Clusters of comorbidities in idiopathic pulmonary fibrosis

Prior, Thomas Skovhus; Hoyer, Nils; Hilberg, Ole; Shaker, Saher Burhan; Davidsen, Jesper Rømhild; Rasmussen, Finn; Bendstrup, Elisabeth

doi:10.1016/j.rmed.2021.106490

Cited by 19 publications

(23 citation statements)

References 44 publications

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“…As patients with uILD are mainly elderly, comorbidities could have an important impact on outcomes. The present study shows that comorbidities are common in patients with uILD, but the burden and impact of comorbidities were less pronounced in uILD than in other fibrotic ILDs [ 1 – 4 ]. We found a median number of two comorbidities, whereas Prior et al reported a median of six comorbidities in IPF [ 4 ] and Wälscher et al, reported a median number of three in chronic hypersensitivity pneumonitis (cHP) [ 3 ].…”

Section: Discussionmentioning

confidence: 92%

“…The present study shows that comorbidities are common in patients with uILD, but the burden and impact of comorbidities were less pronounced in uILD than in other fibrotic ILDs [ 1 – 4 ]. We found a median number of two comorbidities, whereas Prior et al reported a median of six comorbidities in IPF [ 4 ] and Wälscher et al, reported a median number of three in chronic hypersensitivity pneumonitis (cHP) [ 3 ]. These differences were observed even though our uILD patients were older (median age 70 years) than patients with cHP (mean age 63 years), or the same age as patients with IPF (67.4 to 72.9 years).…”

Section: Discussionmentioning

confidence: 92%

“…These differences were observed even though our uILD patients were older (median age 70 years) than patients with cHP (mean age 63 years), or the same age as patients with IPF (67.4 to 72.9 years). A larger proportion of our patients (34.5%) were never smokers compared to IPF (19.0–26.7%), which may partly explain the difference in the comorbidity burden [ 4 , 6 ]. Krauss et al reported 38.6% never smokers in their uILD cohort, which was similar to our findings in the present study, but lower than in cHP (50.2%) [ 3 , 12 ].…”

Section: Discussionmentioning

confidence: 99%

“…Comorbidities are common in many interstitial lung diseases (ILDs) including idiopathic pulmonary fibrosis (IPF) and hypersensitivity pneumonitis [ 1 – 4 ], but the frequency and prognostic impact in unclassifiable interstitial lung disease (uILD) remains elusive.…”

Section: Introductionmentioning

confidence: 99%

“…The burden of comorbidities and their association with survival has been characterized in patients with IPF, but there is only limited information about the frequency and type of comorbidities and the impact on survival in patients with uILD [ 1 , 2 , 4 , 12 ]. No previous studies have explored combinations of comorbidities and whether they may represent specific phenotypes in uILD.…”

Section: Introductionmentioning

confidence: 99%

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Comorbidities in unclassifiable interstitial lung disease

et al. 2022

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Background Comorbidities are common in interstitial lung diseases (ILD) and have an important association with survival, but the frequency and prognostic impact of comorbidities in unclassifiable interstitial lung disease (uILD) remains elusive. We aimed to describe the prevalence of comorbidities and assess the impact on survival in patients with uILD. Furthermore, we aimed to identify and characterize potential phenotypes based on clusters of comorbidities and examine their association with disease progression and survival. Methods Incident patients diagnosed with uILD were identified at two ILD referral centers in Denmark and Germany from 2003 to 2018. The diagnosis uILD was based on multidisciplinary team meetings. Clinical characteristics and comorbidities were extracted from ILD registries and patient case files. Survival analyses were performed using Cox regression analyses, disease progression was analyzed by linear mixed effects models, and clusters of comorbidities were analyzed using self-organizing maps. Results A total of 249 patients with uILD were identified. The cohort was dominated by males (60%), former (49%) or current (15%) smokers, median age was 70 years, mean FVC was 75.9% predicted, and mean DLCO was 49.9% predicted. One-year survival was 89% and three-year survival was 73%. Eighty-five percent of the patients had ≥ 1 comorbidities, 33% had ≥ 3 comorbidities and 9% had ≥ 5 comorbidities. The only comorbidity associated with excess mortality was dyslipidemia. No association between survival and number of comorbidities or the Charlson comorbidity index was observed. Three clusters with different comorbidities profiles and clinical characteristics were identified. A significant annual decline in FVC and DLCO % predicted was observed in cluster 1 and 2, but not in cluster 3. No difference in mortality was observed between the clusters. Conclusions The comorbidity burden in uILD is lower than reported in other types of ILD and the impact of comorbidities on mortality needs further clarification. Three clusters with distinct comorbidity profiles were identified and could represent specific phenotypes. No difference in mortality was observed between clusters, but slower disease progression was observed in cluster 3. Better understanding of disease behavior and mortality will require further studies of subgroups of uILD with longer observation time.

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