, Foley PJ, et al. Class II HLA associations with autoantibodies in scleroderma: a highly signi cant role for HLA-DP. Genes Immun. 2001;2:76–81.

24.\Kuwana M, Kaburaki J, Mimori T, Kawakami Y, Tojo T. Longitudinal analysis of autoantibody response to topoisomerase I in systemic sclerosis. Arthritis Rheum. 2000;43:1074–84.

25.\Hu PQ, Fertig N, Medsger TA Jr, Wright TM. Correlation of serum anti-DNA topoisomerase I antibody levels with disease severity and activity in systemic sclerosis. Arthritis Rheum. 2003;48:1363–73.

26.\Reveille JD, Solomon DH. American College of Rheumatology Ad Hoc Committee of Immunologic Testing Guidelines. Evidence-based guidelines for the use of immunologic tests: anticentromere, Scl-70, and nucleolar antibodies. Arthritis Rheum. 2003;49:399–412.

27.\Feghali-Bostwick C, Medsger TA Jr, Wright TM. Analysis of systemic sclerosis in twins reveals low concordance for disease and high concordance for the presence of antinuclear antibodies. Arthritis Rheum. 2003;48:1956–63.

28.\Zhou X, Tan FK, Wang N, et al. Genome-wide association study for regions of systemic sclerosis susceptibility in a Choctaw Indian population with high disease prevalence. Arthritis Rheum. 2003;48:2585–92.

29.\Makino T, Jinnin M. Genetic and epigenetic abnormalities in systemic sclerosis. J Dermatol. 2016;43:10–8.

30.\Allanore Y, Dieude P, Boileau C. Genetic background of systemic sclerosis: autoimmune genes take centre stage. Rheumatology (Oxford). 2010;49:203–10.

31.\Stock CJW, Renzoni EA. Genetic predictors of systemic sclerosis-­ associated interstitial lung disease: a review of recent literature. Eur J Hum Genet. 2018;26:765–77.

32.\Sumita Y, Sugiura T, Kawaguchi Y, et al. Genetic polymorphisms in the surfactant proteins in systemic sclerosis in Japanese: T/T genotype at 1580 C/T (Thr131Ile) in the SP-B gene reduces the risk of interstitial lung disease. Rheumatology (Oxford). 2008;47:289–91.

33.\European Respiratory Society. Standardized lung function testing. Of cial statement of the European Respiratory Society. Eur Respir J Suppl. 1993;16:1–100.

34.\Wells AU, King AD, Rubens MB, Cramer D, du Bois RM, Hansell DM. Lone CFA: a functional-morphological correlation based on extent of disease on thin-section computed tomography. Am J Respir Crit Care Med. 1997;155:1367–75.

35.\Cottin V, Nunes H, Mouthon L, Gamondes D, Lazor R, Hachulla E, et al. Combined pulmonary brosis and emphysema syndrome in connective tissue disease. Arthritis Rheum. 2011;63:295–304.

36.\Nathan SD, Shlobin OA, Ahmad S, Urbanek S, Barnett SD. Pulmonary hypertension and pulmonary function testing in idiopathic pulmonary brosis. Chest. 2007;131:657–63.

37.\Steen VD, Graham G, Conte C, Owens G, Medsger TA Jr. Isolated diffusing capacity reduction in systemic sclerosis. Arthritis Rheum. 1992;35:765–70.

38.\Hughes JMB, Pride NB. Examination of the carbon monoxide diffusing capacity (DLCO) in relation to its KCO and VA components. Am J Respir Crit Care Med. 2012;186:132–9.

39.\Schurawitzki H, Stiglbauer R, Graninger W, et al. Interstitial lung disease in progressive systemic sclerosis: high resolution CT versus radiography. Radiology. 1990;176:755–9.

40.\Wells AU, Rubens MB, du Bois RM, Hansell DM. Serial CT inbrosing alveolitis: prognostic signi cance of the initial pattern. Am J Roentgenol. 1993;161:1159–65.

41.\Shah RM, Jimenez S, Wechsler R. Signi cance of ground-glass opacity on HRCT in long-term follow-up of patients with systemic sclerosis. J Thorac Imaging. 2007;22:120–4.

42.\Khanna D, Nagaraja V, Tseng CH, et al. Predictors of lung function decline in scleroderma-related interstitial lung disease based on high-resolution computed tomography: implications for cohort enrichment in systemic sclerosis-associated interstitial lung disease trials. Arthritis Res Ther. 2015;17:372.

43.\Moore OA, Goh N, Corte T, et al. Extent of disease on high-­ resolution computed tomography lung is a predictor of decline and mortality in systemic sclerosis-related interstitial lung disease. Rheumatology (Oxford). 2013;52:155–60.

44.\Goh NS, Hoyles RK, Denton CP, et al. Short-term pulmonary function trends are predictive of mortality in interstitial lung disease associated with systemic sclerosis. Arthritis Rheum. 2017;69:1670–8.

45.\Moore OA, Proudman SM, Goh N, et al. Quantifying change in pulmonary function as a prognostic marker in systemic sclerosis-­ related interstitial lung disease. Clin Exp Rheumatol. 2015;33(4 Suppl 91):S111–6.

46.\Bonifazi M, Sverzellati N, Negri E, et al. Pleuroparenchymal broelastosis in systemic sclerosis: prevalence and prognostic impact. Eur Respir J. 2020;56:1902135.

47.\DeMizio DJ, Bernstein EJ. Detection and classi cation of systemic sclerosis-related interstitial lung disease: a review. Curr Opin Rheumatol. 2019;31:553–60.

48.\Elhai M, Hoffmann-Vold AM, Avouac J, et al. Performance of candidate serum biomarkers for systemic sclerosis-interstitial lung disease. Arthritis Rheum. 2019;71:672–82.

