AB0689 Interstitial lung disease in severe connective tissue diseases (CTD)

2013 ◽  
Vol 71 (Suppl 3) ◽  
pp. 678.3-678
Author(s):  
M.G. Danieli ◽  
R. Moretti ◽  
S. Gambini ◽  
L. Paolini ◽  
A. Gabrielli
2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1598.2-1599
Author(s):  
I. Rusu ◽  
L. Muntean ◽  
M. M. Tamas ◽  
I. Felea ◽  
L. Damian ◽  
...  

Background:Interstitial lung disease (ILD) is a common manifestation of connective tissue diseases (CTDs), and is associated with significant morbidity and mortality. Chest high-resolution computed tomography (HRCT) play an important role in the diagnosis of ILD and may provide prognostic information.Objectives:We aimed to characterize the clinical profile and chest HRCT abnormalities and patterns of patients diagnosed with CTDs and ILD.Methods:In this retrospective, observational study we included 80 consecutive patients with CTDs and ILD referred to a tertiary rheumatology center between 2015 and 2019. From hospital charts we collected clinical data, immunologic profile, chest HRCT findings. HRCT patterns were defined according to new international recommendations.Results:Out of 80 patients, 64 (80%) were women, with a mean age of 55 years old. The most common CTD associated with ILD was systemic sclerosis (38.8%), followed by polymyositis (22.5%) and rheumatoid arthritis (18.8%). The majority of patients had dyspnea on exertion (71.3%), bibasilar inspiratory crackles were present in 56.3% patients and 10% had clubbing fingers. Antinuclear antibodies (ANA) were present in 78.8% patients, and the most frequently detected autoantibodies against extractable nuclear antigen were anti-Scl 70 (28.8%), followed by anti-SSA (anti-Ro, 17.5%), anti-Ro52 (11.3%) and anti-Jo (7.5%). Intravenous cyclophosphamide therapy for 6-12 months was used in 35% of patients, while 5% of patients were treated with mycophenolate mofetil.The most frequent HRCT abnormalities were reticular abnormalities and ground glass opacity. Non-specific interstitial pneumonia (NSIP) was identified in 46.3% CTDs patients. A pattern suggestive of usual interstitial pneumonia (UIP) was present in 32.5% patients, mainly in patients with systemic sclerosis. In 21.3% patients the HRCT showed reticulo-nodular pattern, micronodules and other abnormalities, not diagnostic for UIP or NSIP pattern.Conclusion:Nonspecific interstitial pneumonia (NSIP) is the most common HRCT pattern associated with CTDs. Further prospective longitudinal studies are needed in order to determine the clinical and prognostic significance of various HRCT patterns encountered in CTD-associated ILD and for better patient management.References:[1]Ohno Y, Koyama H, Yoshikaua T, Seki S. State-of-the-Art Imaging of the Lung for Connective Tissue Disease (CTD). Curr Rheumatol Rep. 2015;17(12):69.[2]Walsh SLF, Devaraj A, Enghelmeyer JI, Kishi K, Silva RS, Patel N, et al. Role of imaging in progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150)Disclosure of Interests:None declared


Author(s):  
Gianluca Sambataro ◽  
Domenico Sambataro ◽  
Martina Orlandi ◽  
Sofia Battisti ◽  
Lorenzo Cavagna ◽  
...  

Abstract Objectives Quantitative computed tomography (QCT) is a promising tool for objective assessment of interstitial lung disease (ILD) related to connective tissue diseases (CTD). However, its validity was never investigated. The aim of this study was to assess QCT feasibility, face, and content validity evaluation concerning CTD-ILD. Methods A rheumatologist and a chest radiologist conceived an online survey with nine statements: Two about general issue involving CTD-ILD, one for the face validity, three both for content validity and feasibility. Each statement had to be rated with a score from 0 to 100, respectively, (complete disagreement and agreement). We considered a statement supported by the experts if the median score was ≥75.0. Results A panel of 14 experts (11 from Europe, three from America) with a nine years median experience was enrolled. All the statements about feasibility, face and content validity were supported, except for QCT capability to recognize elementary lesions. Conclusions The panel of experts supported feasibility, face, and content validity of QCT assessment concerning CTD-ILD. This may stimulate a greater use in clinical practice and further studies to confirm its discriminative properties and its construct validity.


