FEATURES OF INTERSTITIAL LUNG DAMAGE IN SYSTEMIC SCLERODERMA (LITERATURE REVIEW AND AUTHORS' OWN DATA)


Cite item

Full Text

Open Access Open Access
Restricted Access Access granted
Restricted Access Subscription or Fee Access

Abstract

Interstitial lung damage (ILD) in systemic scferoderma (progressive systemic sclerosis) adversely affects the prognosis and is one of the leading causes of death. Early diagnosis and timely treatment can improve the prognosis of the disease. Therefore, in patients with the Raynaud's phenomenon, which is debut of the disease in most patients, it is necessary to pay attention to the signs characteristic of systemic rheumatic diseases (swelling of hands, myafgia in the proximal limbs, morning stiffness, arthralgia and arthritis, erythematous or hemorrhagic eruptions, xeronosus, dysphagia, anemia, cytopenia, etc.). According to the chest MSCT, ILD is detected in 80% of patients with SS. Histological findings are similar to that of idiopathic ILD, but the histological type does not determine the prognosis. The course of ILD in SS is relatively benign, often subclinical. According to the functional pulmonary tests, restrictive ventilation disorders are the most typical, and diffusing lung capacity is reduced in 80% of patients. Pulmonary tests have a prognostic value at the first examination - the lower the norm, the worse the prognosis. It is necessary to perform chest MSCT in all patients with a newly diagnosed SS, since the quantitative (prevalence) and qualitative (frosted glass, honeycomb lung) parameters influence the choice of therapeutic tactics. Treatment of ILD is carried out onfy in patients with obvious signs of progression determined by the dynamics of the decline in functional pulmonary function tests. For the treatment of ILD, immunosuppressants are used to slow down the progression. The drugs of choice include cyclophosphamide, an alternative to which is mycophenolate mofetil. Currently, genetically engineered biological preparations are being tested, in particular, tocilizumab, rituximab, and others.

Full Text

Restricted Access

About the authors

Lidiya P. ANANYEVA

Email: lpana@yandex.ru
MD, professor, head of the Laboratory of microcirculation and inflammation, V.A. Nasonova Research Institute of Rheumatology. 115522, Moscow, 34a Kashyrskoe shosse

Olga A. KONEVA

Email: alloy@yandex.ru
PhD, senior researcher, Laboratory of microcirculation and inflammation, V.A. Nasonova Research Institute of Rheumatology. 115522, Moscow, 34a Kashyrskoe shosse

Lyudmila V. TEPLOVA

Email: teplival@mail.ru
PhD, head of 22nd Department of rheumatology, O.M. Filatov City Clinical Hospital 111539, Moscow, 23 Veshnyakovskaya St

Viktor N. LESNYAK

Email: lesnyak_kb83@mail.ru
PhD, head of the Department of radiology with the MRI rooms, Federal Scientific Clinical Center of Specialized Types of Medical Care and Medical Technologies. 115682, Moscow, 28 Orekhovy bul

