Achalazja
Epidemiologia
Achalazja to rzadka choroba przełyku charakteryzująca się zaburzeniami motoryki, brakiem perystaltyki oraz niedostatecznym rozkurczem dolnego zwieracza przełyku (LES) podczas połykania. Epidemiologia achalazji wykazuje zróżnicowaną zapadalność, wahającą się od 0,03 do 26,0/100 000 osób rocznie, oraz chorobowość od około 0,05% (50/100 000) do 162,1/100 000, w zależności od regionu i metod diagnostycznych, zwłaszcza manometrii wysokiej rozdzielczości (HRM). Zapadalność i chorobowość rosną, co może wynikać z lepszej diagnostyki i świadomości klinicznej. Achalazja dotyka obu płci i wszystkich grup etnicznych, z pikami zachorowań w 3. i 6. dekadzie życia. Występują różnice geograficzne i etniczne, co sugeruje udział czynników genetycznych. Choroba jest powolna, a jej długotrwałe trwanie (powyżej 10-15 lat) wiąże się ze zwiększonym ryzykiem rozwoju raka przełyku, zwłaszcza płaskonabłonkowego, z ryzykiem względnym 16-28-krotnie wyższym, choć bezwzględne ryzyko pozostaje niskie (0,59/100 osobolat).
- Epidemiologia achalazji
- Nadzór i monitorowanie pacjentów z achalazją
- Achalazja a ryzyko rozwoju raka przełyku
- Kontrowersje dotyczące nadzoru endoskopowego
- Zalecenia dotyczące nadzoru endoskopowego
- Dane epidemiologiczne z różnych krajów
- Grupy specjalne i czynniki ryzyka
- Wyzwania w badaniach epidemiologicznych achalazji
Epidemiologia achalazji
Achalazja jest stosunkowo rzadkim schorzeniem przełyku, charakteryzującym się zaburzeniami motoryki, niedostatecznym rozkurczem dolnego zwieracza przełyku (LES) w odpowiedzi na połykanie oraz brakiem perystaltyki przełyku. Dostępne dane epidemiologiczne wskazują na zróżnicowaną zapadalność na achalazję, wahającą się od 0,03/100 000 osób rocznie w Zimbabwe do 1,63/100 000 osób rocznie w Kanadzie. Większość raportowanych współczynników zapadalności skupia się w przedziale 0,5-1,2/100 000 osób rocznie12. Chorobowość (prevalence) achalazji szacuje się na około 10/100 000 osób34.
Warto zauważyć, że zapadalność na achalazję wydaje się rosnąć w ostatnich dekadach. Dane wskazują, że od lat 80. XX wieku wskaźniki zapadalności przekraczają 0,8/100 000 osób rocznie, a w badaniach po 2000 roku wzrosły dwukrotnie do około 1,6/100 000 osób rocznie56. Nie jest jednak pewne, czy odzwierciedla to rzeczywisty wzrost zachorowań, czy raczej większą świadomość i lepszą diagnostykę schorzenia7.
Chorobowość achalazji również wzrasta. Kanadyjskie badanie populacyjne wykazało, że rozpowszechnienie achalazji wzrosło z 2,51/100 000 w 1996 roku do 10,82/100 000 w 2007 roku, pomimo stosunkowo stabilnej zapadalności w tym samym okresie8. Wzrost chorobowości obserwowano u obu płci, choć był on bardziej wyraźny u mężczyzn, co odzwierciedla fakt, że achalazja jest chorobą o powolnym przebiegu9.
Niedoszacowanie rozpowszechnienia achalazji
Istnieją przesłanki wskazujące, że rzeczywista częstość występowania achalazji może być niedoszacowana. Niektóre badania sugerują, że trudności w identyfikacji pacjentów z achalazją mogą prowadzić do znacznego zaniżenia danych epidemiologicznych10. Niedawne amerykańskie badanie epidemiologiczne, przeprowadzone na obszarach z wysoką dostępnością manometrii wysokiej rozdzielczości (HRM), wykazało chorobowość rzędu 162,1/100 000 i roczną zapadalność 26,0/100 000, co jest przynajmniej 2-3 razy większe niż wcześniejsze szacunki1112.
