Multidisciplinary Meetings in Interstitial Lung Disease: Polishing the Gold Standard

Background:Dermatomyositis is a multi organ autoimmune disease wich is commonly complicated with interstitial lung disease. Chest high-resolution computed tomography (HRCT) is still considered the diagnostic gold standard for interstitial lung disease and is quantification, however he increasing use of lung ultrasound may play an important role in the future.Objectives:The aim of our study was to determine the diagnostic value of lung ultrasound in the detection and progression of interstitial pulmonary disease in patients with dermatomyositis.Methods:Twenty two subjects with dermatomyositis diagnosed according to the American College of Rheumatology criteria were enrolled (6 males, 16 females; mean age: 15,8 ± 8.8 years; range: 6 to 29 years). All subjects underwent high resolution computed tomography followed by transthoracic ultrasound for comet tail sign detection and pleural irregularity in order to predict the degree of interstitial lung disease. The modified transthoracic ultrasound assessment was performed at 06 thoracic regions each side. The Warrick score was calculated according standard high-resolution chest computed tomography images that were evaluated independently from each other by two thoracics radiologists.Results:A significantly positive correlation between transthoracic ultrasound and the severity of pulmonary involvement, as seen by the number of B lines (Spearman ‘s correlation coefficient = 0.80, p < 0.001), and the number of positive areas (regions wih more than 3 B lines) (Spearman ‘s correlation coefficient = 0.75, p < 0.001) were found. When compared with high-resolution chest computed tomography as the gold standard method, the sensitivity, specificity, of transthoracic ultrasound was 96.4%, 83,3% respectively. Addicionally the number of B lines (sum of 35 or more B lines) and a number of B lines positive areas (7 or more regions with at least 3 B lines) cut of allowed to discriminate the inflamatory pattern (ground glass) to those with structural pattern (honeycomb and pulmonary cysts).Conclusion:Our study showed that transthoracic ultrasound comet tails scoring system could be useful in the assessment of the pulmonary involvement in patients with dermatomyositis, and should be considered as a primary screening exam to exclude lung involvement, rather than routine chest CT scans in assimptomatic patients.Disclosure of Interests:None declared

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Patients with Interstitial Lung Disease Secondary to Autoimmune Diseases: How to Recognize Them?

Diagnostics ◽

10.3390/diagnostics10040208 ◽

2020 ◽

Vol 10 (4) ◽

pp. 208 ◽

Cited By ~ 4

Author(s):

Domenico Sambataro ◽

Gianluca Sambataro ◽

Francesca Pignataro ◽

Giovanni Zanframundo ◽

Veronica Codullo ◽

...

Keyword(s):

Interstitial Lung Disease ◽

Lung Disease ◽

Gold Standard ◽

Clinical Laboratory ◽

Diagnostic Assessment ◽

Multidisciplinary Teams ◽

First Line ◽

Secondary Objective ◽

Rheumatic Conditions ◽

Severity Of The Disease

The diagnostic assessment of patients with Interstitial Lung Disease (ILD) can be challenging due to the large number of possible causes. Moreover, the diagnostic approach can be limited by the severity of the disease, which may not allow invasive exams. To overcome this issue, the referral centers for ILD organized Multidisciplinary Teams (MDTs), including physicians and experts in complementary discipline, to discuss the management of doubtful cases of ILD. MDT is currently considered the gold standard for ILD diagnosis, but it is not often simple to organize and, furthermore, rheumatologists are still not always included. In fact, even if rheumatologic conditions represent a common cause of ILD, they are sometimes difficult to recognize, considering the variegated clinical features and their association with all possible radiographic patterns of ILD. The first objective of this review is to describe the clinical, laboratory, and instrumental tests that can drive a diagnosis toward a possible rheumatic disease. The secondary objective is to propose a set of first-line tests to perform in all patients in order to recognize any possible rheumatic conditions underlying ILD.