References

  1. Гусева Н.Г. Системная склеродермия. В кн.: Я.А. Сигидин, Н.Г. Гусева, М.М. Иванова. Диффузные болезни соединительной ткани. М.: Медицина, 2004. С. 341-57. [Guseva N.G. Systemic sclerosis. In the book: Ya.A. Sigidin, N.G. Guseva, M.M. Ivanova. Diffuse connective tissue diseases. M.: Medicine, 2004. P. 341-57 (in Russ.)]
  2. Varga J., Abraham D. Systemic sclerosis: a prototypic multisystem fibrotic disorder. J. Clin. Invest. 2007;1 17:557-6.
  3. Tyndall A.J., Bannert 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(10):1809-15.
  4. Bakst R., Merola J.E., Franks A.G. Jr, Sanchez M. Raynaud's phenomenon: pathogenesis and management. J. Am. Acad. Dermatol. 2008;59:633-53.
  5. Алекперов Р.Т. Синдром Рейно как мультидисциплинарная проблема. Альманах клинической медицины. 2014;35:94-100. [Alekperov R.T. Raynaud's syndrome as a multidisciplinary problem. Almanac of clinical medicine. 2014;35:94-100 (in Russ.)]
  6. Avouac J., Fransen J., Walker U.A., Riccieri V., Smith V., Muller C., Miniati I., Tarner I.H., Randone S.B., Cutolo M., Allanore Y., Distler O., Valentini G., Czirjak L., Müller-Ladner U., Furst D.E., Tyndall A., Matucci-Cerinic M. Preliminary criteria for the very early diagnosis of systemic sclerosis: results of Delphi Consensus Study from EULAR Scleroderma Trials and Research Group. Ann. Rheun. Dis. 2011;70:476-81.
  7. Corte T.G., Copley S.J., Desai S.R., Zappala C.J., Hansell D.M., Nicholson A.G., Colby T.V., Renzoni E., Maher T.M., Wells A.U. Significance of connective tissue disease features in idiopathic interstitial pneumonia. Eur. Respir. J. 2012;39:661-8.
  8. Wells A.U., Denton C.P. Interstitial lung disease in connective tissue disease -- mechanisms and management. Nat. Rev. Rheumatol. 2014;10(12):728-39.
  9. Nichtyanova S.I., Tang E.C., Coghlan J.G., Wells A.U., Black C.M., Denton C.P. Improved survival in systemic sclerosis is associated with better ascertainment of internal organ disease: a retrospective cohort study. QJM. 2010;103:109-15.
  10. Steen V.D., Medsger A.T. Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum. 2000;43:2437-44.
  11. Bouros D., Wells A.U., Nicholson A.G., Colby T.V., Polychronopoulos V., Pantelidis P., Haslam P.L., Vassilakis D.A., Black C.M., du Bois R.M. Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am. J. Respir. Crit. Care Med. 2002;165:1581-6.
  12. Park J.H., Kim D.S., Park I.N., Jang S.J., Kitaichi M., Nicholson A.G., Colby T.V. Prognosis of fibrotic interstitial pneumonia: idiopathic versus collagen vascular disease-related subtypes. Am. J. Respir. Crit. Care Med. 2007;175:705-1 1.
  13. Afeltra A., Zennaro D., Garzia P., Gigante A., Vadacca M., Ruggiero A., Dardes N., Navajas M.F., Zobel B.B., Amoroso A. Prevalence of interstitial lung involvement in patients with connective tissue diseases assessed with high-resolution computed tomography. Scand. J. Rheumatol. 2006;35(5):388-94.
  14. Теплова Л.В., Ананьева Л.П., Лесняк В.Н., Старовойтова М.Н., Десинова О.В., Невская Т.А., Александрова Е.Н. Системная склеродермия с интерстициальным поражением легких: сравнительная клиническая характеристика с больными без поражения легких. Научно-практическая ревматология. 2010;48(3):41 -7. [Teplova L.V., Ananyeva L.P., Lesnyak V.N., Starovoitova M.N., Desinova O.V., Nevskaya T.A., Aleksandrova Ye.N. Scleroderma systematica with interstitial lung lesion: comparative clinical characteristics with patients without lung lesion. Scientific practical rheumatology. 2010;48(3):41-7 (in Russ.)]
  15. Launay D., Remy-Jardin M., Michon-Pasturel U., Mastora I., Hachulla E., Lambert M., Delannoy V., Queyrel V., Duhamel A., Matran R., De Groote P., Hatron P.Y. High resolution computed tomography in fibrosing alveolitis associated with systemic sclerosis. J. Rheumatol. 2006;33:1789-801.
  16. Goldin J.G., Lynch D.A., Strollo D.C., Suh R.D., Schraufnagel D.E., Clements P.J., Elashoff R.M., Furst D.E., Vasunilashorn S., McNitt-Gray M.F., Brown M.S., Roth M.D., Tashkin D.P. High-resolution CT scan findings in patients with symptomatic scleroderma-related interstitial lung disease. Chest. 2008;134(2):358-67.
  17. Ананьева Л.П., Теплова Л.В., Лесняк В.Н., Конева О.А., Овсянникова О.Б., Старовойтова М.Н., Десинова О.В. Клиническая оценка проявлений интерстициальных поражений легких при системной склеродермии по данным компьютерной томографии высокого разрешения. Научно-практическая ревматология. 2011 ;49(2):30-9. [Ananyeva L.P., Teplova L.V., Lesnyak V.N., Koneva O.A., Ovsyannikova O.B., Starovoitova M.N., Desinov O.V. Clinical assessment of the manifestations of interstitial lung lesions in patients with systemic sclerosis according to the computer tomography of high resolution. Scientific practical rheumatology. 2011;49(2):30-9 (in Russ.)]
