Włóknienie płuc idiopatyczne – Rokowania, prognozy i postęp choroby

Włóknienie płuc idiopatyczne
Rokowania, prognozy i postęp choroby

Idiopatyczne włóknienie płuc (IPF) charakteryzuje się postępującym spadkiem funkcji płuc i mediana przeżycia wynosi 3-5 lat od diagnozy, choć 20-25% pacjentów żyje ponad 10 lat. Rokowanie opiera się na ocenie progresji choroby, uwzględniając parametry czynnościowe (spadek FVC ≥5-10% i DLCO ≥10-15% w ciągu 6-12 miesięcy), kliniczne (męska płeć, starszy wiek, duszność, nadciśnienie płucne) oraz radiologiczne (CTFLV/TLV% >11%). Test 6-minutowego marszu (6MWT) i saturacja SpO2 na jego końcu są istotnymi predyktorami przeżycia. Indeksy prognostyczne GAP i rozszerzony DO-GAP (łączący parametry wydolności wysiłkowej) poprawiają dokładność przewidywania śmiertelności. Biomarkery surowicze, takie jak miR-21-5p w pęcherzykach zewnątrzkomórkowych, wykazują potencjał prognostyczny niezależny od klasycznych parametrów.

Idiopatyczne włóknienie płuc – Rokowanie

Czynniki wpływające na rokowanie
Wielowymiarowe indeksy prognostyczne
Wpływ leczenia na rokowanie
Monitorowanie pacjentów z IPF
Zaostrzenia IPF

Podsumowanie rokowania w IPF

Kolejne rozdziały

Idiopatyczne włóknienie płuc – Rokowanie

Idiopatyczne włóknienie płuc (IPF) jest najczęstszą i najbardziej śmiertelną z idiopatycznych śródmiąższowych zapaleń płuc. Choroba charakteryzuje się postępującym spadkiem funkcji płuc i jakości życia u większości pacjentów. Mediana przeżycia pacjentów z IPF wynosi od 3 do 5 lat od momentu diagnozy, co czyni tę chorobę porównywalną do nowotworów o złym rokowaniu¹²³. Należy jednak podkreślić, że istnieje duża zmienność w przebiegu choroby wśród pacjentów, a rzeczywisty zakres przeżycia jest szeroki – nawet 20-25% pacjentów z IPF żyje ponad 10 lat od momentu diagnozy⁴.

Czynniki wpływające na rokowanie

Ocena rokowania w IPF opiera się na określeniu progresji, stabilności lub poprawy choroby, wykorzystując nie tylko najważniejsze parametry czynności płuc, ale także inne czynniki, takie jak kliniczne, radiologiczne, a nawet serologiczne⁵. Klasyczne czynniki prognostyczne, które wpływają na śmiertelność, obejmują parametry czynnościowe, kliniczne i radiologiczne⁶.

Czynniki kliniczne

Warunki kliniczne związane z gorszym rokowaniem są różnorodne i zależą od danych epidemiologicznych, takich jak męska płeć i starszy wiek, objawy takie jak podstawowa duszność, czy charakterystyka ewolucji choroby⁷. Występowanie nadciśnienia płucnego zarówno w momencie diagnozy, jak i w trakcie obserwacji, jest czynnikiem złego rokowania związanym z pogorszeniem przeżycia pacjentów z IPF⁸⁹. Kobiety i młodsi pacjenci z IPF zazwyczaj mają lepsze rokowanie, ale niekoniecznie, jeśli jednocześnie cierpią na nadciśnienie płucne lub wymagają długoterminowej tlenoterapii¹⁰.

Parametry czynności płuc

Progresja, stabilność lub poprawa choroby są klasycznie definiowane przez kryteria czynności płuc¹¹. Spośród nich, spadek wartości procentowej pojemności życiowej (FVC) jest parametrem czynności płuc, który najlepiej przewiduje śmiertelność¹². Spadek FVC o co najmniej 10% lub zdolności dyfuzyjnej płuc dla tlenku węgla (DLCO) o co najmniej 15% są uważane za punkty graniczne definiujące progresję choroby, a tym samym są czynnikami złego rokowania¹³.

Obecnie akceptuje się spadek między 5% a 10% FVC lub DLCO jako istotny punkt graniczny pogorszenia, a tym samym umożliwiający identyfikację pacjentów z gorszym rokowaniem i krótszym przeżyciem¹⁴. Spadek FVC lub DLCO o co najmniej 10% w okresie sześciu do 12 miesięcy przewiduje zwiększone ryzyko śmiertelności¹⁵.

Badania wykazały, że spadek DLCO o ≥10% w pierwszym roku po wstępnej diagnozie IPF wiąże się z wyższym ryzykiem przyszłej śmiertelności, a wpływ prognostyczny tego spadku jest niezależny od spadku FVC. Seryjne monitorowanie DLCO może oferować dodatkowe informacje prognostyczne w porównaniu z samym monitorowaniem FVC, co potencjalnie może wpływać na wczesne decyzje terapeutyczne u pacjentów nowo zdiagnozowanych z IPF¹⁶¹⁷.

Ponadto, określenie DLCO poniżej 35-40% wartości przewidywanej wydaje się być związane z przeżyciem krótszym niż dwa lata¹⁸.

Test 6-minutowego marszu

Test 6-minutowego marszu (6MWT) jest powszechnie używany w chorobach serca, przewlekłej obturacyjnej chorobie płuc (POChP) i nadciśnieniu płucnym. Jego użyteczność opiera się głównie na różnicy między poziomem saturacji tlenem mierzonym pulsoksymetrycznie (SpO2) na początku i na końcu marszu, czyli całkowitej desaturacji, a także na zmniejszeniu dystansu przebytego w kolejnych badaniach¹⁹.

Parametry 6MWT uważane za predyktory gorszego przeżycia w IPF to pomiar SpO2 na końcu sześciu minut oraz przebyty dystans²⁰. 6MWT dostarcza również informacji o tym, kiedy rozpocząć ambulatoryjną suplementację tlenem²¹.

Zmniejszony dystans w 6MWT (6MWD) oraz hipoksemia wysiłkowa są czynnikami silnie prognostycznymi dla całkowitej śmiertelności. Dodanie tych zmiennych do indeksu GAP (Gender-Age-Physiology) znacząco poprawia dyskryminację modelu²²²³.

Tomografia komputerowa o wysokiej rozdzielczości

Znaczenie tomografii komputerowej o wysokiej rozdzielczości (HRCT) w IPF nie wynika jedynie z jej wartości jako narzędzia diagnostycznego na tyle silnego, by zepchnąć na drugi plan otwartą biopsję płuc, która była uważana do niedawna za złoty standard diagnozy, ale także z jej istotnej roli w monitorowaniu i ocenie rokowania choroby, biorąc pod uwagę, że HRCT jest w stanie określić początkowy zasięg i postęp choroby w czasie²⁴²⁵.

Ilościowa analiza objętościowa CT może być przydatna do oceny stopnia zwłóknienia w płucach. Stosunek objętości zwłóknienia płuc w CT do całkowitej objętości płuc (CTFLV/TLV%) może być cennym biomarkerem do precyzyjnego przewidywania średnio-długoterminowego rokowania u poszczególnych pacjentów z IPF²⁶.

Badania wykazały, że wyjściowy CTFLV/TLV% był znacząco skorelowany z czasem przeżycia. Wartość graniczna 11% dla CTFLV/TLV% wykazała istotną różnicę w śmiertelności po 5 latach. Ryzyko śmierci wzrasta bezpośrednio wraz ze wzrostem początkowego CTFLV/TLV%. Opracowano formuły do przewidywania prawdopodobieństwa śmierci pacjentów z IPF w okresie od jednego do pięciu lat, które mogą służyć jako odniesienie w podejmowaniu decyzji klinicznych dla poszczególnych pacjentów²⁷.

