Rituximab therapy is more effective than cyclophosphamide therapy for Japanese patients with anti‐topoisomerase I‐positive systemic sclerosis‐associated interstitial lung disease

2019 ◽  
Vol 46 (11) ◽  
pp. 1006-1013 ◽  
Author(s):  
Satoshi Ebata ◽  
Ayumi Yoshizaki ◽  
Takemichi Fukasawa ◽  
Shunsuke Miura ◽  
Takehiro Takahashi ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Topoisomerase I ◽  
Japanese Patients ◽  
Cyclophosphamide Therapy

scholarly journals Predictors of progression in systemic sclerosis patients with interstitial lung disease

2020 ◽  
Vol 55 (5) ◽  
pp. 1902026 ◽  
Author(s):  
Oliver Distler ◽  
Shervin Assassi ◽  
Vincent Cottin ◽  
Maurizio Cutolo ◽  
Sonye K. Danoff ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Disease Progression ◽  
Topoisomerase I ◽  
Diagnostic Tools ◽  
Systemic Autoimmune Disease ◽  
High Resolution Computed Tomography ◽  
Time Period ◽  
Organ Systems

Systemic sclerosis (SSc) is a systemic autoimmune disease affecting multiple organ systems, including the lungs. Interstitial lung disease (ILD) is the leading cause of death in SSc.There are no valid biomarkers to predict the occurrence of SSc-ILD, although auto-antibodies against anti-topoisomerase I and several inflammatory markers are candidate biomarkers that need further evaluation. Chest auscultation, presence of shortness of breath and pulmonary function testing are important diagnostic tools, but lack sensitivity to detect early ILD. Baseline screening with high-resolution computed tomography (HRCT) is therefore necessary to confirm an SSc-ILD diagnosis. Once diagnosed with SSc-ILD, patients' clinical courses are variable and difficult to predict, although certain patient characteristics and biomarkers are associated with disease progression. It is important to monitor patients with SSc-ILD for signs of disease progression, although there is no consensus about which diagnostic tools to use or how often monitoring should occur. In this article, we review methods used to define and predict disease progression in SSc-ILD.There is no valid definition of SSc-ILD disease progression, but we suggest that either a decline in forced vital capacity (FVC) from baseline of ≥10%, or a decline in FVC of 5–9% in association with a decline in diffusing capacity of the lung for carbon monoxide of ≥15% represents progression. An increase in the radiographic extent of ILD on HRCT imaging would also signify progression. A time period of 1–2 years is generally used for this definition, but a decline over a longer time period may also reflect clinically relevant disease progression.

scholarly journals AB0451 TO EVALUATE THE PREVALENCE OF INTERSTITIAL LUNG DISEASE (ILD) AND/OR PULMONARY ARTERIAL HYPERTENSION (PAH) IN PATIENTS AFFECTED BY SYSTEMIC SCLEROSIS (SSC) AND TO DETERMINE THE FACTORS ASSOCIATED WITH ILD

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1253.1-1253
Author(s):  
R. Ortega Castro ◽  
R. Mariscal-Ocaña ◽  
M. Rojas-Giménez ◽  
J. Calvo Gutierrez ◽  
A. Escudero Contreras ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Topoisomerase I ◽  
Systolic Pressure ◽  
University Hospital ◽  
Pulmonary Artery Systolic Pressure ◽  
Skin Involvement ◽  
Factors Associated ◽  
Anticentromere Antibodies

