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Profile of Connective Tissue Disease Associated Interstitial Lung Disease in Rio de Janeiro State University
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A1682 - Profile of Connective Tissue Disease Associated Interstitial Lung Disease in Rio de Janeiro State University
Author Block: B. Rangel1, P. R. Ribeiro1, C. H. Costa1, R. Rufino2; 1Rio de Janeiro State University, Rio de Janeiro, Brazil, 2Rio de Janeiro State University, Rio De Janeiro 22261-020, Brazil.
Introduction:
The interstitial lung diseases (ILD) represents a heterogeneous group of pneumopathies classified according to clinical, radiological and histopathological findings. The damage begins in the interstitial, and may also involve airways and alveoli. ILD have several causes, among them connective tissue disorders (CTD), and may present as a complication or even before the onset of other symptoms. The most common diseases are systemic sclerosis (SS), systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), dermatomyositis (DER) and mixed connective tissue disease (MCTD).
Objective:
To trace the profile ofILD cases associated with CTD, treated in the ILD Service in Piquet Carneiro Clinic of the Rio de Janeiro State University and compare the data obtained with the literature.
Materials and methods:
Descriptive study based on data frompatients attended at theILD Service between November 2016 and November 2017. All cases with diagnosis of any CTD signed by rheumatologist and pulmonologist. All patients had CT scans and pulmonary function tests (PFT).
Results:
In the period, 106 cases of ILD due to CTD were attended, of which 60 were SS (56.6%), 33 MCTD (31.1%), 18 RA (16.9%), 13 SLE (12.3%), 13 Sjogren's syndrome (12.3%),9 DER (8.5%), 5 Indeterminate Connective Tissue Disease(4.7%) and 2 Anti-synthetase Syndrome (1.9%). The mean age was 57,3 years and 84,9% were female. The NSIP was the most common pattern in the chest tomography, with 37.3%; followed by UIP (30.1%), COP (4.8%) and LIP (2.4%). In 22.9% there was no characteristic pattern and 2.5% there were no changes. The NSIP pattern was the most common in SS (48%) and DMTC (60%), while the UIP pattern was
the most common in RA (42%). In the PFT, 72% presented restrictive pattern,19% had PFT within the reference values, and 9% could not perform it. The mean FVC in NSIP pattern was 56,1% and in UIP pattern 63,5%. Lung biopsy was required in only 4 patients.
Conclusion:
Among the CTD associated with ILD, scleroderma was the most frequent. In relation to sex, females were more affected. The predominant pattern in chest CT was NSIP, followed by the UIP. Such data from the study were similar to those in the literature. The restrictive disorder was predominant in PFT. It is worth mentioning that chest CT was enough to determine the disease pattern in most cases, and lung biopsy was performed in a small percentage of the patients.
Author Block: B. Rangel1, P. R. Ribeiro1, C. H. Costa1, R. Rufino2; 1Rio de Janeiro State University, Rio de Janeiro, Brazil, 2Rio de Janeiro State University, Rio De Janeiro 22261-020, Brazil.
Introduction:
The interstitial lung diseases (ILD) represents a heterogeneous group of pneumopathies classified according to clinical, radiological and histopathological findings. The damage begins in the interstitial, and may also involve airways and alveoli. ILD have several causes, among them connective tissue disorders (CTD), and may present as a complication or even before the onset of other symptoms. The most common diseases are systemic sclerosis (SS), systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), dermatomyositis (DER) and mixed connective tissue disease (MCTD).
Objective:
To trace the profile ofILD cases associated with CTD, treated in the ILD Service in Piquet Carneiro Clinic of the Rio de Janeiro State University and compare the data obtained with the literature.
Materials and methods:
Descriptive study based on data frompatients attended at theILD Service between November 2016 and November 2017. All cases with diagnosis of any CTD signed by rheumatologist and pulmonologist. All patients had CT scans and pulmonary function tests (PFT).
Results:
In the period, 106 cases of ILD due to CTD were attended, of which 60 were SS (56.6%), 33 MCTD (31.1%), 18 RA (16.9%), 13 SLE (12.3%), 13 Sjogren's syndrome (12.3%),9 DER (8.5%), 5 Indeterminate Connective Tissue Disease(4.7%) and 2 Anti-synthetase Syndrome (1.9%). The mean age was 57,3 years and 84,9% were female. The NSIP was the most common pattern in the chest tomography, with 37.3%; followed by UIP (30.1%), COP (4.8%) and LIP (2.4%). In 22.9% there was no characteristic pattern and 2.5% there were no changes. The NSIP pattern was the most common in SS (48%) and DMTC (60%), while the UIP pattern was
the most common in RA (42%). In the PFT, 72% presented restrictive pattern,19% had PFT within the reference values, and 9% could not perform it. The mean FVC in NSIP pattern was 56,1% and in UIP pattern 63,5%. Lung biopsy was required in only 4 patients.
Conclusion:
Among the CTD associated with ILD, scleroderma was the most frequent. In relation to sex, females were more affected. The predominant pattern in chest CT was NSIP, followed by the UIP. Such data from the study were similar to those in the literature. The restrictive disorder was predominant in PFT. It is worth mentioning that chest CT was enough to determine the disease pattern in most cases, and lung biopsy was performed in a small percentage of the patients.
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