Ilościowe narzędzia do oceny IPF oparte na analizie opacifikacji płuc (OFL) wykazały podobną skuteczność jak zaawansowane narzędzia do analizy wieloteksturowej (MTFL) w prognozowaniu przeżycia 3-letniego. Nie zaobserwowano istotnej różnicy w ilościowej ocenie choroby, korelacji z parametrami spirometrycznymi i związkiem z rokowaniem pacjenta między dedykowanym i uznanym narzędziem do analizy wieloteksturowej płuc CALIPER a badanym narzędziem opartym na opacyfikacji Pneumonia²⁸²⁹.

Biomarkery serologiczne

Mimo że nie istnieje pojedynczy biomarker szeroko stosowany klinicznie do ustalenia rokowania lub do stosowania podczas monitorowania IPF, ostatnie badania z niektórymi markerami surowiczymi są związane z rokowaniem choroby³⁰³¹.

Wykazano, że mikroRNA w pęcherzykach zewnątrzkomórkowych surowicy (EV) ma potencjał jako biomarker prognostyczny dla IPF. Badania pokazały, że miR-21-5p w surowiczych EV był niezależnie związany ze śmiertelnością w ciągu następnych 30 miesięcy, nawet po dostosowaniu do innych zmiennych³². W analizie przeżycia pacjenci z IPF, których podstawowy poziom miR-21-5p w surowiczych EV był wysoki, mieli znacznie gorsze rokowanie w ciągu 30 miesięcy³³³⁴.

Wielowymiarowe indeksy prognostyczne

W ostatnich latach opracowano indeksy wielowymiarowe, aby zidentyfikować najbardziej dokładne czynniki prognostyczne w IPF, a także ustanowić praktyczną i klinicznie użyteczną metodę ich integracji i przewidywania indywidualnego ryzyka śmiertelności³⁵.

Indeks GAP

Najbardziej szeroko zwalidowanym narzędziem prognostycznym jest opracowany w 2012 roku przez Ley i wsp. indeks GAP, który obejmuje płeć (Gender), wiek (Age) i fizjologię (Physiology – FVC i DLCO)³⁶. Indeks GAP jest łatwym w użyciu modelem predykcji śmiertelności wyjściowej w IPF³⁷.

Oryginalny indeks GAP miał statystykę C 0,676 (95% CI 0,635 do 0,717) po zastosowaniu do kohort pacjentów z IPF³⁸.

Indeks DO-GAP

Dodanie parametrów wydolności wysiłkowej (dystans w 6MWT i hipoksemia wysiłkowa) do indeksu GAP znacząco poprawiło dyskryminację modelu. Nowy indeks, nazwany indeksem DO-GAP (Distance-Oxygen-GAP), wykazał poprawioną dyskryminację w kohorcie walidacji wewnętrznej w porównaniu z oryginalnym i przepasowanym indeksem GAP, a także zadowalającą kalibrację³⁹.

Prosty, oparty na punktach model predykcji ryzyka wyjściowego, łączący parametry wydolności wysiłkowej z oryginalnym indeksem GAP, może poprawić prognozowanie u pacjentów z IPF i ma potencjalne zastosowanie zarówno w badaniach naukowych, jak i praktyce klinicznej⁴⁰.

Wpływ leczenia na rokowanie

Kluczowe badania, które doprowadziły do nowego paradygmatu leczenia IPF, wykorzystywały zastępczy punkt końcowy spadku FVC w ciągu 52 tygodni, wykazując złagodzenie utraty funkcji o około 50% w ciągu roku⁴¹.

Dalsza analiza, która obejmowała rozszerzony test otwarty (INPULSIS-ON) i badanie eksploracyjne fazy IIIb (łącznie ponad 1000 pacjentów), sugerowała 5-letnie przedłużone przeżycie w leczonej grupie pacjentów (mediana przeżycia 3 lata w grupie placebo i 8 lat w grupie leczonej)⁴².

Analiza 2-letniej obserwacji niemieckiego rejestru IPF wykazała znaczne zmniejszenie śmiertelności z 87% w grupie leczonej w porównaniu do 46% u pacjentów bez leczenia w ciągu 1 roku oraz z 62% w porównaniu do 21% odpowiednio w 2-letnim okresie⁴³.

Systematyczny przegląd i metaanaliza 26 badań obejmujących prawie 13 000 pacjentów wykazały zmniejszenie śmiertelności z wszystkich przyczyn, z ryzykiem względnym 0,55 (95% CI, 0,45-0,66) na korzyść leków przeciwzwłóknieniowych (antifibrotyków)⁴⁴.

Monitorowanie pacjentów z IPF

Główne powody monitorowania pacjentów z idiopatycznym włóknieniem płuc to ocena progresji choroby, która może prowadzić do zmiany leczenia, oraz identyfikacja chorób współistniejących i powikłań, które mogą wpływać na decyzje o rozpoczęciu lub zaprzestaniu leczenia przeciwzwłóknieniowego, rozpoczęciu tlenoterapii, skierowaniu na przeszczep płuc i rozpoczęciu działań paliatywnych⁴⁵⁴⁶.

Seryjne badania kontrolne zarówno FVC, jak i DLCO są konieczne dla bardziej kompleksowej oceny pacjentów z IPF⁴⁷.

Zaostrzenia IPF

Zaostrzenie IPF (AE-IPF) może być spowodowane znaną przyczyną (taką jak infekcja, aspiracja lub leki) lub być idiopatyczne (bez znanego czynnika wywołującego). Stan ten jest związany ze złym rokowaniem i jest odpowiedzialny za większość hospitalizacji związanych z IPF⁴⁸.

Czynnik prognostyczny	Wpływ na rokowanie	Wartość graniczna
Spadek FVC	Silny predyktor śmiertelności	≥5-10% w ciągu 6-12 miesięcy
Spadek DLCO	Niezależny predyktor śmiertelności	≥10-15% w ciągu 6-12 miesięcy
DLCO wyjściowe	Korelacja z przeżyciem	<35-40% wartości przewidywanej
6MWT – dystans	Predyktor przeżycia	Zmniejszony dystans w kolejnych badaniach
6MWT – desaturacja	Predyktor przeżycia	Istotna desaturacja podczas wysiłku
CTFLV/TLV%	Biomarker prognostyczny	>11%
miR-21-5p w surowiczych EV	Niezależny predyktor śmiertelności	Wysokie poziomy wyjściowe
Nadciśnienie płucne	Czynnik złego rokowania	Obecność przy diagnozie lub w trakcie monitorowania
Zaostrzenie IPF (AE-IPF)	Związane ze złym rokowaniem	Wystąpienie zdarzenia

Podsumowanie rokowania w IPF

Rokowanie w idiopatycznym włóknieniu płuc pozostaje wyzwaniem ze względu na nieprzewidywalny przebieg choroby. Mediana przeżycia wynosi 3-5 lat od momentu diagnozy, jednak indywidualne różnice mogą być znaczne. Wprowadzenie leków przeciwzwłóknieniowych (nintedanib, pirfenidon) znacząco zmieniło perspektywy pacjentów, zmniejszając tempo progresji choroby i poprawiając przeżycie⁴⁹. Wielowymiarowe indeksy prognostyczne, takie jak GAP i DO-GAP, umożliwiają bardziej precyzyjne przewidywanie rokowania u poszczególnych pacjentów.