Background:Systemic sclerosis (SSc) is a chronic autoimmune disease that carries significant mortality. Despite diagnostic and therapeutic advances in recent years, there is still a significant percentage of patients who do not present a complete clinical response, with the associated increase in morbidity and mortality. Specifically, pulmonary disease is frequent and entails a poor prognosis, with interstitial lung disease (ILD) and pulmonary hypertension (PAH) being the two most important complications, the first and second cause of mortality, respectively.Objectives:To evaluate the prevalence of ILD and/or PAH in patients affected by SSc and to determine the factors associated with ILD.Methods:Cross-sectional observational study of 102 patients diagnosed with SSc (Limited, Diffuse, SSc without scleroderma or Pre-scleroderma), treated between 1975 and 2020 at the Reina Sofia University Hospital in Cordoba. A descriptive study of the cohort was carried out and factors independently associated with ILD were evaluated using a multiple logistic regression model.Results:102 patients were included, 87.3% of these were female with an average age of 50.8 (14) years. There were 20 deaths (19.8%), from which 55% died because of SSc and the main reason was ILD and/or PAH. Respiratory complications (as ILD or as PAH) were present in 59 patients (57.8%), of whom 52 were diagnosed with ILD (90.4% with a pattern of non-specific interstitial pneumonia) and 25 PAH, whose mean pulmonary artery systolic pressure was 47.16 (18.54) mmHg. Anti-topoisomerase I antibodies were positive in 34.6% of patients who developed ILD, while anticentromere antibodies were more frequent in SSc without interstitial lung disease (80%). Independent factors associated with ILD were type of SSc, proximal skin involvement, anticentromere antibodies, current treatment with corticoids and the death.Conclusion:Just over half of the patients with SSc have lung disease (as ILD or as PAH). The main risk factors associated with ILD are proximal skin involvement and treatment with glucocorticoids, probably in the context of more severe forms that require more treatment. Anticentromere antibodies are more prevalent in patients with Limited SSc and their expression decreases the risk of developing ILD in these patients.References:[1]Orlandi M, Barsotti S, Lepri G, et al. Clin Exp Rheumatol 2018 Jul-Aug; 36 Suppl 113: 3-23[2]Hao Y, Hudson M, Baron M, et al. Arthritis Rheumatol. 2017;69(5):1067-1077.[3]Furue M, Mitoma C, Mitoma H, et al. Immunol Res. 2017 Aug; 65: 790-7.[4]Nihtyanova SI, Schreiber BE, Ong VH, et al. Arthritis Rheumatol. 2014 Jun; 66: 1625-35.Disclosure of Interests:None declared.

scholarly journals High Prevalence of Acute Exacerbation of Interstitial Lung Disease in Japanese Patients with Systemic Sclerosis

2016 ◽  
Vol 239 (4) ◽  
pp. 297-305 ◽  
Author(s):  
Fumiko Tomiyama ◽  
Ryu Watanabe ◽  
Tomonori Ishii ◽  
Yukiko Kamogawa ◽  
Yoko Fujita ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Acute Exacerbation ◽  
Japanese Patients ◽  
High Prevalence

scholarly journals Clinical Relevance of the Serial Measurement of Krebs von den Lungen-6 Levels in Patients with Systemic Sclerosis-Associated Interstitial Lung Disease

Diagnostics ◽  
2021 ◽  
Vol 11 (11) ◽  
pp. 2007
Author(s):  
Yuichiro Shirai ◽  
Ryosuke Fukue ◽  
Yuko Kaneko ◽  
Masataka Kuwana
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Topoisomerase I ◽  
Significant Proportion ◽  
High Resolution Computed Tomography ◽  
Extensive Disease ◽  
Serial Measurement ◽  
Level Measurement ◽  
Wide Range

Krebs von den Lungen-6 (KL-6) levels measured at baseline have been reported as a circulating biomarker useful for the detection, evaluation of severity and assessment of risk of the progression of interstitial lung disease (ILD) in patients with systemic sclerosis (SSc). In this retrospective study, longitudinal changes in serum KL-6 levels over 2 years were examined in 110 patients with SSc using prospectively collected cohort data. Serum KL-6 levels fluctuated in a significant proportion of the patients but remained stable in the remaining patients. A wide range of variability of longitudinal KL-6 levels was associated with the presence of ILD, diffuse cutaneous SSc, positive anti-topoisomerase I antibodies, negative anticentromere antibodies, increased ILD extent on high-resolution computed tomography, extensive disease, low pulmonary function parameters, high KL-6 levels at baseline and immunomodulatory treatment. Extensive disease was consistently identified as an independent factor associated with variability in KL-6 levels in different models of multiple regression analysis. We failed to demonstrate correlations between trends for KL-6 level changes during the 6 months after SSc diagnosis and ILD progression over 2 years in patients with SSc-ILD. Serum KL-6 levels fluctuate in SSc patients with ILD, especially in those with extensive disease, but the clinical utility of a serial KL-6 level measurement remains uncertain.

