Complex restrictive pulmonary function pattern in connective tissue disorders (CTD) with interstitial lung disease (ILD)

Background: The aim of this paper was to evaluate the thoracic manifestations associated with the Connective tissue disorders, with an emphasis on interstitial and airway disease pattern on the High Resolution computed tomography (HRCT) findings. Subjects and Methods: The present study was conducted for a period of one year. A total of 50 patients with various connective tissue disorders having respiratory complaints were evaluated. Results: During the study period 50 patients (80%females and 20%males) underwent evaluation. Cough and dyspnea were the most common presenting symptoms. Variety of thoracic abnormalities weredetected in 67 (95%) cases. Most common abnormality detected on HRCT was interstitial fibrosis/interstitial lung disease present in (60%) cases. Most common parenchymal abnormalities seen were reticulations (61.4%), ground glass opacification (40%), mosaic attenuation (32.8%) and honeycombing (24.3%). Airway abnormalities seen were bronchiectasis (48.5%), emphysema (12.8%), and ground glass nodules (2.8%). Conclusion: Interstitial lung disease is the most common pulmonary manifestation among patients with connective tissue disorders, and early detection and prompt treatment is expected to improve the outcome.

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Serum KL-6 as predictive and prognostic marker of interstitial lung disease in childhood connective tissue diseases: a pilot study

Reumatismo ◽

10.4081/reumatismo.2021.1399 ◽

2021 ◽

Vol 73 (3) ◽

Author(s):

R. El-Beheidy ◽

A.M. Domouky ◽

H. Zidan ◽

Y.A. Amer

Keyword(s):

Interstitial Lung Disease ◽

Connective Tissue ◽

Lung Disease ◽

Lupus Erythematosus ◽

Connective Tissue Diseases ◽

Control Group ◽

Connective Tissue Disorders ◽

Juvenile Systemic Lupus Erythematosus ◽

Median Serum ◽

Juvenile Systemic Sclerosis

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.

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Interstitial Lung Disease-Associated Pulmonary Hypertension in the Connective Tissue Disorders

Pulmonary Hypertension and Interstitial Lung Disease ◽

10.1007/978-3-319-49918-5_9 ◽

2017 ◽

pp. 165-180

Author(s):

Debabrata Bandyopadhyay ◽

Tanmay S. Panchabhai ◽

Kristin B. Highland

Keyword(s):

Pulmonary Hypertension ◽

Interstitial Lung Disease ◽

Connective Tissue ◽

Lung Disease ◽

Connective Tissue Disorders

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AB0698 MID-Term Effects of Rituximab in Connective Tissue Disorders Related Interstitial Lung Disease (ILD)

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2015-eular.6310 ◽

2015 ◽

Vol 74 (Suppl 2) ◽

pp. 1131.2-1131 ◽

Cited By ~ 1

Author(s):

G. Lepri ◽

J. Avouac ◽

P. Airò ◽

F. Anguita Santos ◽

S. Bellando Randone ◽

...

Keyword(s):

Interstitial Lung Disease ◽

Connective Tissue ◽

Lung Disease ◽

Connective Tissue Disorders

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Raynaud`s Phenomenon and MMP-7 are Clinicoserologic Biomarkers in Patients with Connective Tissue Disease Associated Interstitial Lung Disease.

10.21203/rs.3.rs-20929/v1 ◽

2020 ◽

Author(s):

Sang Wan Chung ◽

So Youn Shin ◽

Seung Hyeun Lee ◽

Hye Sook Choi ◽

Myung Jae Park ◽

...

Keyword(s):

Interstitial Lung Disease ◽

Connective Tissue ◽

Pulmonary Function ◽

Lung Disease ◽

Connective Tissue Diseases ◽

Serum Levels ◽

Multivariable Logistic Regression Analysis ◽

Distinctive Features ◽

Extent Score ◽

Matrix Metalloproteinase 7

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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A study of respiratory system involvement in autoimmune connective tissue diseases: a marker of morbidity

International Journal of Research in Dermatology ◽

10.18203/issn.2455-4529.intjresdermatol20202660 ◽

2020 ◽

Vol 6 (4) ◽

pp. 532

Author(s):

Neha T. Solanki ◽

Sahana P. Raju ◽

Deepmala Budhrani ◽

Bharti K. Patel

Keyword(s):

Systemic Sclerosis ◽

Interstitial Lung Disease ◽

Connective Tissue ◽

Pulmonary Function ◽

Lung Disease ◽

Pulmonary Function Tests ◽

Tertiary Care ◽

Connective Tissue Diseases ◽

Cutaneous Manifestations ◽

Respiratory Involvement

Background: The auto-immune connective tissue diseases (AICTD) are polygenic clinical disorders having heterogeneous overlapping clinical features. Certain features like autoimmunity, vascular abnormalities, arthritis/arthralgia and cutaneous manifestations are common to them. Lung involvement can present in AICTDs in form of: pleurisy, acute/ chronic pneumonitis, pulmonary artery hypertension (PAH), shrinking lung syndrome, diffuse alveolar damage, pulmonary embolism (PE), bronchiolitis obliterans organizing pneumonia, pulmonary infections, cardiogenic pulmonary edema, etc. High-resolution computed tomography (HRCT) plays an important role in identifying patients with respiratory involvement. Pulmonary function tests are a sensitive tool detecting interstitial lung disease.Methods: The present study is an observational study carried out on 170 patients of AICTD in department of Dermatology, Venereology and Leprosy at a tertiary care centre during a period of 2 years from October 2017 to August 2019. Detailed history, examination and relevant investigations like chest X-ray, pulmonary function test (PFT), HRCT thorax were done as indicated.Results: The overall incidence of respiratory involvement was 56.7% with maximum involvement in systemic sclerosis cases (82.8% of cases). 45.7% of patients of systemic lupus erythematosus had respiratory involvement, most common being pleural effusion in 11.5%. Impaired PFT’s were seen in 82.8% cases of systemic sclerosis (SSc) and all cases of UCTD. Interstitial lung disease was seen in 34.7% and 25% cases of SSc and DM respectively. PAH was found in 15.2% cases of SSc and 9.8% cases of mixed connective tissue diseases.Conclusions: AICTD are multisystem disorders in which pulmonary involvement can be an important cause of morbidity to the patient and early detection is necessary for prevention of long-term respiratory complications.

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