Regularne monitorowanie parametrów czynności płuc (FVC, DLCO), wydolności wysiłkowej (6MWT) oraz obrazowania (HRCT) pozwala na wczesne wykrywanie progresji choroby i odpowiednią modyfikację leczenia. Badania nad nowymi biomarkerami, takimi jak miR-21-5p w pęcherzykach zewnątrzkomórkowych surowicy, otwierają perspektywę na bardziej spersonalizowane podejście do oceny rokowania i leczenia IPF w przyszłości⁵⁰.

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Materiały źródłowe

#1 Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis
https://pmc.ncbi.nlm.nih.gov/articles/PMC6024649/
Idiopathic pulmonary fibrosis (IPF), a devastating progressive interstitial lung disease (ILD) with no known cause, is the most common and deadly of the idiopathic interstitial pneumonias. With a median survival of 35 years following diagnosis, IPF is characterized by a progressive decline in lung function and quality of life in most patients. Prognostic factors recognized classically that influence mortality include functional, clinical and radiological parameters. However, in recent years, there has also been progress in the knowledge of genetic factors and biomarkers that may be useful in the prognostic evaluation of these patients. On the other hand, the monitoring of the disease throughout its evolution is key to improving the prognosis of the patients, as it allows for taking therapeutic measures based on this evolution, even early remission for lung transplantation. This article reviews the main prognostic factors of the disease, as well as the most useful way to monitor the disease follow-up.
#2 Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis
https://www.mdpi.com/2076-3271/6/2/51
Prognostic factors recognized classically that influence mortality include functional, clinical and radiological parameters. […] With a median survival of 3â5 years following diagnosis, IPF is characterized by a progressive decline in lung function and quality of life in most patients. […] The presence of pulmonary hypertension at the diagnosis or follow-up is a factor of bad prognosis related to a worsening in the survival of patients with IPF. […] The decline between 5% and 10% of FVC or DLco as the significant cut-off point is currently accepted to be worsening, and, therefore, able to identify patients with worse prognosis and shorter survival. […] A decline in FVC or DLco of at least 10% over six to 12 months predicts an increased risk of mortality. […] The 6 MWT also provides data on when to start ambulatory oxygen supplementation.
#3 SciELO Brazil – Idiopathic pulmonary fibrosis: current diagnosis and treatment Idiopathic pulmonary fibrosis: current diagnosis and treatment
https://www.scielo.br/j/jbpneu/a/LxHMH8dXfJCpBTzC6qyH9xB/
Idiopathic pulmonary fibrosis (IPF) is a devastating chronic lung disease without a clear recognizable cause. […] Mortality is high, and most patients have an estimated survival of 3-5 years without treatment, which is comparable to cancers with poor prognosis. […] Prognostication in IPF can be challenging since the disease course, although usually progressive, might be unpredictable. […] The most widely validated prognostic tool was developed in 2012 by Ley et al, the GAP index, which comprises Gender, Age, and Physiology (FVC and DLco). […] AE-IPF is either triggered by a known cause (such as infection, aspiration, or drugs) or idiopathic (untriggered). This condition is associated with poor prognosis and is responsible for most IPF-related hospitalizations. […] The pivotal trials that culminated in the new paradigm of IPF treatment used a surrogate endpoint of FVC decline over 52 weeks, demonstrating an attenuation of functional loss by approximately 50% in a year.
#4 Prognosis and monitoring of idiopathic pulmonary fibrosis – UpToDate
https://www.uptodate.com/contents/prognosis-and-monitoring-of-idiopathic-pulmonary-fibrosis
Prognosis and monitoring of idiopathic pulmonary fibrosis […] The prognosis and monitoring of IPF will be reviewed here. The natural history of IPF is most often described as one of insidious decline in lung function resulting in progression to respiratory failure and death on average within approximately four to five years after the initial diagnosis. However, there is great variability in disease course among individual patients with IPF, and survival is influenced by several factors including variable progression rates, occurrence of acute exacerbations, and comorbid disease. […] Mortality â The median survival of IPF has been reported to range from two to five years. This estimate reflects the range of average life expectancies observed in cohorts of IPF patients, rather than the limits of an individual patient’s life expectancy. This nuance is important, as the actual range of survival of individual IPF patients is quite broad, with up to 20 to 25 percent of patients living beyond 10 years.
#5 Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis
https://pmc.ncbi.nlm.nih.gov/articles/PMC6024649/
Evaluation of Idiopathic Pulmonary Fibrosis (IPF) prognosis is based on the determination of disease progression, stability or improvement, using not only the most important ones, lung function parameters, but also others such as clinical, radiological and even serological. […] The clinical conditions that have been associated with a worse prognosis are diverse and depend on epidemiological data such as male gender and age, symptoms such as baseline dyspnoea, or characteristics of the disease evolution expecting a phenotype or another depending on the exacerbations. […] The presence of pulmonary hypertension at the diagnosis or follow-up is a factor of bad prognosis related to a worsening in the survival of patients with IPF. […] Progression, stability or improvement of the disease are classically defined by lung function criteria.
#6 Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis
https://www.mdpi.com/2076-3271/6/2/51
Prognostic factors recognized classically that influence mortality include functional, clinical and radiological parameters. […] With a median survival of 3â5 years following diagnosis, IPF is characterized by a progressive decline in lung function and quality of life in most patients. […] The presence of pulmonary hypertension at the diagnosis or follow-up is a factor of bad prognosis related to a worsening in the survival of patients with IPF. […] The decline between 5% and 10% of FVC or DLco as the significant cut-off point is currently accepted to be worsening, and, therefore, able to identify patients with worse prognosis and shorter survival. […] A decline in FVC or DLco of at least 10% over six to 12 months predicts an increased risk of mortality. […] The 6 MWT also provides data on when to start ambulatory oxygen supplementation.
#7 Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis
https://pmc.ncbi.nlm.nih.gov/articles/PMC6024649/
Evaluation of Idiopathic Pulmonary Fibrosis (IPF) prognosis is based on the determination of disease progression, stability or improvement, using not only the most important ones, lung function parameters, but also others such as clinical, radiological and even serological. […] The clinical conditions that have been associated with a worse prognosis are diverse and depend on epidemiological data such as male gender and age, symptoms such as baseline dyspnoea, or characteristics of the disease evolution expecting a phenotype or another depending on the exacerbations. […] The presence of pulmonary hypertension at the diagnosis or follow-up is a factor of bad prognosis related to a worsening in the survival of patients with IPF. […] Progression, stability or improvement of the disease are classically defined by lung function criteria.
#8 Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis
https://pmc.ncbi.nlm.nih.gov/articles/PMC6024649/
Evaluation of Idiopathic Pulmonary Fibrosis (IPF) prognosis is based on the determination of disease progression, stability or improvement, using not only the most important ones, lung function parameters, but also others such as clinical, radiological and even serological. […] The clinical conditions that have been associated with a worse prognosis are diverse and depend on epidemiological data such as male gender and age, symptoms such as baseline dyspnoea, or characteristics of the disease evolution expecting a phenotype or another depending on the exacerbations. […] The presence of pulmonary hypertension at the diagnosis or follow-up is a factor of bad prognosis related to a worsening in the survival of patients with IPF. […] Progression, stability or improvement of the disease are classically defined by lung function criteria.
#9 Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis
https://www.mdpi.com/2076-3271/6/2/51