Warto odnotować, że w badaniu przeprowadzonym w Chicago, przy zastosowaniu manometrii wysokiej rozdzielczości, szacunkowe rozpowszechnienie achalazji osiągnęło wartość około 76 na 100 000 populacji, zbliżając się do 0,1%, co nadal klasyfikuje achalazję jako chorobę rzadką, ale znacznie bardziej rozpowszechnioną niż wcześniej szacowano13.
Rozkład demograficzny achalazji
Achalazja może dotykać osoby obu płci, wszystkich ras i w każdym wieku1415. Choroba dotyczy w równym stopniu kobiet i mężczyzn, choć niektóre badania wykazywały nieco wyższą częstość występowania u kobiet, a jedno badanie wskazało na wyższą zapadalność u mężczyzn161718.
Achalazja może występować w każdym wieku, ale jej początek przed okresem dojrzewania jest rzadki19. Niektóre badania sugerują bimodalny rozkład zapadalności według wieku, z pikami około 30. i 60. roku życia, podczas gdy inne wskazują na ogólnie zwiększone ryzyko achalazji wraz z wiekiem2021. Najczęściej achalazja jest diagnozowana u pacjentów między 25. a 60. rokiem życia2223.
Interesujące różnice w zapadalności na achalazję obserwowano w różnych populacjach. Badanie przeprowadzone przez Mayberry i wsp. wykazało, że achalazja jest znacznie częstsza w Republice Irlandii w porównaniu z sąsiednimi krajami. Podobnie, badanie analizujące zapadalność na achalazję w Nowej Zelandii wykazało różnice między grupami etnicznymi – mieszkańcy wysp Pacyfiku mieli zapadalność 1,3/100 000 osób rocznie w porównaniu do 0,2/100 000 osób rocznie u osób pochodzenia maoryskiego. Może to odzwierciedlać wpływ czynników genetycznych na etiologię achalazji24.
Nadzór i monitorowanie pacjentów z achalazją
Istnieje kontrowersja dotycząca optymalnego leczenia achalazji oraz tego, czy wskazane jest prowadzenie nadzoru (surveillance) w celu wczesnego rozpoznania późnych powikłań2526. Dane z długoterminowej obserwacji pacjentów z achalazją wskazują, że strategie nadzoru mogą być korzystne po czasie trwania choroby dłuższym niż 10-15 lat2728.
Achalazja a ryzyko rozwoju raka przełyku
Achalazja jest uznanym czynnikiem ryzyka rozwoju raka przełyku, jednak dokładna częstość występowania raka przełyku u pacjentów z achalazją jest trudna do ustalenia29. Wiele badań sugeruje związek między rakiem płaskonabłonkowym przełyku a achalazją, z 16- do 28-krotnie zwiększonym ryzykiem rozwoju raka przełyku30.
Mimo że względne ryzyko jest zwiększone, bezwzględne ryzyko raka u pacjentów z achalazją pozostaje niskie, a zapadalność na raka przełyku wynosi około 0,59 na 100 osobolat31. Większość zgonów u pacjentów z achalazją wynika z przyczyn niezwiązanych z tym schorzeniem, co prowadzi do oczekiwanej długości życia równoważnej populacji ogólnej32.
Kontrowersje dotyczące nadzoru endoskopowego
Pomimo dobrze udokumentowanego ryzyka rozwoju raka przełyku u pacjentów z achalazją, najnowsze wytyczne Amerykańskiego Towarzystwa Endoskopii Przewodu Pokarmowego (ASGE) stwierdziły, że rutynowy nadzór endoskopowy jest niewystarczający, ponieważ ogólna bezwzględna liczba zachorowań na raka jest niska – ponad 400 endoskopii byłoby wymaganych do wykrycia jednego nowotworu33.
Mimo braku ustalonego konsensusu dotyczącego nadzoru pacjentów z achalazją, istnieje rosnąca zgoda co do regularnego nadzoru endoskopowego w celu badań przesiewowych w kierunku raka i oceny objawów po leczeniu34. Większość specjalistów nie popiera nadzoru pod kątem raka w achalazji ze względu na bardzo niskie postrzegane ryzyko i doświadczenie anegdotyczne35.
Aktualne badania wskazują, że nadzór endoskopowy może być wskazany u pacjentów z czynnikami wysokiego ryzyka, w tym u mężczyzn, osób powyżej 40. roku życia, z długą historią objawów achalazji (ponad 10 lat), przełykiem Barretta, przewlekłą chorobą refluksową przełyku oraz regularnym spożywaniem alkoholu i paleniem tytoniu36.
