Materiały źródłowe

• #1 Seven Things Everyone Should Know about Interstitial Lung Disease

  https://www.nationaljewish.org/education/health-information/living-with-chronic-lung-disease/seven-things-everyone-should-know-about-interstitial-lung-disease

  Those diagnosed with ILD used to be given three to five years to live. Thats changed, and setting the record straight on that account is one of seven things everyone should know about interstitial lung disease. […] That three- to five-year prognosis is outdated for all but one form of ILD, according to pulmonologist Joshua Solomon, MD, who leads the Center for Interstitial Lung Disease at National Jewish Health. […] A trend toward earlier diagnosis combined with two U.S. Food and Drug Administration (FDA)-approved medications and several other promising drugs now in clinical trials at National Jewish Health and elsewhere are among the reasons that, as Dr. Solomon says, ILD is no longer a death sentence by any stretch. […] If the cause is ILD, the earlier you are diagnosed, the better your prospects will be.

• #2

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7278097/

  A total of 82 patients with ILD who developed ARF were admitted to the ICU during the study period. […] Overall in-hospital mortality was 65.9%, and 90-day and 1-year mortality were 69.5% and 76.8%, respectively. […] High in-hospital and long-term mortality rates were observed in ILD patients with ARF requiring MV. Poor oxygenation during hospitalization could serve as a predictive factor of poor prognosis. […] The in-hospital mortality among the study subjects was 65.8%. Worse oxygenation on days 5 and 7 independently predicted in-hospital mortality. […] In conclusion, in patients with ILD developing ARF requiring MV, the in-hospital mortality rate was high and long-term outcome was poor. Of note, poor PF ratio on day 3 and day 5 after the onset of ARF are risk factors for in-hospital mortality.

• #3 Acute Respiratory Failure in Critically Ill Patients with Interstitial Lung Disease | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0104897

  Hospital and 1-year mortality rates were 41% and 54% respectively. […] Mortality is high in ILD-associated ARF. CT and echocardiography are valuable prognostic tools. […] Three factors were independently associated with hospital mortality by multivariable analysis: pre-capillary pulmonary hypertension (OR, 4.55; 95%CI (1.2017.33)), traction bronchiectasies and/or honeycombing on CT scan (OR, 7.68; 95%CI (1.7833.22)), and acute kidney injury (OR, 10.60; 95%CI (2.2549.97)). […] Multivariate analysis of variables associated with 1-year mortality showed that respiratory SOFA score (OR, 2.20; 95%CI (1.014.76)), performance status 2 (OR, 4.80; 95%CI (1.1020.91)), traction bronchiectasies and/or honey combing (OR, 6.30; 95%CI (1.5026.52)) and mechanical ventilation (OR, 5.18; 95%CI (1.1822.75)) were associated with poorer survival.

• #4 Predictors of long-term prognosis in rheumatoid arthritis-related interstitial lung disease | Scientific Reports

  https://www.nature.com/articles/s41598-022-13474-w

  The aim of the study was to identify specific clinical and serum protein biomarkers that are associated with longitudinal outcome of RA-associated interstitial lung disease (RA-ILD). […] Progression versus non-progression was defined based on changes in Quantitative Modified HRCT scores and PFTs over time. […] At Year 5, 32% of patients demonstrated progressive RA-ILD, 35% were stable, and 33% improved. […] Baseline age and rheumatoid factor (RF) were significantly different between RA-ILD outcomes of progression vs. no-progression (p<0.05). [...] Changes in levels of CXCL11/I-TAC and MMP13 over 5 years also distinguished pulmonary outcomes (p<0.05). [...] A final binary logistic regression model revealed that baseline age and changes in serum MMP13 as well as CXCL11/I-TAC were associated with RA-ILD progression at Year 5 (p<0.01), with an AUC of 0.7772.