Prognostic factors recognized classically that influence mortality include functional, clinical and radiological parameters. […] With a median survival of 3â5 years following diagnosis, IPF is characterized by a progressive decline in lung function and quality of life in most patients. […] The presence of pulmonary hypertension at the diagnosis or follow-up is a factor of bad prognosis related to a worsening in the survival of patients with IPF. […] The decline between 5% and 10% of FVC or DLco as the significant cut-off point is currently accepted to be worsening, and, therefore, able to identify patients with worse prognosis and shorter survival. […] A decline in FVC or DLco of at least 10% over six to 12 months predicts an increased risk of mortality. […] The 6 MWT also provides data on when to start ambulatory oxygen supplementation.
#10 Prognosis Predictions for IPF Patients – Pulmonary Fibrosis News Forums
https://pulmonaryfibrosisnews.com/forums/forums/topic/prognosis-predictions-for-ipf-patients/
Prognosis Predictions for IPF Patients […] Per the article, women and younger people living with IPF tend to have a better prognosis, but not necessarily if they also live with Pulmonary Hypertension or have been on long-term oxygen use/therapy. […] I believe youâre right in terms of this statistic being published before the two anti-fibrotic medications became available â it would be nice to get results of a longterm study on IPF taking both Ofev and Esbriet and compare their survival rates so we can update that hope-killing statistic of only 3-5 years. […] I know I have an email outstanding back to you â Iâll try to reply today 🙂 […] I have no idea how accurate this prognosis is, especially since I believe these figures were published before Ofev and Esbriet became available. […] It is definitely very important that we get a better and more reliable handle on life expectancies. […] I guess either OFEV or Esbriet stopped my fibrosis from increasing. […] I have ipf and in my 11th year. […] Iâm glad youâve found something that has improved your QoLâ¦ that is so important! […] We certainly need better prognosticators for this disease!
#11 Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis
https://pmc.ncbi.nlm.nih.gov/articles/PMC6024649/
Evaluation of Idiopathic Pulmonary Fibrosis (IPF) prognosis is based on the determination of disease progression, stability or improvement, using not only the most important ones, lung function parameters, but also others such as clinical, radiological and even serological. […] The clinical conditions that have been associated with a worse prognosis are diverse and depend on epidemiological data such as male gender and age, symptoms such as baseline dyspnoea, or characteristics of the disease evolution expecting a phenotype or another depending on the exacerbations. […] The presence of pulmonary hypertension at the diagnosis or follow-up is a factor of bad prognosis related to a worsening in the survival of patients with IPF. […] Progression, stability or improvement of the disease are classically defined by lung function criteria.
#12 Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis
https://pmc.ncbi.nlm.nih.gov/articles/PMC6024649/
Among them, the decrease in FVC % is the parameter of lung function that best predicts mortality. […] A decline greater than or equal to 10% of the FVC or greater than or equal to 15% of the DLco are considered the cut-off points to define disease progression and therefore are poor prognosis factors. […] The six-monthly or annual fall 10% of FVC or 15% of DLco, which are considered as the gold standard to establish the prognosis of the disease, are no longer providing a real and complete evaluation of the patients risk. […] The determination of DLco less than 3540% of the predicted seems to be related to less than two years of survival. […] The 6 MWT has generally been used in heart diseases, chronic obstructive pulmonary diseases (COPD) and pulmonary hypertension, and its usefulness in these diseases is mainly based on the difference between the oxygen saturation by pulse oximetry (SpO2) level at the beginning and at the end of the walk, i.e., the total desaturation, as well as the decrease of the distance walked at successive records.
#13 Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis
https://pmc.ncbi.nlm.nih.gov/articles/PMC6024649/
Among them, the decrease in FVC % is the parameter of lung function that best predicts mortality. […] A decline greater than or equal to 10% of the FVC or greater than or equal to 15% of the DLco are considered the cut-off points to define disease progression and therefore are poor prognosis factors. […] The six-monthly or annual fall 10% of FVC or 15% of DLco, which are considered as the gold standard to establish the prognosis of the disease, are no longer providing a real and complete evaluation of the patients risk. […] The determination of DLco less than 3540% of the predicted seems to be related to less than two years of survival. […] The 6 MWT has generally been used in heart diseases, chronic obstructive pulmonary diseases (COPD) and pulmonary hypertension, and its usefulness in these diseases is mainly based on the difference between the oxygen saturation by pulse oximetry (SpO2) level at the beginning and at the end of the walk, i.e., the total desaturation, as well as the decrease of the distance walked at successive records.
#14 Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis
https://www.mdpi.com/2076-3271/6/2/51
Prognostic factors recognized classically that influence mortality include functional, clinical and radiological parameters. […] With a median survival of 3â5 years following diagnosis, IPF is characterized by a progressive decline in lung function and quality of life in most patients. […] The presence of pulmonary hypertension at the diagnosis or follow-up is a factor of bad prognosis related to a worsening in the survival of patients with IPF. […] The decline between 5% and 10% of FVC or DLco as the significant cut-off point is currently accepted to be worsening, and, therefore, able to identify patients with worse prognosis and shorter survival. […] A decline in FVC or DLco of at least 10% over six to 12 months predicts an increased risk of mortality. […] The 6 MWT also provides data on when to start ambulatory oxygen supplementation.
#15 Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis
https://www.mdpi.com/2076-3271/6/2/51
Prognostic factors recognized classically that influence mortality include functional, clinical and radiological parameters. […] With a median survival of 3â5 years following diagnosis, IPF is characterized by a progressive decline in lung function and quality of life in most patients. […] The presence of pulmonary hypertension at the diagnosis or follow-up is a factor of bad prognosis related to a worsening in the survival of patients with IPF. […] The decline between 5% and 10% of FVC or DLco as the significant cut-off point is currently accepted to be worsening, and, therefore, able to identify patients with worse prognosis and shorter survival. […] A decline in FVC or DLco of at least 10% over six to 12 months predicts an increased risk of mortality. […] The 6 MWT also provides data on when to start ambulatory oxygen supplementation.
#16 Prognostic implication of 1-year decline in diffusing capacity in newly diagnosed idiopathic pulmonary fibrosis | Scientific Reports
https://www.nature.com/articles/s41598-024-59649-5
The progression of idiopathic pulmonary fibrosis (IPF) is assessed through serial monitoring of forced vital capacity (FVC). Currently, data regarding the clinical significance of longitudinal changes in diffusing capacity for carbon monoxide (DLCO) is lacking. We investigated the prognostic implications of a 1-year decline in DLCO in 319 patients newly diagnosed with IPF at a tertiary hospital between January 2010 and December 2020. […] Multivariable analysis showed that a 1-year decline in FVC5% predicted (aHR 2.74, 95% CI 1.88-4.00) and 1-year decline in DLCO10% predicted (aHR 2.31, 95% CI 1.47-3.62) were independently associated with a higher risk of subsequent mortality. The prognostic impact of a decline in DLCO remained significant regardless of changes in FVC, presence of emphysema, or radiographic indications of pulmonary hypertension.
#17 Prognostic implication of 1-year decline in diffusing capacity in newly diagnosed idiopathic pulmonary fibrosis | Scientific Reports
https://www.nature.com/articles/s41598-024-59649-5
The major finding of our study was that a significant decline in DLCO was associated with an increased risk of mortality, even after adjusting for other well-known prognostic factors of IPF, including age, sex, baseline FVC, and 1-year decline in FVC. […] A decline in DLCO10% predicted over the first year after the initial diagnosis of IPF was associated with a higher risk of future mortality, and this prognostic impact was independent of a decline in FVC. Serial monitoring of DLCO may offer additional prognostic insights compared with monitoring of FVC alone, which could potentially influence early treatment decisions in patients newly diagnosed with IPF. Therefore, serial follow-up assessments of both FVC and DLCO are necessary for a more comprehensive evaluation of patients with IPF.