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Lymphangioleiomyomatosis

Journal of Clinical Images and Medical Case Reports ◽

10.52768/2766-7820/1378 ◽

2021 ◽

Vol 2 (5) ◽

Author(s):

Klein Dantis ◽

◽

Ranganath TG ◽

Keyword(s):

Interstitial Lung Disease ◽

Lung Disease ◽

Gold Standard ◽

Pulmonary Rehabilitation ◽

Histopathological Diagnosis ◽

Neoplastic Disease ◽

European Respiratory Society ◽

Free Survival ◽

Bronchodilator Therapy ◽

History Of

Lymphangioleiomyomatosis (LAM) is a rare systematic neoplastic disease exclusively seen in middle-aged women with an incidence of 5-9 per million. They can occur sporadically or in association with tuberous sclerosis. Histopathological diagnosis is the gold standard. Median transplant-free survival from the time of diagnosis is 23 years. We here by present premenstrual female with history of recurrent dyspnea with differential diagnosis for various interstitial lung disease diagnosed to have LAM. She was managed with bronchodilator therapy and pulmonary rehabilitation as per European respiratory society guidelines. Keywords: lymphangioleiomyomatosis; interstitial lung disease; uniportal VATS; histopathology

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OP0063 QUANTITATIVE COMPUTED TOMOGRAPHY PREDICTS 10-YEAR MORTALITY IN INTERSTITIAL LUNG DISEASE RELATED TO SYSTEMIC SCLEROSIS

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.2239 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 42.1-42

Author(s):

A. Ariani ◽

E. Bravi ◽

M. De Santis ◽

V. Hax ◽

S. Parisi ◽

...

Keyword(s):

Systemic Sclerosis ◽

Interstitial Lung Disease ◽

Lung Disease ◽

Mortality Risk ◽

Gold Standard ◽

Prediction Models ◽

Quantitative Computed Tomography ◽

Chest Ct ◽

Qualitative Assessment ◽

Increased Risk

Background:Interstitial lung disease (ILD) is the main cause of death in Systemic Sclerosis (SSc). Chest CT is the gold standard in detecting ILD although it is not easy to understand its prognostic value. ILD qualitative assessment is almost worthless. Goh et al. semi quantitative score of ILD extent is related to mortality risk but it is burdened by relevant inter/intra-readers variability. An operator independent algorithm based on voxel-wise analysis proved to identify SSc patients with an increased risk of mortality according to prediction models.Objectives:To verify if quantitative analysis of chest CT (QCT) predict 10 years-mortality in SSc patients.Methods:SSc patients with availability of a chest CT were enrolled in 13 different centers. The CT voxel-wise analysis with a free software (www.horosproject.com) provided QCT indexes: kurtosis, skewness, mean lung attenuation and standard deviation. Patients characteristics, autoimmune profile and pulmonary function test were collected. The follow-up interval lasted from the date of chest CT to the one of the last visit or death. Each QCT index cutoff, established in a previous study (1), clustered patients in two groups. Kaplan-Meier analysis estimated and compared survival in the above mentioned groups. p < 0.05 was considered statistically significant.Results:Five hundred sixty three SSc patients were enrolled (35938 patient-months); 52.4% had ILD detectable at CT scan. For each QCT index cutoff the cohort was split in two subgroups without differences in terms of sex, age, disease duration, autoimmune profile. All QCT indexes’ cutoff selected subgroups with statistically different survival rate (e.g in Figure 1).Figure 1Conclusion:QCT can arise as the new gold standard in identifying SSc patients with poor prognosis. The real possibility to stratify SSc subjects according mortality risk will have a pivotal role in ILD treatment decisional process with the incoming anti-fibrotic drugs.References:[1]Ariani A et al. Rheumatology 2017Disclosure of Interests:Alarico Ariani: None declared, Elena Bravi: None declared, Maria De Santis: None declared, Vanessa Hax: None declared, Simone Parisi: None declared, Federica Lumetti: None declared, Francesco Girelli: None declared, Marta Saracco: None declared, Fabio De Gennaro: None declared, Alessandro Giollo: None declared, Masen Abdel Jaber: None declared, Francesco Bozzao: None declared, Mario Silva: None declared, Maria Chiara Ditto: None declared, Claudia Lomater: None declared, Flavio Mozzani: None declared, Daniele Santilli: None declared, eleonora Di Donato: None declared, Andrea Becciolini Speakers bureau: Sanofi-Genzyme, UCB and AbbVie, Francesco Pucciarini: None declared, Lorenzo Canziani: None declared, Flavio Cesare Bodini: None declared, eugenio arrigoni: None declared, M Bredemeier: None declared, Rafael Mendonça da Silva Chakr: None declared, Amelia Spinella: None declared, Luca Idolazzi: None declared, Roberto Bortolotti: None declared, Paola Tomietto: None declared, Elisa Baratella: None declared, Saverio Tollot: None declared, Dilia Giuggioli: None declared, Fabio Fischetti: None declared, Enrico Fusaro: None declared, Nicola Sverzellati: None declared, Carlo Alberto Scirè: None declared

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