Zalecenia dotyczące nadzoru endoskopowego
Chociaż większość wytycznych wskazuje na niewystarczające dowody do poparcia rutynowych badań przesiewowych u pacjentów z achalazją, eksperci sugerują, że nadzór endoskopowy może być rozważany u pacjentów z chorobą trwającą ponad 10 lat, zwłaszcza jeśli jest powiązana z innymi czynnikami ryzyka3738.
Pacjent powinien być poinformowany o ryzyku rozwoju raka przełyku, szczególnie po 10 latach choroby, nawet jeśli jest leczony39. Nadzór może prowadzić do wczesnego rozpoznania nowotworu lub późnych powikłań i powinien być brany pod uwagę po 10-15 latach trwania choroby40.
Endoskopia co trzy lata jest uważana za akceptowalne praktyczne podejście do nadzoru w kierunku raka przełyku w długotrwałej achalazji4142. Nawet jeśli operacja achalazji zwykle poprawia objawy przechodzenia pokarmu, nowotwory przełyku nadal mogą się rozwijać w niektórych przypadkach, a liczba guzów rozwijających się po wielu latach nie jest nieistotna43.
Potrzebne są dalsze badania, aby określić, czy takie strategie nadzoru poprawią ogólne wyniki leczenia44. Istnieje również potrzeba utworzenia krajowej bazy danych w celu zbadania naturalnej historii, długoterminowego zarządzania i efektywności kosztowej strategii nadzoru45.
Dane epidemiologiczne z różnych krajów
Korea Południowa
Badanie przeprowadzone w Korei Południowej zidentyfikowało łącznie 3 105 przypadków achalazji w okresie 5 lat, co daje chorobowość 6,29/100 000 osób. W 2011 roku wykryto 191 nowych przypadków achalazji, co wskazuje na zapadalność 0,39/100 000 w tym roku4647. Zakładając czas trwania choroby dla achalazji na 20 lat, obliczona chorobowość tej choroby w Korei wynosi 7,8/100 000 osób48.
Holandia
Według holenderskich danych ubezpieczenia zdrowotnego z 2018 roku, zapadalność i chorobowość achalazji wynosiły odpowiednio 2,2 na 100 000 populacji rocznie i 15,3 na 100 000 populacji49.
Tajwan
Badanie przeprowadzone na Tajwanie wykazało średnią roczną zapadalność na poziomie 1,64 (95% przedział ufności 1,22-2,05) na 100 000 osób50.
Stany Zjednoczone
W dużym badaniu epidemiologicznym w USA, spośród 36 750 450 osób w bazie danych, zidentyfikowano 19 840 osób z achalazją, co daje ogólną chorobowość 0,05%51. Dodatkowo, badania wykazały wzrost hospitalizacji i związanych z tym kosztów leczenia achalazji w ciągu ostatnich 16 lat w Stanach Zjednoczonych, z nieproporcjonalnym wzrostem u pacjentów poniżej 65 roku życia i mniejszości rasowych52.
Włochy
Retrospektywne długoterminowe badanie przeprowadzone w regionie Veneto we Włoszech wykazało ogólną zapadalność na achalazję wynoszącą 1,59 przypadków/100 000/rok53.
Grupy specjalne i czynniki ryzyka
Występowanie achalazji jest zwiększone u pacjentów z urazem rdzenia kręgowego (SCI)54. W retrospektywnym badaniu (1990-2013) z Holandii, średnia zapadalność na achalazję u dzieci wynosiła 0,1 na 100 000 osób rocznie55. Achalazja u dzieci jest rzadka, ale jej częstość występowania rośnie. Badanie brytyjskie wykazało roczną zapadalność na poziomie 0,18/100 00056.
