Interstitial lung disease in systemic sclerosis (systemic scleroderma)
In systemic sclerosis (SSc), interstitial lung disease (ILD) is common (>80%) and worsens the prognosis of the disease, but severe progressive damage develops only in 8–10% of cases. Interstitial changes in the lungs occur early (within the first 3–5 years of the disease). The histological ma...
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IMA-PRESS LLC
2021-03-01
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| Series: | Современная ревматология |
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| Online Access: | https://mrj.ima-press.net/mrj/article/view/1114 |
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| author | L. P. Ananyeva I. E. Tyurin O. A. Koneva L. A. Garzanova A. M. Lila |
| author_facet | L. P. Ananyeva I. E. Tyurin O. A. Koneva L. A. Garzanova A. M. Lila |
| author_sort | L. P. Ananyeva |
| collection | DOAJ |
| description | In systemic sclerosis (SSc), interstitial lung disease (ILD) is common (>80%) and worsens the prognosis of the disease, but severe progressive damage develops only in 8–10% of cases. Interstitial changes in the lungs occur early (within the first 3–5 years of the disease). The histological manifestations are similar to those of idiopathic ILD.The main tool for screening and diagnosing of ILD associated with SSc is high-resolution computed tomography of the lungs, resulting data influence the choice of therapy. In most patients a relatively intact and stable forced vital capacity of the lungs is recorded for a long time, but the diffusion capacity of the lungs decreases early and steadily. Pulmonary functional tests have prognostic value.The choice of the optimal therapy for SSc with lung lesions is based on general disease activity (the severity of inflammation and fibrosis) and the its severity, rate of progression of the disease in general and the leading pathology – interstitial pneumonia (IP) – in particular. In patients with SSc and severe or progressive IP, treatment with mycophenolate mofetil (MMF), cyclophosphamide, nintedanib, or nintedanib in combination with MMF if appropriate, should be considered. If this therapy is ineffective, rituximab may be used. |
| format | Article |
| id | doaj-art-2d1e7e8a2a0446d6a02adb862ff96e81 |
| institution | DOAJ |
| issn | 1996-7012 2310-158X |
| language | Russian |
| publishDate | 2021-03-01 |
| publisher | IMA-PRESS LLC |
| record_format | Article |
| series | Современная ревматология |
| spelling | doaj-art-2d1e7e8a2a0446d6a02adb862ff96e812025-08-20T03:20:58ZrusIMA-PRESS LLCСовременная ревматология1996-70122310-158X2021-03-01151S16210.14412/1996-7012-2021-1S-1-622336Interstitial lung disease in systemic sclerosis (systemic scleroderma)L. P. Ananyeva0I. E. Tyurin1O. A. Koneva2L. A. Garzanova3A. M. Lila4V.A. Nasonova Research Institute of RheumatologyRussian Medical Academy of Continuous Professional EducationV.A. Nasonova Research Institute of RheumatologyV.A. Nasonova Research Institute of RheumatologyV.A. Nasonova Research Institute of Rheumatology; Russian Medical Academy of Continuous Professional EducationIn systemic sclerosis (SSc), interstitial lung disease (ILD) is common (>80%) and worsens the prognosis of the disease, but severe progressive damage develops only in 8–10% of cases. Interstitial changes in the lungs occur early (within the first 3–5 years of the disease). The histological manifestations are similar to those of idiopathic ILD.The main tool for screening and diagnosing of ILD associated with SSc is high-resolution computed tomography of the lungs, resulting data influence the choice of therapy. In most patients a relatively intact and stable forced vital capacity of the lungs is recorded for a long time, but the diffusion capacity of the lungs decreases early and steadily. Pulmonary functional tests have prognostic value.The choice of the optimal therapy for SSc with lung lesions is based on general disease activity (the severity of inflammation and fibrosis) and the its severity, rate of progression of the disease in general and the leading pathology – interstitial pneumonia (IP) – in particular. In patients with SSc and severe or progressive IP, treatment with mycophenolate mofetil (MMF), cyclophosphamide, nintedanib, or nintedanib in combination with MMF if appropriate, should be considered. If this therapy is ineffective, rituximab may be used.https://mrj.ima-press.net/mrj/article/view/1114systemic sclerosis (scleroderma)interstitial lung diseaselung function testshigh-resolution computed tomographydiagnosticstreatment |
| spellingShingle | L. P. Ananyeva I. E. Tyurin O. A. Koneva L. A. Garzanova A. M. Lila Interstitial lung disease in systemic sclerosis (systemic scleroderma) Современная ревматология systemic sclerosis (scleroderma) interstitial lung disease lung function tests high-resolution computed tomography diagnostics treatment |
| title | Interstitial lung disease in systemic sclerosis (systemic scleroderma) |
| title_full | Interstitial lung disease in systemic sclerosis (systemic scleroderma) |
| title_fullStr | Interstitial lung disease in systemic sclerosis (systemic scleroderma) |
| title_full_unstemmed | Interstitial lung disease in systemic sclerosis (systemic scleroderma) |
| title_short | Interstitial lung disease in systemic sclerosis (systemic scleroderma) |
| title_sort | interstitial lung disease in systemic sclerosis systemic scleroderma |
| topic | systemic sclerosis (scleroderma) interstitial lung disease lung function tests high-resolution computed tomography diagnostics treatment |
| url | https://mrj.ima-press.net/mrj/article/view/1114 |
| work_keys_str_mv | AT lpananyeva interstitiallungdiseaseinsystemicsclerosissystemicscleroderma AT ietyurin interstitiallungdiseaseinsystemicsclerosissystemicscleroderma AT oakoneva interstitiallungdiseaseinsystemicsclerosissystemicscleroderma AT lagarzanova interstitiallungdiseaseinsystemicsclerosissystemicscleroderma AT amlila interstitiallungdiseaseinsystemicsclerosissystemicscleroderma |