#18 Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis
https://pmc.ncbi.nlm.nih.gov/articles/PMC6024649/
Among them, the decrease in FVC % is the parameter of lung function that best predicts mortality. […] A decline greater than or equal to 10% of the FVC or greater than or equal to 15% of the DLco are considered the cut-off points to define disease progression and therefore are poor prognosis factors. […] The six-monthly or annual fall 10% of FVC or 15% of DLco, which are considered as the gold standard to establish the prognosis of the disease, are no longer providing a real and complete evaluation of the patients risk. […] The determination of DLco less than 3540% of the predicted seems to be related to less than two years of survival. […] The 6 MWT has generally been used in heart diseases, chronic obstructive pulmonary diseases (COPD) and pulmonary hypertension, and its usefulness in these diseases is mainly based on the difference between the oxygen saturation by pulse oximetry (SpO2) level at the beginning and at the end of the walk, i.e., the total desaturation, as well as the decrease of the distance walked at successive records.
#19 Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis
https://pmc.ncbi.nlm.nih.gov/articles/PMC6024649/
Among them, the decrease in FVC % is the parameter of lung function that best predicts mortality. […] A decline greater than or equal to 10% of the FVC or greater than or equal to 15% of the DLco are considered the cut-off points to define disease progression and therefore are poor prognosis factors. […] The six-monthly or annual fall 10% of FVC or 15% of DLco, which are considered as the gold standard to establish the prognosis of the disease, are no longer providing a real and complete evaluation of the patients risk. […] The determination of DLco less than 3540% of the predicted seems to be related to less than two years of survival. […] The 6 MWT has generally been used in heart diseases, chronic obstructive pulmonary diseases (COPD) and pulmonary hypertension, and its usefulness in these diseases is mainly based on the difference between the oxygen saturation by pulse oximetry (SpO2) level at the beginning and at the end of the walk, i.e., the total desaturation, as well as the decrease of the distance walked at successive records.
#20 Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis
https://pmc.ncbi.nlm.nih.gov/articles/PMC6024649/
The parameters of the 6 MWT considered as predictors of worse survival in IPF are also the SpO2 measurement at the end of the six minutes and the distance walked. […] The importance of the High-Resolution Computed Tomography (HRCT) in IPF is not only for its value as a diagnostic tool strong enough to relegate to a second step the surgical open lung biopsy, which was considered until just a few years ago as the gold standard for diagnosis, but also for its relevant role in monitoring and evaluating prognosis of the disease, considering that HRCT is able to determine the initial extension and its progress over time. […] Despite there not being a single biomarker in widespread clinical use to establish the prognosis or to use during the monitoring of the IPF, recent studies with some serum markers are found to be related to the prognosis of the disease.
#21 Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis
https://www.mdpi.com/2076-3271/6/2/51
Prognostic factors recognized classically that influence mortality include functional, clinical and radiological parameters. […] With a median survival of 3â5 years following diagnosis, IPF is characterized by a progressive decline in lung function and quality of life in most patients. […] The presence of pulmonary hypertension at the diagnosis or follow-up is a factor of bad prognosis related to a worsening in the survival of patients with IPF. […] The decline between 5% and 10% of FVC or DLco as the significant cut-off point is currently accepted to be worsening, and, therefore, able to identify patients with worse prognosis and shorter survival. […] A decline in FVC or DLco of at least 10% over six to 12 months predicts an increased risk of mortality. […] The 6 MWT also provides data on when to start ambulatory oxygen supplementation.
#22 Derivation and validation of a simple multidimensional index incorporating exercise capacity parameters for survival prediction in idiopathic pulmonary fibrosis | Thorax
https://thorax.bmj.com/content/78/4/368
The gender-age-physiology (GAP) index is an easy-to-use baseline mortality prediction model in idiopathic pulmonary fibrosis (IPF). […] The addition of these variables to the GAP index significantly improved model discrimination. […] A simple point-based baseline-risk prediction model incorporating exercise capacity predictors into the original GAP index may improve prognostication in patients with IPF. […] Distance ambulated during 6min walk testing and exertional hypoxia are strongly predictive of mortality in IPF and the addition of these factors to the GAP index improves overall model performance. […] The distance-oxygen-GAP index, a simple, point-based baseline-risk prediction model incorporating exercise capacity parameters into the GAP index improves mortality prediction in patients with IPF and has potential utility in research studies as well as clinical practice.
#23 Derivation and validation of a simple multidimensional index incorporating exercise capacity parameters for survival prediction in idiopathic pulmonary fibrosis | Thorax
https://thorax.bmj.com/content/78/4/368
The original GAP index had a C-statistic of 0.676 (95% CI 0.635 to 0.717) when applied to our cohort. […] Notably, reduced 6MWD and exertional hypoxia were factors strongly predictive of overall mortality. […] The addition of these variables to the GAP index significantly improved model discrimination. […] Our new index demonstrated improved discrimination in the internal validation cohort compared with the original and refitted GAP index as well as satisfactory calibration. […] 6MWD and exertional hypoxia were significant prognostic factors strongly associated with overall survival. These prognostic factors were then combined into a new model, the DO-GAP index, which demonstrated improved outcome discrimination and calibration compared with the original GAP index.
#24 Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis
https://pmc.ncbi.nlm.nih.gov/articles/PMC6024649/
The parameters of the 6 MWT considered as predictors of worse survival in IPF are also the SpO2 measurement at the end of the six minutes and the distance walked. […] The importance of the High-Resolution Computed Tomography (HRCT) in IPF is not only for its value as a diagnostic tool strong enough to relegate to a second step the surgical open lung biopsy, which was considered until just a few years ago as the gold standard for diagnosis, but also for its relevant role in monitoring and evaluating prognosis of the disease, considering that HRCT is able to determine the initial extension and its progress over time. […] Despite there not being a single biomarker in widespread clinical use to establish the prognosis or to use during the monitoring of the IPF, recent studies with some serum markers are found to be related to the prognosis of the disease.
#25 Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis
https://www.mdpi.com/2076-3271/6/2/51
The importance of the High-Resolution Computed Tomography (HRCT) in IPF is not only for its value as a diagnostic tool strong enough to relegate to a second step the surgical open lung biopsy, but also for its relevant role in monitoring and evaluating prognosis of the disease. […] Despite there not being a single biomarker in widespread clinical use to establish the prognosis or to use during the monitoring of the IPF, recent studies with some serum markers are found to be related to the prognosis of the disease. […] The optimal multidimensional index should probably emerge from a multicenter effort as a prospective study involving a large number of new patients, a reasonable number of variables, with a sufficiently long follow-up period in order to reflect adverse events and mortality. […] The main reasons for monitoring patients with Idiopathic Pulmonary Fibrosis are to evaluate the disease progression that can lead to a change in treatment and identify comorbidities and complications that may influence when to initiate or stop antifibrotic treatment.