  18. Goh N.S., Desai S.R., Veeraraghavan S., Hansell D.M., Copley S.J., Maher T.M., Corte T.J., Sander C.R., Ratoff J., Devaraj A., Bozovic G., Denton C.P., Black C.M., du Bois R.M., Wells A.U. Interstitial lung disease in systemic sclerosis: a simple staging system. Am. J. Respir. Crit. Care Med. 2008;177(1 1):1248-54.
  19. Moore O.A., Goh N., Corte T., Rouse H., Hennessy O., Thakkar V., Byron J., Sahhar J., Roddy J., Gabbay E., Youssef P., Nash P., Zochling J., Proudman S.M., Stevens W., Nikpour M. 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(1):155-60.
  20. Goldin J., Elashoff R., Kim H.J., Yan X., Lynch D., Strollo D., Roth M.D., Clements P., Furst D.E., Khanna D., Vasunilas horn S., Li G., Tashkin D.P. 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(5):1333-40.
  21. Khanna D., Chi-Hong Tseng, Farmani N., Steen V., Furst D.E., Clements P.J., Roth M.D., Goldin J., Elashoff R., Seibold J.R., Saggar R., Tashkin D.P. Clinical course of lung physiology in patients with scleroderma and interstitial lung disease: Analysis of the Scleroderma Lung Study Placebo Group. Arthritis Rheum. 2011;63(10):3078-85.
  22. Assassi S., Sharif R., Lasky R.E., McNearney T.A., Estrada-Y-Martin R.M., Draeger H., Nair D.K., Fritzler M.J., Reveille J.D., Arnett F.C., Mayes M.D. Predictors of interstitial lung disease in early systemic sclerosis: a prospective longitudinal study of the GENISOS cohort. Arthritis Res. Ther. 2010;12:R166.
  23. Plastiras S.C., Karadimitrakis S.P., Ziakas P.D., Vlachoyiannopoulos P.G., Moutsopoulos H.M., Tzelepis G.E. Scleroderma lung: initial forced vital capacity as predictor of pulmonary function decline. Arthritis Rheum. 2006;55(4):598-602.
  24. Gilson M., Zerkak D., Wipff J., Dusser D., Dinh-Xuan A.T., Abitbol V., Chaussade S., Legmann P., Kahan A., Allanore Y. Prognostic factors for lung function in systemic sclerosis: prospective study of 105 cases. Eur. Respir. J. 2010;35(1):112-7.
  25. Winstone T.A., Assayag D., Wilcox P.G., Dunne J.V., Hague C.J., Collard H.R., Ryerson C.J. Predictors of mortality and progression in scleroderma-associated interstitial lung disease: a systematic review. Chest. 2014;146(2):422-36.
  26. Сосновская А.В., Фомин В.В., Попова Е.Н. Клиническое значение сурфактантного белка D как биомаркера фиброза легких у пациентов с системной склеродермией в зависимости от наличия гастроэзофагеального рефлюкса. Терапевтический архив. 2015;87(3):42-7. [Sosnovskaya A.V., Fomin V.V., Popova Ye.N. The clinical significance of surfactant protein D as a biomarker of pulmonary fibrosis in patients with systemic scleroderma, depending on the presence of gastroesophageal reflux. Therapeutic archive. 2015;87(3):42-7 (in Russ.)]
  27. Au K., Khanna D., Clements P.J., et al. Current concepts in disease-modifying therapy for systemic sclerosis-associated interstitial lung disease: lessons from clinical trials. Curr. Rheumatol. Rep. 2009;11(2):111-9.
  28. Kowal-Bielecka O., Fransen J., Avouac J., Becker M., Kulak A., Allanore Y., Distler O., Clements P., Cutolo M., Czirjak L., Damjanov N., Del Galdo F., Denton C.P., Distler J.H.W., Foeldvari I., Figelstone K., Frerix M., Furst D.E., Guiducci S., Hunzelmann N., Khanna D., Matucci-Cerinic M., Herrick A.L., van den Hoogen F., van Laar J.M., Riemekasten G., Silver R., Smith V., Sulli A., Tarner I., Tyndall A., Welling J., Wigley F., Valentini G., Walker U.A., Zulian F., Müller-Ladner U. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann. Rheum. Dis. 2016. pii: annrheumdis-2016-209909. [Epub ahead of print]
  29. Omair M.A., Alahmadi A., Johnson S.R. Safety and effectiveness of mycophenolate in systemic sclerosis. A systematic review. LoS One. 2015;10(5):e0124205.
  30. Khanna D., Denton C.P., Jahreis A., , van Laar J.M., Frech T.M., Anderson M.E., Baron M., Chung L., Fierlbeck G., Lakshminarayanan S., Allanore Y., Pope J.E., Riemekasten G., Steen V., Müller-Ladner U., Lafyatis R., Stifano G., Spotswood H., Chen-Harris H., Dziadek S., Morimoto A., Sornasse T., Siegel J., Furst D.E. Safety and efficacy of subcutaneous tocilizumab in adults with systemic sclerosis (faSScinate): a phase 2, randomised, controlled trial. Lancet. 2016;387(10038):2630-40.

Supplementary files

Supplementary Files
Action
1. JATS XML
Download

This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies