Early Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis: A Narrative Review

Alsomali, H; Palmer, Evelyn; Aujayeb, A; Funston, Wendy

doi:10.1007/s41030-023-00216-0

Cited by 9 publications

(11 citation statements)

References 95 publications

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“…Three expert pathologists have shown a clear correlation between the presence of focal UIP- like fibrosis and a less favorable outcome. The focal UIP pattern as defined in this paper is particularly significant, as its rapid recognition can facilitate the prompt initiation of treatment and improve patient outcomes [29,30].…”

Section: Discussionmentioning

confidence: 99%

Focal Usual Interstitial Pneumonia-like Fibrosis is a Core Prognostic Factor in Progressive Pulmonary Fibrosis

Tsushima,

Okoshi,

Ishijima

et al. 2023

Preprint

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Progressive pulmonary fibrosis (PPF) is a newly recognized clinical phenotype of interstitial lung diseases in the 2022 interstitial pulmonary fibrosis (IPF) guidelines. This category is based entirely on clinical and radiological factors, and the background histopathology is unknown. Our objective was to investigate the histopathological characteristics of PPF and to examine the correlation between usual interstitial pneumonia (UIP) and prognosis in this new disease type. We hypothesized that the presence of UIP like fibrosis predicts patients’ survival in PPF cases.We selected 201 cases fulfilling the clinical criteria of PPF from case archives. Cases diagnosed as IPF by a multidisciplinary team were excluded. Whole slide images were evaluated by three pathologists who were blind to clinical and radiological data. We measured areas of UIP-like fibrosis and calculated what percentage of the total lesion area they occupied.The presence of focal UIP-like fibrosis amounting to 10% or more of the lesion area was seen in 148 (73.6%), 168 (83.6%), and 165 (82.1%) cases for each pathologist respectively. The agreement of the recognition of UIP-like fibrosis in PPF cases was above κ = 0.6 between all pairs. Survival analysis showed that the presence of focal UIP-like fibrosis correlated with worsened survival under all parameters tested (p < 0.001).The presence of UIP-like fibrosis is a core pathological feature of clinical PPF and its presence within diseased areas is associated with poorer prognosis. This study highlights the importance of considering the presence of focal UIP like fibrosis in the evaluation and management of PPF.

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Section: Discussionmentioning

confidence: 99%

Focal Usual Interstitial Pneumonia-like Fibrosis is a Core Prognostic Factor in Progressive Pulmonary Fibrosis

Tsushima,

Okoshi,

Ishijima

et al. 2023

Preprint

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“…Gejala sistemik yaitu pasien merasa lemas disertai penurunan gradual kemampuan melakukan aktivitas sehari-hari. 8 Hemoptisis dapat ditemukan bila ada komplikasi seperti infeksi, emboli paru, keganasan, atau vaskulitis pulmoner. 3,9 Gejala dapat muncul dalam durasi yang tidak menentu, bisa selama berbulan-bulan hingga hitungan tahun.…”

Section: Gambar 1 Klasifikasi Interstitial Lung Disease (Ild)unclassified

“…Sensitivitas HRCT berkaitan dengan kriteria histopatologi. 8 5. Uji Faal Paru Perubahan fisiologis paru paling umum pada ILD dengan fibrosis menunjukkan adanya gangguan restriktif paru dengan penurunan kapasitas total paru dan KVP.…”

Section: Diagnosis Pemeriksaan Fisikunclassified

“…Uji Faal Paru Perubahan fisiologis paru paling umum pada ILD dengan fibrosis menunjukkan adanya gangguan restriktif paru dengan penurunan kapasitas total paru dan KVP. 8,12 Pasien IPF biasanya mengalami penurunan KVP, kapasitas paru total, dan kapasitas difusi paru. Gangguan obstruktif lebih sedikit ditemukan, biasanya pada pasien ILD dengan diagnosis sekunder seperti asma atau penyakit paru obstruktif kronis (PPOK).…”

Section: Diagnosis Pemeriksaan Fisikunclassified

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Memahami Penyakit Paru Interstisial: Pengenalan Dini pada Layanan Kesehatan Primer

Latona Sidarta,

Rasmin

2024

Cermin Dunia Kedokteran

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Penyakit paru interstisial atau interstitial lung disease (ILD) mencakup sekelompok gangguan paru heterogen dengan etiologi dan presentasi klinis yang bervariasi. Diagnosis dini ILD sangat penting untuk intervensi tepat waktu dan perbaikan pasien. Minireview ini menyoroti pentingnya diagnosis dini ILD di pusat kesehatan primer, dengan fokus pada manfaat dan tantangan terkait deteksi dini, serta peran profesional kesehatan primer dalam mencapai diagnosis tepat waktu. Profesional kesehatan primer memainkan peran penting dalam mengenali tanda dan gejala awal ILD, melakukan evaluasi awal, dan membimbing pasien melalui proses diagnostik. Upaya kolaboratif antara profesional kesehatan primer, pulmonolog, dan radiolog sangat penting untuk mengatasi tantangan tersebut dan memastikan diagnosis tepat waktu.

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“…Global incidence and prevalence is estimated to be in the range 0.09–1.30 and 0.33–4.51 per 10,000 persons respectively, but there is wide variation in the estimates reported by individual countries [ 4 , 5 ]. While IPF remains a relatively rare disease, there is some evidence to suggest that prevalence is increasing [ 6 ]. However, it is unclear whether this is due to increased recognition, changes in disease nomenclature and classification, or a true increase [ 5 ].…”

Section: Introductionmentioning

confidence: 99%

Validation of the recording of idiopathic pulmonary fibrosis in routinely collected electronic healthcare records in England

et al. 2023

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Background Routinely-collected healthcare data provide a valuable resource for epidemiological research. Validation studies have shown that for most conditions, simple lists of clinical codes can reliably be used for case finding in primary care, however, studies exploring the robustness of this approach are lacking for diseases such as idiopathic pulmonary fibrosis (IPF) which are largely managed in secondary care. Method Using the UK’s Clinical Practice Research Datalink (CPRD) Aurum dataset, which comprises patient-level primary care records linked to national hospital admissions and cause-of-death data, we compared the positive predictive value (PPV) of eight diagnostic algorithms. Algorithms were developed based on the literature and IPF diagnostic guidelines using combinations of clinical codes in primary and secondary care (SNOMED-CT or ICD-10) with/without additional information. The positive predictive value (PPV) was estimated for each algorithm using the death record as the gold standard. Utilization of the reviewed codes across the study period was observed to evaluate any change in coding practices over time. Result A total of 17,559 individuals had a least one record indicative of IPF in one or more of our three linked datasets between 2008 and 2018. The PPV of case-finding algorithms based on clinical codes alone ranged from 64.4% (95%CI:63.3–65.3) for a “broad” codeset to 74.9% (95%CI:72.8–76.9) for a “narrow” codeset comprising highly-specific codes. Adding confirmatory evidence, such as a CT scan, increased the PPV of our narrow code-based algorithm to 79.2% (95%CI:76.4–81.8) but reduced the sensitivity to under 10%. Adding evidence of hospitalisation to the standalone code-based algorithms also improved PPV, (PPV = 78.4 vs. 64.4%; sensitivity = 53.5% vs. 38.1%). IPF coding practices changed over time, with the increased use of specific IPF codes. Conclusion High diagnostic validity was achieved by using a restricted set of IPF codes. While adding confirmatory evidence increased diagnostic accuracy, the benefits of this approach need to be weighed against the inevitable loss of sample size and convenience. We would recommend use of an algorithm based on a broader IPF code set coupled with evidence of hospitalisation.

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Early Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis: A Narrative Review

Cited by 9 publications

References 95 publications

Focal Usual Interstitial Pneumonia-like Fibrosis is a Core Prognostic Factor in Progressive Pulmonary Fibrosis

Focal Usual Interstitial Pneumonia-like Fibrosis is a Core Prognostic Factor in Progressive Pulmonary Fibrosis

Memahami Penyakit Paru Interstisial: Pengenalan Dini pada Layanan Kesehatan Primer

Validation of the recording of idiopathic pulmonary fibrosis in routinely collected electronic healthcare records in England

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