#26 Medium-long term prognosis prediction for idiopathic pulmonary fibrosis patients based on quantitative analysis of fibrotic lung volume | Respiratory Research | Full Text
https://respiratory-research.biomedcentral.com/articles/10.1186/s12931-022-02276-3
Quantitative volume analysis of CT might be useful for evaluating the extent of fibrosis in the lung. […] The CTFLV/TLV% could be a valuable biomarker for precisely predicting the medium-long term prognosis of individual patients with IPF. […] The univariate analysis indicated that CTFLV/TLV% along with DLCO%pred, KCO%pred and GAP index were significantly correlated with survival. […] However, only CTFLV/TLV% was meaningful in the multivariate analysis for prognostic prediction (HR 1.114, 95% CI 1.0471.184, P=0.0006), and the best cutoff was 11%, based on receiver operating characteristic (ROC) curve analysis. […] Given the CTFLV/TLV% data, the death probability of a patient at 1 year, 3 years and 5 years could be calculated by using a particular formula. […] The formulas were tested by the validation cohort, showed high sensitivity (88.2%), specificity (92.8%) and accuracy (90.3%).
#27 Medium-long term prognosis prediction for idiopathic pulmonary fibrosis patients based on quantitative analysis of fibrotic lung volume | Respiratory Research | Full Text
https://respiratory-research.biomedcentral.com/articles/10.1186/s12931-022-02276-3
Therefore, we suggest that the baseline DLCO%pred, KCO%pred, and GAP index, and especially the baseline CTFLV/TLV%, are predictors of the survival time for patients with IPF. […] The baseline CTFLV/TLV% showed a significant difference between the two groups: 29.6%11.6% in the death group and 10.7%7.1% in the survival group. […] The KaplanMeier survival curve demonstrated a significant difference in mortality at 5 years, which was defined by a cutoff point of 11% for CTFLV/TLV%. […] The logistic equation was as follows: A = – 3.5749 + 0.2016 Ã CTFLV/TLV%. […] It is clear from the equation that the risk of death increased directly with the increase in the baseline CTFLV/TLV%. […] These formulas were validated in the validation cohort with high accuracy. […] The baseline CTFLV/TLV% was significantly correlated with the survival time. […] Formulas to predict the probability of one to five years death of IPF patients were calculated and might serve as a reference in clinical decision-making for individual patients.
#28 Comparing multi-texture fibrosis analysis versus binary opacity-based abnormality detection for quantitative assessment of idiopathic pulmonary fibrosis | Scientific Reports
https://www.nature.com/articles/s41598-025-85135-7
Automated tools for quantification of idiopathic pulmonary fibrosis (IPF) can aid in ensuring reproducibility, however their complexity and costs can differ substantially. […] The prognostic values for 3-year patient survival of OFL, LSS and MTFL were investigated by multivariable Cox-proportional-hazards (CPH) models including sex, age and TLC and including sex, age and VC. […] No significant difference between OFL and MTFL was observed (median and interquartile range: OFL=29% [20-38%], MTFL=31% [19-45%]; P=0.44). […] Both analyses were significant predictors in the multivariable CPH analysis including TLC (hazard-ratios: MTFL 1.03 [1.01-1.06], P=0.02; OFL 1.03 [1.00-1.06], P=0.03). […] OFL showed significance in Kaplan-Meier analysis (MTFL: P=0.17; OFL: P=0.03). […] The results of the current study show that OFL quantification of this tool did not significantly differ and showed high correlation in linear regression analysis to MTFL measured by the sophisticated CALIPER lung texture analysis tool.
#29 Comparing multi-texture fibrosis analysis versus binary opacity-based abnormality detection for quantitative assessment of idiopathic pulmonary fibrosis | Scientific Reports
https://www.nature.com/articles/s41598-025-85135-7
No substantial difference in association of OFL and MTFL with the prognosis of 42 IPF patients was observed in multivariable CPH models along age, sex and TLC. […] Kaplan-Meier analysis indicate association of OFL to patient survival that could not be seen for MTFL. […] Overall, there are no significant differences in usability between the tools, except for the fact that the opacity-based Pneumonia tool allows for adjustments. […] For the current cohort of IPF patients, no substantial difference in quantification of disease, correlation to spirometry parameters and association with patient prognosis was observed between the dedicated and established CALIPER multi-texture lung analysis tool and the investigated opacity-based Pneumonia tool.
#30 Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis
https://pmc.ncbi.nlm.nih.gov/articles/PMC6024649/
The parameters of the 6 MWT considered as predictors of worse survival in IPF are also the SpO2 measurement at the end of the six minutes and the distance walked. […] The importance of the High-Resolution Computed Tomography (HRCT) in IPF is not only for its value as a diagnostic tool strong enough to relegate to a second step the surgical open lung biopsy, which was considered until just a few years ago as the gold standard for diagnosis, but also for its relevant role in monitoring and evaluating prognosis of the disease, considering that HRCT is able to determine the initial extension and its progress over time. […] Despite there not being a single biomarker in widespread clinical use to establish the prognosis or to use during the monitoring of the IPF, recent studies with some serum markers are found to be related to the prognosis of the disease.
#31 Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis
https://www.mdpi.com/2076-3271/6/2/51
The importance of the High-Resolution Computed Tomography (HRCT) in IPF is not only for its value as a diagnostic tool strong enough to relegate to a second step the surgical open lung biopsy, but also for its relevant role in monitoring and evaluating prognosis of the disease. […] Despite there not being a single biomarker in widespread clinical use to establish the prognosis or to use during the monitoring of the IPF, recent studies with some serum markers are found to be related to the prognosis of the disease. […] The optimal multidimensional index should probably emerge from a multicenter effort as a prospective study involving a large number of new patients, a reasonable number of variables, with a sufficiently long follow-up period in order to reflect adverse events and mortality. […] The main reasons for monitoring patients with Idiopathic Pulmonary Fibrosis are to evaluate the disease progression that can lead to a change in treatment and identify comorbidities and complications that may influence when to initiate or stop antifibrotic treatment.
#32 Serum extracellular vesicular miR-21-5p is a predictor of the prognosis in idiopathic pulmonary fibrosis | Respiratory Research | Full Text
https://respiratory-research.biomedcentral.com/articles/10.1186/s12931-016-0427-3
Serum EV miR-21-5p was independently associated with mortality during the following 30 months, even after adjustment for other variables. […] In the survival analysis, IPF patients whose baseline serum EV miR-21-5p was high had a significantly poorer prognosis over 30 months. […] Our results suggest that serum EV miR-21-5p has potential as a prognostic biomarker for IPF. […] The baseline levels of the serum EV miR-21-5p were significantly associated with the disease progression (the decline in the percent-predicted VC) and mortality. […] Our study is the first report to suggest that the microRNA in the serum EVs could be a promising candidate for a prognostic biomarker in patients with IPF. […] We demonstrated that the association between miR-21-5p and mortality was significant even after adjusting for gender, KL-6, %VC and age.
#33 Serum extracellular vesicular miR-21-5p is a predictor of the prognosis in idiopathic pulmonary fibrosis | Respiratory Research | Full Text
https://respiratory-research.biomedcentral.com/articles/10.1186/s12931-016-0427-3
Serum EV miR-21-5p was independently associated with mortality during the following 30 months, even after adjustment for other variables. […] In the survival analysis, IPF patients whose baseline serum EV miR-21-5p was high had a significantly poorer prognosis over 30 months. […] Our results suggest that serum EV miR-21-5p has potential as a prognostic biomarker for IPF. […] The baseline levels of the serum EV miR-21-5p were significantly associated with the disease progression (the decline in the percent-predicted VC) and mortality. […] Our study is the first report to suggest that the microRNA in the serum EVs could be a promising candidate for a prognostic biomarker in patients with IPF. […] We demonstrated that the association between miR-21-5p and mortality was significant even after adjusting for gender, KL-6, %VC and age.
