Demographic and clinical predictors of progression and mortality in connective tissue disease-associated interstitial lung disease: a retrospective cohort study

Chan, Chrystal; Ryerson, Christopher J.; Dunne, James V.; Wilcox, Pearce

doi:10.1186/s12890-019-0943-2

Cited by 45 publications

(37 citation statements)

References 41 publications

(59 reference statements)

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“…The commonly reported PFT parameters that are associated with ILD prognosis include FVC and DLco. [ 4 5 6 ] Nathan et al . [ 4 ] reported that the median survival among IPF patients with baseline FVC percent predicted <55% was 27.4 months and among those with DLco percent predicted <35% was 31.3 months compared to 55.6 months for FVC >70% and 67.3 months for DLco >50%.…”

Section: Discussionmentioning

confidence: 99%

“…[ 1 2 3 ] Studies in ILD have identified important physiological prognostic markers associated with reduced survival, such as forced vital capacity (FVC), total lung capacity (TLC), diffusion capacity of the lung for carbon monoxide (DLco), 6-min walk distance (6MWD), and desaturation during the 6-min walk test (6MWT). [ 4 5 6 7 8 ] However, the cited studies did not specify what proportions of these patients have underlying pulmonary hypertension (PH). Estimates suggest that the prevalence of PH among ILD patients ranges between 30% and 74% depending on the ILD subtype and studied population.…”

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confidence: 99%

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Predictors of mortality in interstitial lung disease patients without pulmonary hypertension

Alhamad

Cal

2020

Ann Thorac Med

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BACKGROUND: There is a paucity of information regarding prognostic factors associated with reduced survival in interstitial lung disease (ILD) patients without pulmonary hypertension (PH). AIMS: The aim of this study was to determine physiological and hemodynamic parameters that impact survival among ILD patients without PH based on right heart catheterization (RHC). METHODS: Consecutive ILD patients who underwent RHC ( n = 169) at one center were included. The information analyzed included demographics and physiological and hemodynamic parameters. Cox regression models were used to identify independent predictors of survival. RESULTS: The mean age was 55.0 years, and 49.7% of the patients were females. Thirty-three patients died, and two underwent transplantation. Patients with predicted diffusion capacity of the lung for carbon monoxide <35%, walking distance <300 m, and 6-min walk test (6MWT) final oxygen saturation measured by pulse oximetry (SpO 2 ) <85% were significantly associated with an increased mortality risk ( P = 0.022, P < 0.0001, and P = 0.049, respectively; all by log-rank analysis). Advanced age, idiopathic pulmonary fibrosis diagnosis, reduced forced vital capacity, and low cardiac index were independent predictors of increased mortality in the ILD cohort. CONCLUSIONS: Our study demonstrates that parameters obtained from baseline pulmonary function tests and 6MWTs are important determinants of survival in ILD patients without PH. Importantly, cardiac index was the only hemodynamic variable independently associated with survival. Thus, in the absence of PH, when ILD patients perform poorly during the 6MWT manifested as reduced walking distance and desaturation at the end of the test, cardiovascular impairment must be ruled out.

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Section: Discussionmentioning

confidence: 99%

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confidence: 99%

Predictors of mortality in interstitial lung disease patients without pulmonary hypertension

Alhamad

Cal

2020

Ann Thorac Med

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“…These differences may be attributable to the different genetic and ethnic backgrounds of the study subjects and also to the heterogeneity of the non-IPF patients in the above publications [5,7,11]. There are also some previous studies suggesting that ethnic background might influence the clinical features and disease course of the ILD patients [21,22]. AE-ILD has been reported to be the most common individual cause for a respiratory deterioration in different types of ILDs accounting for 29-55% [5,6,10,11] of respiratory hospitalizations, values consistent with our own findings.…”

Section: Plos Onementioning

confidence: 93%

Causes of acute respiratory hospitalizations predict survival in fibrosing interstitial lung diseases