49.\De Lauretis A, Sestini P, Pantelidis P, et al. Serum interleukin 6 is predictive of early functional decline and mortality in interstitial lung disease associated with systemic sclerosis. J Rheumatol. 2013;40:435–46.

50.\Goh NS, Veeraraghavan S, Desai SR, et al. Bronchoalveolar lavage cellular pro les in patients with systemic sclerosis-associated interstitial lung disease are not predictive of disease progression. Arthritis Rheum. 2007;56:2005–12.

51.\Strange C, Bolster MB, Roth MD, et al. Scleroderma Lung Study Research Group. Bronchoalveolar lavage and response to cyclophosphamide in scleroderma interstitial lung disease. Am J Respir Crit Care Med. 2008;177:91–8.

52.\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 brosis in scleroderma. Arthritis Rheum. 2006;54:3962–70.

53.\Seibold JR, Denton CP, Furst DE, et al. Randomized, prospective, placebo-controlled trial of bosentan in interstitial lung disease secondary to systemic sclerosis. Arthritis Rheum. 2010;62:2101–8.

54.\Khanna D, Lin CJF, Furst DE, et al. Tocilizumab in systemic sclerosis: a randomised, double-blind, placebo-controlled, phase 3 trial. Lancet Respir Med. 2020;8:963–74.

55.\Distler O, Highland KB, Gahlemann M, et al. Nintedanib for systemic sclerosis-associated interstitial lung disease. N Engl J Med. 2019;380:2518–28.

56.\Tashkin DP, Roth MD, Clements PJ, et al. Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial. Lancet Respir Med. 2016;4:708–19.

12  Systemic Sclerosis and the Lung

205

 

 

57.\Ebata S, Yoshizaki A, Oba K, et al. Safety and ef cacy of rituximab in systemic sclerosis (DESIRES): a double-blind, investigator-­ initiated, randomised, placebo-controlled trial. Lancet Rheumatol. 2021;3:e489–97.

58.\Kowal-Bielecka O, Landewe 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.

59.\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.

60.\White B, Moore WC, Wigley FM, Xiao HQ, Wise RA. Cyclophosphamide is associated with pulmonary function and survival bene t in patients with scleroderma and alveolitis. Ann Intern Med. 2000;132:947–54.

61.\Tzouvelekis A, Galanopoulos N, Bouros E, et al. Effect and safety of mycophenolate mofetil or sodium in systemic sclerosis-­ associated interstitial lung disease: a meta-analysis. Pulm Med. 2012;2012:143637.

62.\Steen VD, Medsger TA Jr. Case-control study of corticosteroids and other drugs that could precipitate or protect from the development of scleroderma renal crisis. Arthritis Rheum. 1998;41:1613–9.

63.\DeMarco PJ, Weisman MH, Seibold JR, et al. Predictors and outcomes of scleroderma renal crisis: the high-dose versus low-dose D-penicillamine in early diffuse systemic sclerosis trial. Arthritis Rheum. 2002;46:2983–9.

64.\Khanna D, et al. Safety and ef cacy of subcutaneous tocilizumab in systemic sclerosis: results from the open-label period of a phase II randomised controlled trial (faSScinate). Ann Rheum Dis. 2018;77:212–20.

65.\Highland KB, Distler O, Kuwana M, et al. Ef cacy and safety of nintedanib in patients with systemic sclerosis-associated interstitial lung disease treated with mycophenolate: a subgroup analysis of the SENSCIS trial. Lancet Respir Med. 2021;9:96–106.

66.\Flaherty KR, Wells AU, Cottin V, et al. Nintedanib in progressive brosing interstitial lung diseases. N Engl J Med. 2019;381:1718–27.

67.\Daoussis D, Liossis SN, Tsamandas AC, et al. Experience with rituximab in scleroderma: results from a 1-year, proof-of-principle study. Rheumatology. 2010;49:271–80.

68.\Daoussis D, Liossis SN, Tsamandas AC, et al. Effect of long-term treatment with rituximab on pulmonary function and skin brosis in patients with diffuse systemic sclerosis. Clin Exp Rheumatol. 2012;30:S17–22.

69.\Sem M, Molberg O, Lund MB, Gran JT. Rituximab treatment of the anti-synthetase syndrome: a retrospective case series. Rheumatology. 2009;48:968–71.

70.\Keir GJ, Maher TM, Hansell DM, et al. Severe interstitial lung disease in connective tissue disease: rituximab as rescue therapy. Eur Respir J. 2012;40:641–8.

71.\Daoussis D, Liossis SN, Tsamandas AC, et al. Experience with rituximab in scleroderma: results from a 1-year, proof-of-principle study. Rheumatology (Oxford). 2010;49:271–80.

72.\Burt RK, Shah SJ, Dill K, et al. Autologous non-myeloablative haemopoietic stem-cell transplantation compared with pulse cyclophosphamide once per month for systemic sclerosis (ASSIST): an open-label, randomised phase 2 trial. Lancet. 2011;378:498–506.

73.\Nash RA, McSweeney PA, Crofford LJ, et al. High-dose immunosuppressive therapy and autologous hematopoietic cell transplantation for severe systemic sclerosis: long-term follow-up of the US multicenter pilot study. Blood. 2007;110:1388–96.

74.\van Laar JM, Farge D, Sont JK, et al. Autologous hematopoietic stem cell transplantation vs intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis: a randomized clinical trial. JAMA. 2014;311:2490–8.

75.\Sullivan KM, Goldmuntz EA, Keyes-Elstein L, et al. Myeloablative autologous stem-cell transplantation for severe scleroderma. N Engl J Med. 2018;378:35–47.

Данная книга находится в списке для перевода на русский язык сайта https://meduniver.com/