Etiologia genetyczna jest udokumentowana w części przypadków achalazji. Zespół potrójnego A (choroba Allgrove’a) jest rzadkim schorzeniem, które objawia się achalazją, brakiem łez, niewrażliwością na ACTH i zaburzeniami neurologicznymi. Opisano kilka przypadków rodzinnych. Badano polimorfizmy w genie syntazy tlenku azotu, ale dane są sprzeczne57.
| Kraj/Region | Zapadalność (na 100 000 osób rocznie) | Chorobowość (na 100 000 osób) | Źródło badania |
|---|---|---|---|
| Kanada | 1,63 | 10,82 (2007) | Badanie populacyjne |
| USA (Chicago, z użyciem HRM) | 26,0 | 162,1 | Badanie epidemiologiczne |
| Korea Południowa | 0,39 | 6,29 | Badanie populacyjne |
| Holandia | 2,2 | 15,3 | Dane ubezpieczenia zdrowotnego (2018) |
| Tajwan | 1,64 | Brak danych | Badanie populacyjne |
| Włochy (region Veneto) | 1,59 | Brak danych | Badanie retrospektywne |
| Zimbabwe | 0,03 | Brak danych | Badanie epidemiologiczne |
| USA (ogólna populacja) | Brak danych | 0,05% (50 na 100 000) | Duże badanie epidemiologiczne |
Wyzwania w badaniach epidemiologicznych achalazji
Ze względu na rzadkość choroby, informacje epidemiologiczne na temat achalazji są ograniczone58. Większość badań epidemiologicznych opiera się na danych szpitalnych z lokalnych obszarów, a większość badań pochodzi z krajów zachodnich59.
Kilka czynników utrudnia uzyskanie dokładnych danych dotyczących zapadalności i chorobowości achalazji60. Dokładne oszacowanie zapadalności i chorobowości achalazji, zdefiniowanej przez HRM, wymagałoby bardzo dużego populacyjnego badania z wykorzystaniem manometrii wysokiej rozdzielczości61.
Biorąc pod uwagę słabą penetrację najbardziej zaawansowanych kryteriów diagnostycznych i technologii, można bezpiecznie stwierdzić, że rzeczywista liczba pacjentów z achalazją na całym świecie pozostaje nieznana62. Mimo tych wyzwań, dostęp do dobrze zorganizowanych krajowych systemów ubezpieczenia zdrowotnego, jak w przypadku Korei, może dać kilka korzyści w badaniu epidemiologii achalazji63.
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Materiały źródłowe
- #1 Achalasia: A review of clinical diagnosis, epidemiology, treatment and outcomeshttps://pmc.ncbi.nlm.nih.gov/articles/PMC3793135/
Achalasia is a relatively rare condition. A summary of studies published to date on achalasia incidence and prevalence is shown in Table 1. The incidence of achalasia varied between studies, with reports as low as 0.03/100000 per year in Zimbabwe to 1.63/100000 per year in Canada. The majority of incidence rates reported clustered between 0.5-1.2 per 100000/year. In an attempt to investigate changing incidence rates over time, we plotted incidence rates against the mid-timepoint within the study periods. As shown in Figure 3, incidence rates of achalasia appear to be rising, with most reports since the 1980s exceeding rates of 0.8/100000 per year, which has doubled to 1.6/100000 per year in post-2000 studies. Whether this reflects a true rise in incidence, or greater awareness and improved diagnosis of the condition remains uncertain though.
- #2 Achalasia: A review of clinical diagnosis, epidemiology, treatment and outcomeshttps://www.wjgnet.com/1007-9327/full/v19/i35/5806.htm
Achalasia is a relatively rare condition. […] The incidence of achalasia varied between studies, with reports as low as 0.03/100000 per year in Zimbabwe to 1.63/100000 per year in Canada. […] The majority of incidence rates reported clustered between 0.5-1.2 per 100000/year. […] As shown in Figure 3, incidence rates of achalasia appear to be rising, with most reports since the 1980s exceeding rates of 0.8/100000 per year, which has doubled to 1.6/100000 per year in post-2000 studies. […] There are no distinct patterns of achalasia incidence in terms of age and sex distribution; it can affect both genders, all races and all ages. […] A few studies have suggested a bimodal distribution of incidence by age, with peaks at around age 30 and 60 years, while others have pointed towards a generally increased risk of achalasia with increased age.
- #3 Achalasia – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK519515/
Achalasia is very rare, occurring with an annual incidence of roughly one per 100000 people and a prevalence of 10 per 100000. […] However, a recent study showed increasing hospitalizations and associated costs for achalasia over the last 16 years in the United States with a disproportionate increase in patients under 65 years of age and racial minorities. […] Achalasia occurs with equal frequency in both males and females. The disorder typically affects people between the second to the fifth decade of life with a peak incidence between the ages of 30 to 60 years. […] Outside the United States, rates of achalasia vary from 0.1 to 1 per every 100000 people per year. […] Studies show that relapse rates are higher if the initial treatment was pneumatic dilatation. However, complications have been noted to be much higher in patients who underwent a Heller myotomy compared to those who underwent pneumatic dilatation.
