Interstitial Lung Disease in the Connective Tissue Diseases

Antin‐Ozerkis, Danielle; Rubinowitz, Ami N.; Evans, Jane DeRose; Homer, Robert J.; Matthay, Richard A.

doi:10.1016/b978-0-323-48024-6.00013-6

Cited by 5 publications

(5 citation statements)

References 299 publications

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“…Sixty patients were with diffuse cutaneous SSc (42.6%) based on the SSc classification system . The diagnosis of SSc‐ILD was made by the approach of combining respiratory symptoms, PFT and chest HRCT . During the follow‐up of SSc patients without ILD at baseline, the chest HRCT was the mainstay for diagnosis of new‐onset ILD.…”

Section: Methodsmentioning

confidence: 99%

Serum levels of Krebs von den Lungen‐6 as a promising marker for predicting occurrence and deterioration of systemic sclerosis‐associated interstitial lung disease from a Chinese cohort

Cao

et al. 2018

Int J of Rheum Dis

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Aim A prospective and longitudinal study to investigate the correlations between Krebs von den Lungen‐6 (KL‐6) serum levels and systemic sclerosis associated with interstitial lung disease (SSc‐ILD). Method Blood samples of baseline and the time point at 2 years follow‐up intervals were collected for the measurement of serum KL‐6 levels. The baseline clinical, laboratory characteristics, and incidence density of newly diagnosed ILD during the follow up were compared between SSc patients with elevated serum KL‐6 levels (KL‐6 > 500 U/mL) and those with normal KL‐6 levels (KL‐6 ≤ 500 U/mL) at baseline. Further, we explored the association between serum KL‐6 and deterioration of ILD measured by lung function parameters during follow‐up of 2 years. Results Patients with elevated baseline serum KL‐6 had a significant tendency to have disappearance of the finger pad. The incidence density of new‐onset ILD in SSc patients with elevated baseline serum KL‐6 and those with normal baseline serum KL‐6 was 1.33% and 0.51%, respectively. Among the mild lung injury group, the incidence density of ILD deterioration in SSc patients with elevated baseline serum KL‐6 and those with normal serum KL‐6 was 1.2% and 0.74%, respectively. Conclusion Serum KL‐6 level correlates with the clinical manifestations of microvascular injury. Baseline elevated serum KL‐6 may predict deterioration of lung function of SSc‐ILD patients with mild lung injury.

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Section: Methodsmentioning

confidence: 99%

Serum levels of Krebs von den Lungen‐6 as a promising marker for predicting occurrence and deterioration of systemic sclerosis‐associated interstitial lung disease from a Chinese cohort

Cao

et al. 2018

Int J of Rheum Dis

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“…Pleuropulmonary disease is very common in these patients, at times being the first clinical presentation, and accounting for significant morbidity and mortality in this population. 12 Tansey et al . reported histopathologic findings in a cohort of patients with CVDs including rheumatoid arthritis (RA), polymyositis/dermatomyositis (PM/DM) and Sjogren syndrome (SS).…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Histopathology Findings in Patients With STAT3 Gain of Function Syndrome

et al. 2021

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Introduction and Aim Multiorgan autoimmunity and interstitial lung disease (ILD) are reported in patients with STAT3 GOF syndrome. Results We present lung histopathology findings in 3 such children, two of whom underwent wedge biopsies with adequate diagnostic material. Wedge biopsies showed interstitial cellular expansion with linear and nodular aggregates of CD8 positive T lymphocytes, plasma cells, and histiocytes; consistent with lymphocytic interstitial pneumonia pattern (LIP). CD4+ T cells and CD20+ B cells were present but infrequent in the interstitium. FOXP3 cells ranged from 0-5%. Focal interstitial and intraalveolar histiocytes were also seen. Neutrophils and eosinophils were rare/absent. Non-occlusive peribronchial lymphoid aggregates showed equal T and B cells; likely reactive in nature. Pulmonary vessels appeared normal without vasculitis or hypertensive change. There was no interstitial or subepithelial fibrosis or organizing pneumonia. Interlobular septa and visceral pleura were unremarkable. Conclusion Children with multi-system autoimmune disorders with ILD should be investigated for STAT3 GOF syndrome. Lung wedge biopsies are more informative than transbronchial biopsies, if a tissue sampling is indicated. CD8 dominant T cell inflammation seems to be a key driver of ILD. Although interstitial fibrosis was not seen in our small sample, longer follow up is needed to understand the natural history.

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“…Corticosteroids, such as prednisolone, are one of the most commonly used immunosuppressive therapies for ILD ( 123 ). They have broad anti-inflammatory and immunosuppressive effects.…”

Section: Interstitial Lung Disease Managementmentioning

confidence: 99%

Interstitial lung disease: a review of classification, etiology, epidemiology, clinical diagnosis, pharmacological and non-pharmacological treatment

Althobiani,

Russell,

Jacob

et al. 2024

Front. Med.

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Interstitial lung diseases (ILDs) refer to a heterogeneous and complex group of conditions characterized by inflammation, fibrosis, or both, in the interstitium of the lungs. This results in impaired gas exchange, leading to a worsening of respiratory symptoms and a decline in lung function. While the etiology of some ILDs is unclear, most cases can be traced back to factors such as genetic predispositions, environmental exposures (including allergens, toxins, and air pollution), underlying autoimmune diseases, or the use of certain medications. There has been an increase in research and evidence aimed at identifying etiology, understanding epidemiology, improving clinical diagnosis, and developing both pharmacological and non-pharmacological treatments. This review provides a comprehensive overview of the current state of knowledge in the field of interstitial lung diseases.

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Interstitial Lung Disease in the Connective Tissue Diseases

Cited by 5 publications

References 299 publications

Serum levels of Krebs von den Lungen‐6 as a promising marker for predicting occurrence and deterioration of systemic sclerosis‐associated interstitial lung disease from a Chinese cohort

Serum levels of Krebs von den Lungen‐6 as a promising marker for predicting occurrence and deterioration of systemic sclerosis‐associated interstitial lung disease from a Chinese cohort

Pulmonary Histopathology Findings in Patients With STAT3 Gain of Function Syndrome

Interstitial lung disease: a review of classification, etiology, epidemiology, clinical diagnosis, pharmacological and non-pharmacological treatment

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