• #5 Predictors of long-term prognosis in rheumatoid arthritis-related interstitial lung disease | Scientific Reports

  https://www.nature.com/articles/s41598-022-13474-w

  Collectively, these analyses demonstrated that baseline clinical variables (age, RF) and shifts in levels of selected serum proteins (CXCL11/I-TAC, MMP13) were strongly linked to RA-ILD outcome over time. […] Given the previously discussed morbidity/mortality of RA-ILD, it is necessary to find methods to not only make an early diagnosis, but also to reliably predict disease progression particularly in individuals with subclinical and/or radiographically mild disease. […] Identifying baseline characteristics and longitudinal markers of disease progression would improve prognostication and help guide management of these patients. […] Our final binary logistic regression model revealed that baseline age and changes in serum levels of MMP13 as well as CXCL11 were associated with RA-ILD progression (Yes vs. No) at Year 5.

• #6 Predictors of long-term prognosis in rheumatoid arthritis-related interstitial lung disease | Scientific Reports

  https://www.nature.com/articles/s41598-022-13474-w

  Of note, radiographic disease progression was accompanied by increased levels of CXCL11/I-TAC and MMP13 over this time period, indicating that these serum proteins could serve as prognostic biomarkers in our longitudinal cohort. […] Our present study indicated that older age was a clinical risk factor associated with worse long-term RA-ILD outcomes, correlating positively with the risk of progressive RA-ILD. […] In addition to age, baseline RF titers were also associated with RA-ILD outcome in our study though we did not observe similar associations with baseline levels of anti-CCP antibodies. […] Our study has several limitations. […] However, because one of the two patients with more severe ILD (ILA=3) at baseline died prior to the 5 year endpoint of this study (and was therefore excluded from analysis of changes in serum protein biomarkers), our final logistic regression model does, in fact, represent risk factors for disease development/progression in RA patients with absent, indeterminate, or early/subclinical ILD. […] While baseline age and RF predicted RA-ILD outcomes of progression versus no-progression, shifts in the level of CXCL11 and MMP-13 over 5 years were also correlated with long-term prognosis of RA-ILD.

• #7 Acute Respiratory Failure in Critically Ill Patients with Interstitial Lung Disease | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0104897

  After checking the proportional hazards assumption, the multivariable Cox regression identified ILD-related pulmonary hypertension and CT fibrosis as factors associated with a higher 12-month mortality: hazards ratios (HR): 3.21 (95%CI, 1.457.11; p=0.004) and 2.36 (95%CI, 1.055.32; p=0.038) respectively.

• #8

  https://discovery.ucl.ac.uk/10054265/

  On evaluation of longitudinal variables, increasing CALIPER fibrosis extent was the strongest outcome predictor, and remained so following adjustment for baseline disease severity, and when FVC declines were marginal. […] In uILD patients, CPI, traction bronchiectasis severity and PA diameter independently predicted outcome at baseline. Increasing fibrosis extent measured by CALIPER was the most powerful index of outcome regardless of baseline disease severity and strongly predicted outcome in patients with marginal FVC declines.

• #9 Evaluation of computer-based computer tomography stratification against outcome models in connective tissue disease-related interstitial lung disease: a patient outcome study | BMC Medicine | Full Text

  https://bmcmedicine.biomedcentral.com/articles/10.1186/s12916-016-0739-7

  CALIPER-derived pulmonary vessel volume is an independent predictor of mortality across all CTD-ILD patients. […] Furthermore, automated stratification of CALIPER CT variables represents a novel method of prognostication at least as robust as PFTs in CTD-ILD patients. […] The predictive power of the Stratified CT model, the ILD-GAP model and the Stratified CT-GAP model to determine mortality in the same 179 patients was evaluated using univariate and multivariate Cox mortality analyses with bootstrapping of 1000 randomly generated samples as well as receiver operator characteristic (ROC) curve analysis. […] The improved strength of the Stratified CT-GAP model over the ILD-GAP model identified in the current study is largely a consequence of a confounding effect of the normal range of pulmonary function when PFTs are stratified as thresholds. […] In conclusion, we have demonstrated that, in a large mixed population of CTD-ILD patients, CALIPER pulmonary vessel volume was the CT variable that best predicted mortality and may be a new prognostic index.