Salonen¹,

Vähänikkilä²,

Purokivi³

et al. 2020

PLoS ONE

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Acute exacerbation of ILD (AE-ILD) is a common reason for hospitalization; it is also associated with significant mortality. Less is known about the prognostic significance of other events causing acute, non-elective hospitalizations in ILD patients. ILD patients hospitalized due to acute respiratory worsening were collected from medical records. Reasons for respiratory deterioration were classified into AE-ILDs and other causes. Clinical features and survival data of idiopathic pulmonary fibrosis (IPF) and other types of ILDs were evaluated and compared. In all, 237 patients (138 with IPF and 99 with other ILD) fulfilled the inclusion criteria. Of the non-IPF ILD types, the most prevalent subgroups were connective tissue disease-associated ILD (n = 33) and asbestosis (n = 22). The most common cause for hospitalization was AE-ILD explaining 41% of hospitalizations. Lower respiratory tract infection (22%), subacute progression of ILD (12%) and cardiovascular causes (7.2%) were other common reasons for hospital treatment. Patients with a lower respiratory tract infection had a more favorable prognosis compared with patients with AE-ILD. AE-ILDs were less fatal than cardiovascular or concurrent non-ILD-related causes for hospitalizations in non-IPF patients. High Gender-Age-Physiology (GAP) index was a marker for shortened survival and earlier AE-ILDs in all patients. IPF patients had a significantly shorter overall and post-hospitalization survival time compared with other ILDs. Most respiratory hospitalizations in ILD patients were related to causes other than AE-ILD, which highlights the importance of accurate differential diagnosis in order to target the appropriate treatment for each ILD patient.

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“…CT presented in table [2]. The pleural line was thick in 24%, smooth in 46.0% and irregular in 30.0%.…”

Section: Results Of Chest Ultrasound and High Resolutionmentioning

confidence: 95%

“…ILD is not a single disease, but it includes a group of disorders that are characterized by varying combinations of inflammation and fibrosis may be idiopathic or caused by exposure to organic and inorganic substances [i.e pneumonitis and pneumoconiosis], medical conditions [i.e., connective tissue diseases [CTDs], multisystem diseases, drugs, infection and radiation therapy [2].…”

Section: Introductionmentioning

confidence: 99%

Comparison Study between Chest Ultrasonography and High Resolution CT in Diagnosis and Assessment of Interstitial Lung Diseases

Elmansy

Abdel-Aziz

Shaqueer

et al. 2020

International Journal of Medical Arts

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Background: Chest high-resolution computed tomography [HRCT] is considered the "gold" standard for radiological diagnosis of interstitial lung disease [ILD]. However, it is not available in all health care facilities and its high cost is a limitation for its wide use. Ultrasound could provide a suitable alternative. Aim of the work: To detect the role of transthoracic lung ultrasonography [LUS] in diagnosis and assessment of ILD and to correlate it with HRCT as a gold-standard diagnostic modality. Methods: This included fifty patients who were admitted with clinical features suggestive of ILD. All were clinically evaluated [by full history and physical examination], submitted to pulmonary functions, arterial blood gases, lung ultrasound and HRCT. Results: The total sum of B-lines was 72.64 ± 39.45 and the total positive chest areas [> 3 B-lines] 6.47 ± 2.35. The most common ultrasound finding was thick pleural line. Finally, there was positive [proportional], moderate, significant correlation between Warrick's score [HRCT] and B-lines distance [LUS]. However, Warrick score correlated negatively with each of six-minute walk test, partial arterial oxygen tension and forced vital capacity. Conclusions: Lung ultrasound as a diagnostic modality for ILD reveled that, it is a useful tool, as there was good correlation between LUS and HRCT. LUS is cheap, accessible and radiation-free diagnostic tool. It could play a stand alone or a complementary role in the diagnosis and monitoring of ILD.

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Demographic and clinical predictors of progression and mortality in connective tissue disease-associated interstitial lung disease: a retrospective cohort study

Cited by 45 publications

References 41 publications

Predictors of mortality in interstitial lung disease patients without pulmonary hypertension

Predictors of mortality in interstitial lung disease patients without pulmonary hypertension

Causes of acute respiratory hospitalizations predict survival in fibrosing interstitial lung diseases

Comparison Study between Chest Ultrasonography and High Resolution CT in Diagnosis and Assessment of Interstitial Lung Diseases

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