scholarly journals Role of autoantibodies in the diagnosis and prognosis of interstitial lung disease in autoimmune rheumatic disorders

2021 ◽  
Vol 13 ◽  
pp. 1759720X2110324
Author(s):  
Masataka Kuwana ◽  
Albert Gil-Vila ◽  
Albert Selva-O’Callaghan
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Topoisomerase I ◽  
Initiation Factor ◽  
Severe Condition ◽  
Diagnosis And Prognosis ◽  
Antisynthetase Antibodies ◽  
Substantial Morbidity

Interstitial lung disease (ILD) has been recognized as a frequent manifestation associated with a substantial morbidity and mortality burden in patients with autoimmune rheumatic disorders. Serum autoantibodies are considered good biomarkers for identifying several subsets or specific phenotypes of ILD involvement in these patients. This review features the role of several autoantibodies as a diagnostic and prognostic biomarker linked to the presence ILD and specific ILD phenotypes in autoimmune rheumatic disorders. The case of the diverse antisynthetase antibodies in the antisynthease syndrome or the anti-melanoma differentiation-associated 5 protein (MDA5) antibodies as a marker of a severe condition such as rapidly progressive ILD in patients with clinically amyopathic dermatomyositis are some of the associations herein reported in the group of myositis spectrum disorders. Specific autoantibodies such as the well-known anti-topoisomerase I (anti-Scl70) or the anti-Th/To, anti-U11/U12 ribonucleoprotein, and anti-eukaryotic initiation factor 2B (eIF2B) antibodies seems to be specifically linked to ILD in patients with systemic sclerosis. Overlap syndromes between systemic sclerosis and myositis, also have good ILD biomarkers, which are the anti-PM/Scl and anti-Ku autoantibodies. Lastly, other not so often reported disorders as being associated with ILD but recently most recognized as is the case of rheumatoid arthritis associated ILD or entities herein included in the miscellaneous disorders section, which include anti-neutrophil cytoplasmic antibody-associated interstitial lung disease, Sjögren’s syndrome or the mixed connective tissue disease, are also discussed.

scholarly journals Circulating biomarkers of systemic sclerosis – interstitial lung disease

2020 ◽  
Vol 5 (2_suppl) ◽  
pp. 41-47 ◽  
Author(s):  
Anna-Maria Hoffmann-Vold ◽  
Håvard Fretheim ◽  
Chantal Meier ◽  
Britta Maurer
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Clinical Application ◽  
Topoisomerase I ◽  
Early Stage ◽  
Organ Damage ◽  
Disease Diagnosis ◽  
Surfactant Protein D ◽  
Circulating Biomarkers

Interstitial lung disease is a frequent organ manifestation in systemic sclerosis and is associated with high mortality. It is crucial to diagnose interstitial lung disease in systemic sclerosis and to assess severity and identify patients prone to progression at an early stage to ultimately decrease organ damage and improve outcome. Circulating anti-topoisomerase-I autoantibodies have long been associated with the presence and development of systemic sclerosis – interstitial lung disease, evidence on their potential to further predict the clinical course of systemic sclerosis is however conflicting. C-reactive protein is a marker of infection and systemic inflammation with widespread clinical application and is elevated in systemic sclerosis with a tendency towards higher abundancy in patients with early disease. The role of other circulating biomarkers is promising but hampered by the lack of standardized criteria and guidelines for sample/data collection, analyses, reporting and validation and has not reached prime time for clinical application. However, epithelial markers including Krebs von den Lungen-6 and surfactant protein D and several cytokines and chemokines including CCL2 and CCL18 for severity assessment of systemic sclerosis – interstitial lung disease patients at the time of interstitial lung disease diagnosis and to predict interstitial lung disease progression have been reported and seem to be promising candidate biomarkers in the future.

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