• #10

  https://discovery.ucl.ac.uk/10054265/

  Unclassifiable-interstitial lung disease (uILD) represents a heterogeneous collection of pathologies encompassing those fibrosing lung diseases which do not fulfill current diagnostic criteria. […] We evaluated baseline and longitudinal functional and CT (visual and quantitative computer [CALIPER] analysis) variables to identify outcome predictors in uILD. […] On univariate analysis, CPI was the most powerful functional predictor of mortality (p 0.0001). Visual traction bronchiectasis (p 0.0001), PA diameter (p 0.0001) and honeycombing presence (p = 0.0001) and CALIPER PVV (p = 0.0003) were the strongest CT outcome predictors. […] On multivariate analysis of baseline indices, traction bronchiectasis (p = 0.003), PA diameter (p = 0.003) and CPI (p = 0.0001) independently predicted mortality.

• #11 ILD-GAP Combined with the Charlson Comorbidity Index Score (ILD-GAPC) as a Prognostic Prediction Model in Patients with Interstitial Lung Disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9931459/

  The ILD-GAP scoring system has been widely used to predict the prognosis of patients with interstitial lung disease (ILD). […] The ILD-GAPC provided a greater area under the receiver operating characteristic curve (0.758) for predicting 3-year ILD-related events than the ILD-GAP (0.721). […] The ILD-GAPC model could provide more accurate information for predicting prognosis in patients with ILD than the ILD-GAP model. […] The present study retrospectively investigated the accuracy of predicting ILD prognosis using the ILD-GAP scoring system combined with the Charlson Comorbidity Index score (CCIS) (ILD-GAPC). […] The ILD-GAPC model was found to better predict 3-year ILD-related events and 3-year all-cause mortality than the ILD-GAP model. […] The CCIS, as a summed score of 19 comorbidities weighted according to severity, was developed to assess the risk of death from comorbidities and has been widely used as a prognostic indicator for patients with various diseases.

• #12 ILD-GAP Combined with the Charlson Comorbidity Index Score (ILD-GAPC) as a Prognostic Prediction Model in Patients with Interstitial Lung Disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9931459/

  The ILD-GAP model has been reported to accurately predict mortality in major chronic ILD subtypes such as IPF, iNSIP, CVD-IP, and CHP. […] The ILD-GAPC model was a better predictor of 3-year ILD-related events than the ILD-GAP model. […] A high CCIS not only indicates an increased risk of death from comorbidities but may also indicate a poorer prognosis for ILD itself.

• #13 ILD-GAP combined with the monocyte ratio could be a better prognostic prediction model than ILD-GAP in patients with interstitial lung diseases | BMC Pulmonary Medicine | Full Text

  https://bmcpulmmed.biomedcentral.com/articles/10.1186/s12890-023-02833-6

  The ILD-GAP scoring system is known to be useful in predicting prognosis in patients with interstitial lung disease (ILD). […] We examined whether the ILD-GAP scoring system combined with the monocyte ratio (ILD-GAPM) is superior to the conventional ILD-GAP model in predicting ILD prognosis. […] The ILD-GAPM model may be a more accurate predictor of prognosis for ILD patients than the ILD-GAP model. […] The combination of ILD-GAP and the blood monocyte ratio is estimated to be a highly versatile prognostic model in clinical practice, and in the present study the accuracy for predicting ILD prognosis of the ILD-GAPM model was investigated. […] The blood monocyte ratio could be an important predictor of 3-year ILD-related events among the patients with ILD (especially IPF). Also, the clinical usefulness of the ILD-GAPM model seems to require verification for each ILD subtype, however since the blood monocyte ratio is a very easily measurable biomarker, it is expected that the ILD-GAPM model will be widely accepted as the prognosis prediction tool especially in early-stage ILD patients in the future.

