Familial interstitial lung disease in children: Response to chloroquine treatment in one sibling with desquamative interstitial pneumonitis

Objectives: The burden of interstitial lung disease (ILD) is rising globally. This study aimed to describe and compare characteristics of Connective Tissue Disease-associated ILD with Idiopathic Pulmonary Fibrosis, the two most commonly observed ILDs among outpatients at a tertiary care hospital in Karachi, Pakistan. Methods: A retrospective research study was conducted. Patients with ILD were identified through the outpatient data registry at the Aga Khan University Hospital (AKUH), Karachi from October 2016 to October 2017. We obtained data pertaining to demographics, clinical and radiologic features. A comparative analysis was done to compare the patient characteristics and key features between CTD-ILD and IPF patients. The analysis was done using STATA version 12.0. Results: We identified 184 patients with ILD, which included 52 (29.3%) with CTD-ILD and 62 (35%) with IPF. The most prevalent conditions among CTD-ILD patients included rheumatoid arthritis (42.3%) and scleroderma (25%). Usual interstitial pneumonitis was the common radiologic pattern in RA-ILD (63.6%) and scleroderma (61.5%) while non-specific interstitial pneumonitis was more common in MCTD (85.7%) and SLE (80%). Compared to patients with IPF, those with CTD-ILD were predominantly younger (p<0.001) and female (88.5 % v 45.2%, p<0.001). History of GERD was also significantly lower in CTD-ILD (p=0.05). Conclusion: CTD-ILD patients in our registry were younger and predominantly female compared to IPF. Further studies and ongoing data registry are needed to understand the full spectrum of this disease and long term clinical outcomes. Keywords: Connective tissue disease, Interstitial lung disease, Idiopathic pulmonary fibrosis. Continuous...

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Should we perform surgical lung biopsy when transbronchial cryobiopsy provides unclassifiable interstitial pneumonitis in a patient with diffuse interstitial lung disease?

10.1183/13993003.congress-2020.1577 ◽

2020 ◽

Author(s):

Mehrnaz Asadi Gharabaghi ◽

Esmaeil Idani ◽

Forouzan Mohammadi ◽

Shirin Izadi ◽

Behdad Boroofeh ◽

...

Keyword(s):

Interstitial Lung Disease ◽

Lung Disease ◽

Interstitial Pneumonitis ◽

Lung Biopsy ◽

Surgical Lung Biopsy

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Interaction between the promoter MUC5B polymorphism and mucin expression: is there a difference according to ILD subtype?

Thorax ◽

10.1136/thoraxjnl-2020-214579 ◽

2020 ◽

Vol 75 (10) ◽

pp. 901-903 ◽

Cited By ~ 1

Author(s):

Carmel J Stock ◽

Caterina Conti ◽

Ángeles Montero-Fernandez ◽

Gaetano Caramori ◽

Philip L Molyneaux ◽

...

Keyword(s):

Interstitial Lung Disease ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Lung Disease ◽

Interstitial Pneumonitis ◽

Carrier Status ◽

Immunohistochemical Expression ◽

Disease Patterns ◽

Mucin Expression

The MUC5B promoter variant rs35705950 is associated with idiopathic pulmonary fibrosis (IPF). MUC5B glycoprotein is overexpressed in IPF lungs. We examined immunohistochemical expression of MUC5B in different interstitial lung disease patterns according to rs35705950 T-allele carriage. We observed increased expression of MUC5B in T-allele carriers in both distal airways and honeycomb cysts in patients with IPF (n=23), but no difference in MUC5B expression according to T-carrier status in the distal airways of patients with idiopathic non-specific interstitial pneumonitis (n=17), in scleroderma-associated non-specific interstitial pneumonitis (n=15) or in control lungs (n=20), suggesting that tissue overexpression in MUC5B rs35705950 T-carriers is specific to IPF.

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COVID-19 Associated Interstitial Lung Disease - An Emerging Entity

10.21203/rs.3.rs-116167/v1 ◽

2020 ◽

Author(s):

Ali Bin Sarwar Zubairi ◽

Anjiya Shaikh ◽

Syed Muhammad Zubair ◽

Akbar Shoukat Ali ◽

Safia Awan ◽

...

Keyword(s):

Interstitial Lung Disease ◽

Lung Disease ◽

Interstitial Pneumonitis ◽

Descriptive Analysis ◽

Acute Infection ◽

Fatal Outcome ◽

Case Fatality ◽

Recovery Phase ◽

Hrct Patterns ◽

Usual Interstitial Pneumonitis

Abstract Background: COVID-19 disease associated pulmonary sequalae has been increasingly reported after recovery from acute infection. Therefore, we aim to explore the charactersitics of interstitial lung disease in patients with COVID-19.Methods: An observational study was conducted in patients with COVID-19 associated ILD from April 2020 till September 2020. Patients ≥18 years of age with COVID-19 who were diagnosed with ILD based on respiratory symptoms and HRCT chest imaging after the recovery phase of COVID-19 infection were recruited. Data was recorded on a structured proforma, and descriptive analysis was performed using Stata version 12.1.Results: A total of 30 patients with COVID-associated ILD were identified. The mean age of patients was 59.14 (SD 12.60) and 27 (90%) were males. Four HRCT patterns of interstitial lung disease were seen; organizing pneumonia in 10 (33.33%), non-specific interstitial pneumonitis in 17 (56.67%), usual interstitial pneumonitis in 12 (40%) and probable usual interstitial pneumonitis in 14 (46.67%). Diffuse involvement was found in 15 (50%) patients, while peripheral predominance in 15 (50%) and other significant findings were seen in 8 (26.67%) patients. All patients were treated with corticosteroids. The case fatality rate was 16.67%. Amongst the survivors, 8 (32%) recovered completely, 9 (36%) improved, while 8 (32%) patients had static or progressive disease.Conclusions: This is the first study from Southeast Asia that identified COVID-associated interstitial lung disease in patients who had no pre-existing lung disease, highlighting the importance of timely recognition and treatment of an entity that might lead to fatal outcome.

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