- #4 Achalasia: Pathogenesis, clinical manifestations, and diagnosis – UpToDatehttps://www.uptodate.com/contents/achalasia-pathogenesis-clinical-manifestations-and-diagnosis
Achalasia has been regarded as an uncommon disorder with an annual incidence of approximately 1.6 cases per 100,000 individuals and prevalence of 10 cases per 100,000 individuals. […] Although epidemiologic data on achalasia are limited, its frequency appears to be rising, with one study suggesting that, from 2004 to 2014, the incidence and prevalence of achalasia in central Chicago were two- to threefold greater than estimates from earlier years would have predicted. […] Males and females are affected with equal frequency. The disease can occur at any age, but onset before adolescence is rare. Achalasia is usually diagnosed in patients between the ages of 25 and 60 years.
- #5 Achalasia: A review of clinical diagnosis, epidemiology, treatment and outcomeshttps://pmc.ncbi.nlm.nih.gov/articles/PMC3793135/
Achalasia is a relatively rare condition. A summary of studies published to date on achalasia incidence and prevalence is shown in Table 1. The incidence of achalasia varied between studies, with reports as low as 0.03/100000 per year in Zimbabwe to 1.63/100000 per year in Canada. The majority of incidence rates reported clustered between 0.5-1.2 per 100000/year. In an attempt to investigate changing incidence rates over time, we plotted incidence rates against the mid-timepoint within the study periods. As shown in Figure 3, incidence rates of achalasia appear to be rising, with most reports since the 1980s exceeding rates of 0.8/100000 per year, which has doubled to 1.6/100000 per year in post-2000 studies. Whether this reflects a true rise in incidence, or greater awareness and improved diagnosis of the condition remains uncertain though.
- #6 Achalasia: A review of clinical diagnosis, epidemiology, treatment and outcomeshttps://www.wjgnet.com/1007-9327/full/v19/i35/5806.htm
Achalasia is a relatively rare condition. […] The incidence of achalasia varied between studies, with reports as low as 0.03/100000 per year in Zimbabwe to 1.63/100000 per year in Canada. […] The majority of incidence rates reported clustered between 0.5-1.2 per 100000/year. […] As shown in Figure 3, incidence rates of achalasia appear to be rising, with most reports since the 1980s exceeding rates of 0.8/100000 per year, which has doubled to 1.6/100000 per year in post-2000 studies. […] There are no distinct patterns of achalasia incidence in terms of age and sex distribution; it can affect both genders, all races and all ages. […] A few studies have suggested a bimodal distribution of incidence by age, with peaks at around age 30 and 60 years, while others have pointed towards a generally increased risk of achalasia with increased age.
- #7 Achalasia: A review of clinical diagnosis, epidemiology, treatment and outcomeshttps://pmc.ncbi.nlm.nih.gov/articles/PMC3793135/
Achalasia is a relatively rare condition. A summary of studies published to date on achalasia incidence and prevalence is shown in Table 1. The incidence of achalasia varied between studies, with reports as low as 0.03/100000 per year in Zimbabwe to 1.63/100000 per year in Canada. The majority of incidence rates reported clustered between 0.5-1.2 per 100000/year. In an attempt to investigate changing incidence rates over time, we plotted incidence rates against the mid-timepoint within the study periods. As shown in Figure 3, incidence rates of achalasia appear to be rising, with most reports since the 1980s exceeding rates of 0.8/100000 per year, which has doubled to 1.6/100000 per year in post-2000 studies. Whether this reflects a true rise in incidence, or greater awareness and improved diagnosis of the condition remains uncertain though.
- #8 Achalasia: A review of clinical diagnosis, epidemiology, treatment and outcomeshttps://pmc.ncbi.nlm.nih.gov/articles/PMC3793135/
A Canadian population-based study also considered the prevalence and survival rates of patients with achalasia. Sadowski et al found that the prevalence of achalasia rose from 2.51/100000 in 1996 to 10.82/100000 in 2007, despite a relatively stable incidence over the same time period. The rise in prevalence was seen in both genders but was noted to be more pronounced in males, reflecting the fact that achalasia is a slowly progressive disease. This rise in prevalence was also evident in an Israeli study and was noted in an Icelandic study between 1952 and 2002. It is interesting to note that the Canadian study observed survival of achalasia patients to be significantly lower than the age-sex matched control population. However, others have discerned that the majority of deaths in achalasia patients result from unrelated causes, leading to an equivalent life expectancy to the general population.