• #14 Interstitial lung disease diagnosis and prognosis using an AI system integrating longitudinal data | Nature Communications

  https://www.nature.com/articles/s41467-023-37720-5

  For accurate diagnosis of interstitial lung disease (ILD), a consensus of radiologic, pathological, and clinical findings is vital. […] Management of ILD also requires thorough follow-up with computed tomography (CT) studies and lung function tests to assess disease progression, severity, and response to treatment. […] Dynamic models to predict patient survival rates based on longitudinal data are challenging to create due to disease complexity, variation, and irregular visit intervals. […] When clinical history and associated CT scans are available, the proposed deep learning system can help clinicians diagnose and classify ILD patients and, importantly, dynamically predict disease progression and prognosis. […] Serial CT that can reveal changes in the extent of parenchymal architectural distortion, reticulation, bronchiectasis and honeycombing, allowing for the identification of progressive fibrotic disease which correlates with poorer survival.

• #15 Interstitial lung disease diagnosis and prognosis using an AI system integrating longitudinal data | Nature Communications

  https://www.nature.com/articles/s41467-023-37720-5

  Mortality is often not feasible as an end-point for diseases with chronic progressive fibrosis (such as IPF); change or lack of change in disease extent on HRCT represents a potential means of assessing treatment response. […] The purpose of our study is to develop an AI system that (1) can classify 5 different types of ILD based on initial chest CT scans and relevant clinical history as well as (2) monitor a patients disease progression. […] The joint CNN model showed superior performance in the classification of ILD subtypes. […] Our joint CNN model also performed as well as all human readers in sensitivity when diagnosing CHP, sarcoidosis, NSIP, and other ILD. […] Our Transformer model can evaluate the 3-year survival rate at each visit by integrating information from each visit.

• #16 Interstitial lung disease diagnosis and prognosis using an AI system integrating longitudinal data | Nature Communications

  https://www.nature.com/articles/s41467-023-37720-5

  The confidence of 3-year survival prediction via Transformer was increased with more follow-up information. […] The estimated mortality rate substantially increased from 2.1% then 6.4% then 9.4%. […] Our proposed deep learning system demonstrates high potential in accurately diagnosing five subtypes of ILD. […] We believe the proposed models, which integrate CT images with clinical history, demonstrate equivalent performance to a senior thoracic radiologist and a senior pulmonologist and also evaluate survival rate at each follow-up visit, which could be a useful tool to distinguish ILD subcategories and manage the long-term progression of patients.

• #17 Seven Things Everyone Should Know about Interstitial Lung Disease

  https://www.nationaljewish.org/education/health-information/living-with-chronic-lung-disease/seven-things-everyone-should-know-about-interstitial-lung-disease

  These two drugs could potentially be home runs in terms of slowing down scarring, said Dr. Solomon. […] You will live a better life after transplant. […] Treatments for interstitial lung diseases are very complex, Dr. Solomon says. […] If we think you have untreated reflux, were super aggressive in treating it, because we think itll slow down the progression of your disease if we get control of it, explained Dr. Solomon. […] We know that, if you participate in pulmonary rehab, or you just stay in shape, your quality of life is better, and your breathlessness is better, Dr. Solomon says. […] ILD is a rare disease, but you are not alone.

• #18 Interstitial Lung Disease: Stages, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17809-interstitial-lung-disease

  Since there are many kinds of interstitial lung diseases, any one case could be mild or very serious. Most kinds of ILD cause irreversible lung damage. The most serious ILDs are progressive, where your condition continues to worsen over time. […] The life expectancy for interstitial lung disease depends on the cause and severity. Some people with mild ILD can live normal lives without treatment or with minimal treatment. Your outlook (prognosis) is better if your disease isn’t getting worse (it’s stable). […] For people with the most severe and rapidly worsening forms of interstitial lung disease, life expectancy is around 3-5 years after diagnosis.

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