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

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.

2020 ◽  
Vol 55 (5) ◽  
pp. 1902026 ◽  
Author(s):  
Oliver Distler ◽  
Shervin Assassi ◽  
Vincent Cottin ◽  
Maurizio Cutolo ◽  
Sonye K. Danoff ◽  
...  

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.


2017 ◽  
Vol 53 (3) ◽  
pp. 120-127
Author(s):  
Alfredo Guillen-del Castillo ◽  
Sara Sánchez-Vidaurre ◽  
Carmen P. Simeón-Aznar ◽  
María J. Cruz ◽  
Vicente Fonollosa-Pla ◽  
...  

2016 ◽  
Vol 68 (11) ◽  
pp. 2778-2783 ◽  
Author(s):  
Zoe E. Betteridge ◽  
Felix Woodhead ◽  
Hui Lu ◽  
Gavin Shaddick ◽  
Christopher C. Bunn ◽  
...  

2019 ◽  
Vol 47 (7) ◽  
pp. 991-1000 ◽  
Author(s):  
Marwin Gutierrez ◽  
Carina Soto-Fajardo ◽  
Carlos Pineda ◽  
Alfonso Alfaro-Rodriguez ◽  
Lene Terslev ◽  
...  

Objective.To provide an overview of the role of lung ultrasound (LUS) in the assessment of interstitial lung disease (ILD) in systemic sclerosis (SSc) and to discuss the state of validation supporting its clinical relevance and application in daily clinical practice.Methods.Original articles published between January 1997 and October 2017 were included. To identify all available studies, a detailed search pertaining to the topic of review was conducted according to guidelines of the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA). A systematic search was performed in PubMed and EMBASE. The quality assessment of retrieved articles was performed according to the Oxford Center for Evidence-based Medicine. The methodological quality of the studies was assessed using the Cochrane Handbook for Systematic Reviews and the Quality Assessment of Diagnostic Accuracy Studies–2 tool.Results.From 300 papers identified, 12 were included for the analysis. LUS passed the filter of face, content validity, and feasibility. However, there is insufficient evidence to support criterion validity, reliability, and sensitivity to change.Conclusion.Despite a great deal of work supporting the potential role of LUS for the assessment of ILD-SSc, much remains to be done before validating its use as an outcome measure in ILD-SSc.


2017 ◽  
Vol 47 (2) ◽  
pp. 228-234 ◽  
Author(s):  
Vanessa Hax ◽  
Markus Bredemeier ◽  
Ana Laura Didonet Moro ◽  
Thaís Rohde Pavan ◽  
Marcelo Vasconcellos Vieira ◽  
...  

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 897.2-898
Author(s):  
M. Orlandi ◽  
N. Landini ◽  
G. Sambataro ◽  
C. Nardi ◽  
C. Bruni ◽  
...  

Background:COVID-19 pandemic is a global emergency which may overlap on the clinical and radiological scenario of ILD in SSc. In clinical practice, the striking similarities observed at computed tomography (CT) between the diseases make it difficult to distinguish a COVID-19 superinfection from a progression of SSc-ILD.Objectives:The aim of our study was to identify the main CT features that may help distinguishing SSc-ILD from COVID-19 pneumonia.Methods:22 international readers were included and divided in the radiologist group (RAD) and non-radiologist group (nRAD). The RAD group included non-chest RAD and chest-RAD. A total of 99 patients, 52 with COVID-19 and 47 with SSc-ILD, were included in the study.Results:Fibrosis inside focal ground glass opacities (GGO) in the upper lobes; fibrosis in the lower lobe GGO; reticulations in lower lobes (especially if bilateral and symmetrical or associated with signs of fibrosis) were the CT parameters most frequently associated with SSc-ILD. The CT parameters most frequently associated with COVID- 19 pneumonia were: consolidation (CONS) in the lower lobes, CONS with peripheral (both central/peripheral or patchy distributions), anterior and posterior CONS and rounded-shaped GGOs in the lower lobes. After multivariate analysis, the presence of CONS in the lower lobes (p <0.0001) and signs of fibrosis in GGO in the lower lobes (p <0.0001) remained independently associated with COVID-19 pneumonia or SSc-ILD, respectively. These two variables were combined in a predictive score which resulted positively associated with the COVID-19 diagnosis, with 96.1% sensitivity and 83.3% specificity: 3 different risk class for COVID-19 pneumonia may be identified: high risk for COVID-19 pneumonia (5-9 points); probable overlap COVID-19 pneumonia in SSc-ILD (4 points); low risk for COVID-19 pneumonia (0-3 points).Conclusion:The CT differential diagnosis between COVID-19 Pneumonia and SSc-ILD is possible and may be fostered in practice by the use of a radiological score. In the case where an overlap of both diseases is suspected, the presence of consolidation in the lower lobes may suggest a COVID-19 pneumonia while the presence of fibrosis inside GGO may indicate a SSc-ILD.References:[1]Orlandi M, Landini N, Bruni C, et al. Infection or autoimmunity? The clinical challenge of interstitial lung disease in systemic sclerosis during COVID 19 pandemic. J Rheumatol. 2020 Dec 1: jrheum.200832[2]Simpson S, Kay FU, Abbara S, et al. Radiological Society of North America Expert Consensus Statement on Reporting Chest CT Findings Related to COVID-19. Endorsed by the Society of Thoracic Radiology, the American College of Radiology, and RSNA [published online ahead of print, 2020 Apr 28]. J Thorac Imaging. 2020;10.1097/RTI.0000000000000524.[3]Cheng C, Li C, Zhao T, et al. COVID-19 with rheumatic diseases: a report of 5 cases. Clin Rheumatol. 2020;39(7):2025-2029.[4]Mariano RZ, Rio APTD, Reis F. Covid-19 overlapping with systemic sclerosis. Rev Soc Bras Med Trop. 2020 Sep 21;53:e20200450.Disclosure of Interests:None declared


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 ◽  
...  

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.


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