#34 Serum extracellular vesicular miR-21-5p is a predictor of the prognosis in idiopathic pulmonary fibrosis | Respiratory Research | Full Text
https://respiratory-research.biomedcentral.com/articles/10.1186/s12931-016-0427-3
The Kaplan-Meier analysis showed that the survival of the patients with higher serum EV miR-21-5p levels was significantly worse than that of the patients with lower EV miR-21-5p levels. […] These analyses clearly showed that the survival of the patients with high levels of serum EV miR-21 was significantly worse than that of the patients with low levels of serum EV miR-21.
#35 Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis
https://pmc.ncbi.nlm.nih.gov/articles/PMC6024649/
In recent years, multidimensional indices have been developed to identify the most accurate prognostic factors in IPF, and also to establish a practical and clinically useful method to integrate them and predict individual mortality risk. […] The main reasons for monitoring patients with Idiopathic Pulmonary Fibrosis are to evaluate the disease progression that can lead to a change in treatment and identify comorbidities and complications that may influence when to initiate or stop antifibrotic treatment, when to establish oxygen therapy, when to refer to lung transplantation and when to initiate palliative measures.
#36 SciELO Brazil – Idiopathic pulmonary fibrosis: current diagnosis and treatment Idiopathic pulmonary fibrosis: current diagnosis and treatment
https://www.scielo.br/j/jbpneu/a/LxHMH8dXfJCpBTzC6qyH9xB/
Idiopathic pulmonary fibrosis (IPF) is a devastating chronic lung disease without a clear recognizable cause. […] Mortality is high, and most patients have an estimated survival of 3-5 years without treatment, which is comparable to cancers with poor prognosis. […] Prognostication in IPF can be challenging since the disease course, although usually progressive, might be unpredictable. […] The most widely validated prognostic tool was developed in 2012 by Ley et al, the GAP index, which comprises Gender, Age, and Physiology (FVC and DLco). […] AE-IPF is either triggered by a known cause (such as infection, aspiration, or drugs) or idiopathic (untriggered). This condition is associated with poor prognosis and is responsible for most IPF-related hospitalizations. […] The pivotal trials that culminated in the new paradigm of IPF treatment used a surrogate endpoint of FVC decline over 52 weeks, demonstrating an attenuation of functional loss by approximately 50% in a year.
#37 Derivation and validation of a simple multidimensional index incorporating exercise capacity parameters for survival prediction in idiopathic pulmonary fibrosis | Thorax
https://thorax.bmj.com/content/78/4/368
The gender-age-physiology (GAP) index is an easy-to-use baseline mortality prediction model in idiopathic pulmonary fibrosis (IPF). […] The addition of these variables to the GAP index significantly improved model discrimination. […] A simple point-based baseline-risk prediction model incorporating exercise capacity predictors into the original GAP index may improve prognostication in patients with IPF. […] Distance ambulated during 6min walk testing and exertional hypoxia are strongly predictive of mortality in IPF and the addition of these factors to the GAP index improves overall model performance. […] The distance-oxygen-GAP index, a simple, point-based baseline-risk prediction model incorporating exercise capacity parameters into the GAP index improves mortality prediction in patients with IPF and has potential utility in research studies as well as clinical practice.
#38 Derivation and validation of a simple multidimensional index incorporating exercise capacity parameters for survival prediction in idiopathic pulmonary fibrosis | Thorax
https://thorax.bmj.com/content/78/4/368
The original GAP index had a C-statistic of 0.676 (95% CI 0.635 to 0.717) when applied to our cohort. […] Notably, reduced 6MWD and exertional hypoxia were factors strongly predictive of overall mortality. […] The addition of these variables to the GAP index significantly improved model discrimination. […] Our new index demonstrated improved discrimination in the internal validation cohort compared with the original and refitted GAP index as well as satisfactory calibration. […] 6MWD and exertional hypoxia were significant prognostic factors strongly associated with overall survival. These prognostic factors were then combined into a new model, the DO-GAP index, which demonstrated improved outcome discrimination and calibration compared with the original GAP index.
#39 Derivation and validation of a simple multidimensional index incorporating exercise capacity parameters for survival prediction in idiopathic pulmonary fibrosis | Thorax
https://thorax.bmj.com/content/78/4/368
The original GAP index had a C-statistic of 0.676 (95% CI 0.635 to 0.717) when applied to our cohort. […] Notably, reduced 6MWD and exertional hypoxia were factors strongly predictive of overall mortality. […] The addition of these variables to the GAP index significantly improved model discrimination. […] Our new index demonstrated improved discrimination in the internal validation cohort compared with the original and refitted GAP index as well as satisfactory calibration. […] 6MWD and exertional hypoxia were significant prognostic factors strongly associated with overall survival. These prognostic factors were then combined into a new model, the DO-GAP index, which demonstrated improved outcome discrimination and calibration compared with the original GAP index.
#40 Derivation and validation of a simple multidimensional index incorporating exercise capacity parameters for survival prediction in idiopathic pulmonary fibrosis | Thorax
https://thorax.bmj.com/content/78/4/368
The gender-age-physiology (GAP) index is an easy-to-use baseline mortality prediction model in idiopathic pulmonary fibrosis (IPF). […] The addition of these variables to the GAP index significantly improved model discrimination. […] A simple point-based baseline-risk prediction model incorporating exercise capacity predictors into the original GAP index may improve prognostication in patients with IPF. […] Distance ambulated during 6min walk testing and exertional hypoxia are strongly predictive of mortality in IPF and the addition of these factors to the GAP index improves overall model performance. […] The distance-oxygen-GAP index, a simple, point-based baseline-risk prediction model incorporating exercise capacity parameters into the GAP index improves mortality prediction in patients with IPF and has potential utility in research studies as well as clinical practice.
#41 SciELO Brazil – Idiopathic pulmonary fibrosis: current diagnosis and treatment Idiopathic pulmonary fibrosis: current diagnosis and treatment
https://www.scielo.br/j/jbpneu/a/LxHMH8dXfJCpBTzC6qyH9xB/
Idiopathic pulmonary fibrosis (IPF) is a devastating chronic lung disease without a clear recognizable cause. […] Mortality is high, and most patients have an estimated survival of 3-5 years without treatment, which is comparable to cancers with poor prognosis. […] Prognostication in IPF can be challenging since the disease course, although usually progressive, might be unpredictable. […] The most widely validated prognostic tool was developed in 2012 by Ley et al, the GAP index, which comprises Gender, Age, and Physiology (FVC and DLco). […] AE-IPF is either triggered by a known cause (such as infection, aspiration, or drugs) or idiopathic (untriggered). This condition is associated with poor prognosis and is responsible for most IPF-related hospitalizations. […] The pivotal trials that culminated in the new paradigm of IPF treatment used a surrogate endpoint of FVC decline over 52 weeks, demonstrating an attenuation of functional loss by approximately 50% in a year.
#42 SciELO Brazil – Idiopathic pulmonary fibrosis: current diagnosis and treatment Idiopathic pulmonary fibrosis: current diagnosis and treatment
https://www.scielo.br/j/jbpneu/a/LxHMH8dXfJCpBTzC6qyH9xB/
A further analysis that included the extended open-label trial (INPULSIS-ON) and an exploratory phase IIIb trial (combined for more than 1,000 patients) suggested a 5-year extended survival in the treated group of patients (median survival of 3 years in the placebo group and of 8 years in the treated group). […] The 2-year follow-up analysis of the German IPF registry likewise found a significant reduction in mortality from 87% in the treated group against 46% in patients without treatment in 1 year, and from 62% against 21%, respectively, in the 2-year period. […] More recently, a systematic review and meta-analysis of 26 studies comprising almost 13,000 patients have shown a reduction in mortality from all causes, with a relative risk of 0.55 (95% CI, 0.45-0.66) favoring antifibrotics. […] Therefore, it should be regarded as enough to reassure a probable survival benefit and a significant reduction in mortality due to IPF.