Reumatismo ◽  
2021 ◽  
Vol 73 (3) ◽  
Author(s):  
R. El-Beheidy ◽  
A.M. Domouky ◽  
H. Zidan ◽  
Y.A. Amer

This study was aimed to evaluate serum KL-6 levels to determine if this marker can be used for diagnosing and assessing severity of interstitial lung disease (ILD) in children with connective tissue disorders. In total, 40 patients [18 patients with juvenile systemic lupus erythematosus (JSLE), 10 patients with juvenile idiopathic arthritis (JIA), 8 patients with juvenile mixed connective tissue disease (JMCTD), 3 patients with juvenile systemic sclerosis (JSSc), and 1 patient with juvenile dermatomyositis (JDM)] and 20 healthy controls were included in this study. Age, sex, and duration of CTD and ILD (if any) were recorded. Blood samples from all the patients and controls were examined by ELISA. 20 of the 40 patients with CTD (50%) had ILD, 12 were mild and 8 were severe as assessed by spirometry. The median serum KL-6 level was 102.7 U/mL (76.1-180.8) in the CTD with severe ILD group, 72.2 U/mL (58.4- 100.5) in the CTD with mild ILD group, 56.7 U/mL (35.8-68.5) in the CTD without ILD group, and 52.3 U/mL (32.8-62.4) in the control group. KL-6 levels were significantly higher in the CTD with ILD (p<0.05), at a cutoff of 63.4 U/ml identified by ROC curve, serum KL-6 showed a sensitivity of 95.2% and specificity of 89.7%. KL-6 is a valuable biomarker for diagnostic purposes and to detect severity in ILD in childhood CTD.


2019 ◽  
Vol 94 (2) ◽  
pp. 309-325 ◽  
Author(s):  
Isabel Mira-Avendano ◽  
Andy Abril ◽  
Charles D. Burger ◽  
Paul F. Dellaripa ◽  
Aryeh Fischer ◽  
...  

2020 ◽  
Author(s):  
Sang Wan Chung ◽  
So Youn Shin ◽  
Seung Hyeun Lee ◽  
Hye Sook Choi ◽  
Myung Jae Park ◽  
...  

Abstract Background: The aim of this study was to investigate clinicoserologic biomarkers associated with the development, progression, and prognosis of connective tissue diseases associated interstitial lung disease (CTD-ILD). Methods: We conducted a single center, a retrospective study including 70 incident patients diagnosed with CTD-ILD and 70 age-, sex-, and type of CTD-matched patients without ILD. Clinical informations, pulmonary function test, and chest CT findings were reviewed using medical records. To identify serologic biomarkers, serum interferon-r-induced protein 10 (IP-10), interleukin (IL)-6, IL-8, IL-10, and matrix metalloproteinase 7 (MMP-7) in patients with CTD-ILD and CTD without ILD were measured. Results: A total of 140 patients were enrolled. The mean was 63.3 ± 11.2 years, and 102 (72.9%) patients were female. Raynaud`s phenomenon (OR 5.96, 95% CI 2.11–16.86) was proved to be a risk factor for developing ILD in CTD by multivariable logistic regression analysis. To analyze distinctive features according to the onset of ILD, CTD-ILD was stratified into three groups: ILD-preceding, simultaneous, and CTD-preceding. The majority of the ILD-preceding group (75%) had worse baseline pulmonary function requiring treatment (DLCO <65%). Serum levels of MMP-7 were associated with the development of ILD in patients with CTD, and also had a significant correlation with CT extent score in the present study. Conclusion: In this study, Raynaud`s phenomenon, and serum levels of MMP-7 were clinicoserologic biomarkers CTD-ILD. Therefore, clinicoserologic biomarkers associated with ILD should be assessed in patients with CTD to provide proper management.


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