#43 SciELO Brazil – Idiopathic pulmonary fibrosis: current diagnosis and treatment Idiopathic pulmonary fibrosis: current diagnosis and treatment
https://www.scielo.br/j/jbpneu/a/LxHMH8dXfJCpBTzC6qyH9xB/
A further analysis that included the extended open-label trial (INPULSIS-ON) and an exploratory phase IIIb trial (combined for more than 1,000 patients) suggested a 5-year extended survival in the treated group of patients (median survival of 3 years in the placebo group and of 8 years in the treated group). […] The 2-year follow-up analysis of the German IPF registry likewise found a significant reduction in mortality from 87% in the treated group against 46% in patients without treatment in 1 year, and from 62% against 21%, respectively, in the 2-year period. […] More recently, a systematic review and meta-analysis of 26 studies comprising almost 13,000 patients have shown a reduction in mortality from all causes, with a relative risk of 0.55 (95% CI, 0.45-0.66) favoring antifibrotics. […] Therefore, it should be regarded as enough to reassure a probable survival benefit and a significant reduction in mortality due to IPF.
#44 SciELO Brazil – Idiopathic pulmonary fibrosis: current diagnosis and treatment Idiopathic pulmonary fibrosis: current diagnosis and treatment
https://www.scielo.br/j/jbpneu/a/LxHMH8dXfJCpBTzC6qyH9xB/
A further analysis that included the extended open-label trial (INPULSIS-ON) and an exploratory phase IIIb trial (combined for more than 1,000 patients) suggested a 5-year extended survival in the treated group of patients (median survival of 3 years in the placebo group and of 8 years in the treated group). […] The 2-year follow-up analysis of the German IPF registry likewise found a significant reduction in mortality from 87% in the treated group against 46% in patients without treatment in 1 year, and from 62% against 21%, respectively, in the 2-year period. […] More recently, a systematic review and meta-analysis of 26 studies comprising almost 13,000 patients have shown a reduction in mortality from all causes, with a relative risk of 0.55 (95% CI, 0.45-0.66) favoring antifibrotics. […] Therefore, it should be regarded as enough to reassure a probable survival benefit and a significant reduction in mortality due to IPF.
#45 Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis
https://pmc.ncbi.nlm.nih.gov/articles/PMC6024649/
In recent years, multidimensional indices have been developed to identify the most accurate prognostic factors in IPF, and also to establish a practical and clinically useful method to integrate them and predict individual mortality risk. […] The main reasons for monitoring patients with Idiopathic Pulmonary Fibrosis are to evaluate the disease progression that can lead to a change in treatment and identify comorbidities and complications that may influence when to initiate or stop antifibrotic treatment, when to establish oxygen therapy, when to refer to lung transplantation and when to initiate palliative measures.
#46 Prognosis and Follow-Up of Idiopathic Pulmonary Fibrosis
https://www.mdpi.com/2076-3271/6/2/51
The importance of the High-Resolution Computed Tomography (HRCT) in IPF is not only for its value as a diagnostic tool strong enough to relegate to a second step the surgical open lung biopsy, but also for its relevant role in monitoring and evaluating prognosis of the disease. […] Despite there not being a single biomarker in widespread clinical use to establish the prognosis or to use during the monitoring of the IPF, recent studies with some serum markers are found to be related to the prognosis of the disease. […] The optimal multidimensional index should probably emerge from a multicenter effort as a prospective study involving a large number of new patients, a reasonable number of variables, with a sufficiently long follow-up period in order to reflect adverse events and mortality. […] The main reasons for monitoring patients with Idiopathic Pulmonary Fibrosis are to evaluate the disease progression that can lead to a change in treatment and identify comorbidities and complications that may influence when to initiate or stop antifibrotic treatment.
#47 Prognostic implication of 1-year decline in diffusing capacity in newly diagnosed idiopathic pulmonary fibrosis | Scientific Reports
https://www.nature.com/articles/s41598-024-59649-5
The major finding of our study was that a significant decline in DLCO was associated with an increased risk of mortality, even after adjusting for other well-known prognostic factors of IPF, including age, sex, baseline FVC, and 1-year decline in FVC. […] A decline in DLCO10% predicted over the first year after the initial diagnosis of IPF was associated with a higher risk of future mortality, and this prognostic impact was independent of a decline in FVC. Serial monitoring of DLCO may offer additional prognostic insights compared with monitoring of FVC alone, which could potentially influence early treatment decisions in patients newly diagnosed with IPF. Therefore, serial follow-up assessments of both FVC and DLCO are necessary for a more comprehensive evaluation of patients with IPF.
#48 SciELO Brazil – Idiopathic pulmonary fibrosis: current diagnosis and treatment Idiopathic pulmonary fibrosis: current diagnosis and treatment
https://www.scielo.br/j/jbpneu/a/LxHMH8dXfJCpBTzC6qyH9xB/
Idiopathic pulmonary fibrosis (IPF) is a devastating chronic lung disease without a clear recognizable cause. […] Mortality is high, and most patients have an estimated survival of 3-5 years without treatment, which is comparable to cancers with poor prognosis. […] Prognostication in IPF can be challenging since the disease course, although usually progressive, might be unpredictable. […] The most widely validated prognostic tool was developed in 2012 by Ley et al, the GAP index, which comprises Gender, Age, and Physiology (FVC and DLco). […] AE-IPF is either triggered by a known cause (such as infection, aspiration, or drugs) or idiopathic (untriggered). This condition is associated with poor prognosis and is responsible for most IPF-related hospitalizations. […] The pivotal trials that culminated in the new paradigm of IPF treatment used a surrogate endpoint of FVC decline over 52 weeks, demonstrating an attenuation of functional loss by approximately 50% in a year.
#49 Prognosis Predictions for IPF Patients – Pulmonary Fibrosis News Forums
https://pulmonaryfibrosisnews.com/forums/forums/topic/prognosis-predictions-for-ipf-patients/
Prognosis Predictions for IPF Patients […] Per the article, women and younger people living with IPF tend to have a better prognosis, but not necessarily if they also live with Pulmonary Hypertension or have been on long-term oxygen use/therapy. […] I believe youâre right in terms of this statistic being published before the two anti-fibrotic medications became available â it would be nice to get results of a longterm study on IPF taking both Ofev and Esbriet and compare their survival rates so we can update that hope-killing statistic of only 3-5 years. […] I know I have an email outstanding back to you â Iâll try to reply today 🙂 […] I have no idea how accurate this prognosis is, especially since I believe these figures were published before Ofev and Esbriet became available. […] It is definitely very important that we get a better and more reliable handle on life expectancies. […] I guess either OFEV or Esbriet stopped my fibrosis from increasing. […] I have ipf and in my 11th year. […] Iâm glad youâve found something that has improved your QoLâ¦ that is so important! […] We certainly need better prognosticators for this disease!
#50 Serum extracellular vesicular miR-21-5p is a predictor of the prognosis in idiopathic pulmonary fibrosis | Respiratory Research | Full Text
https://respiratory-research.biomedcentral.com/articles/10.1186/s12931-016-0427-3
Serum EV miR-21-5p was independently associated with mortality during the following 30 months, even after adjustment for other variables. […] In the survival analysis, IPF patients whose baseline serum EV miR-21-5p was high had a significantly poorer prognosis over 30 months. […] Our results suggest that serum EV miR-21-5p has potential as a prognostic biomarker for IPF. […] The baseline levels of the serum EV miR-21-5p were significantly associated with the disease progression (the decline in the percent-predicted VC) and mortality. […] Our study is the first report to suggest that the microRNA in the serum EVs could be a promising candidate for a prognostic biomarker in patients with IPF. […] We demonstrated that the association between miR-21-5p and mortality was significant even after adjusting for gender, KL-6, %VC and age.