- #9 Achalasia: A review of clinical diagnosis, epidemiology, treatment and outcomeshttps://www.wjgnet.com/1007-9327/full/v19/i35/5806.htm
A Canadian population-based study also considered the prevalence and survival rates of patients with achalasia. […] The rise in prevalence was seen in both genders but was noted to be more pronounced in males, reflecting the fact that achalasia is a slowly progressive disease. […] However, others have discerned that the majority of deaths in achalasia patients result from unrelated causes, leading to an equivalent life expectancy to the general population. […] Despite the relative risk being increased, the absolute risk of cancer in patients with achalasia is still small and there would be a large number of examinations required to detect a single cancer. […] The most recent guidelines indicate that surveillance endoscopy is not indicated.
- #10 Achalasia: The Current Clinical Dilemma and Possible Pathogenesishttps://www.jnmjournal.org/view.html?uid=1836&vmd=Full
Achalasia used to be considered as a rare motility disease, with an annual incidence and prevalence of 1.63/100 000 and 10.82/100 000, respectively. However, the data might be significantly underestimated due to difficulties in identifying patients with achalasia. […] A recent American epidemiological survey conducted in areas with a high prevalence of high-resolution manometry (HRM) found that the prevalence and annual incidence of achalasia were 162.1/100 000 and 26.0/100 000 respectively, which were at least 2- to 3-fold greater than previously estimated. […] Therefore, given the poor penetration of the most advanced diagnostic criteria and technology, it is safe to say that the actual number of achalasia patients worldwide remains unknown.
- #11 Achalasia: The Current Clinical Dilemma and Possible Pathogenesishttps://www.jnmjournal.org/view.html?uid=1836&vmd=Full
Achalasia used to be considered as a rare motility disease, with an annual incidence and prevalence of 1.63/100 000 and 10.82/100 000, respectively. However, the data might be significantly underestimated due to difficulties in identifying patients with achalasia. […] A recent American epidemiological survey conducted in areas with a high prevalence of high-resolution manometry (HRM) found that the prevalence and annual incidence of achalasia were 162.1/100 000 and 26.0/100 000 respectively, which were at least 2- to 3-fold greater than previously estimated. […] Therefore, given the poor penetration of the most advanced diagnostic criteria and technology, it is safe to say that the actual number of achalasia patients worldwide remains unknown.
- #12 Advances in the management of oesophageal motility disorders in the era of high-resolution manometry: a focus on achalasia syndromes | Nature Reviews Gastroenterology & Hepatologyhttps://www.nature.com/articles/nrgastro.2017.132
A common observation among early adopters of HRM was increased detection of achalasia, leading to the suspicion that the disease was more common than previously reported. […] Several factors make it difficult to obtain accurate figures on the incidence and prevalence of achalasia. […] Consequently, available estimates, reporting annual incidence and prevalence values of about 1 per 100,000 and 10 per 100,000, respectively, are based on the assumption that all cases are detected. […] An accurate estimate of the incidence and prevalence of achalasia, as defined by HRM, would require a very large population-based HRM study. […] Nonetheless, the estimated incidence of achalasia averaged 2.92 per 100,000 during a 10-year period and the prevalence progressively increased from 15.64 to 32.58 per 100,000 over the same period, with no plateau evident.
- #13 Advances in the management of oesophageal motility disorders in the era of high-resolution manometry: a focus on achalasia syndromes | Nature Reviews Gastroenterology & Hepatologyhttps://www.nature.com/articles/nrgastro.2017.132
Projecting those prevalence estimates forward, and assuming an average life expectance of 82 years in that part of Chicago, achalasia prevalence is anticipated to stabilize at a value about 26 times the incidence or, in this case, at 76 per 100,000 population, approaching 0.1%. […] At this estimated prevalence, achalasia is still a rare disease, but is substantially more prevalent than the prior estimates of 10 per 100,000.
- #14 Achalasia: A review of clinical diagnosis, epidemiology, treatment and outcomeshttps://pmc.ncbi.nlm.nih.gov/articles/PMC3793135/
There are no distinct patterns of achalasia incidence in terms of age and sex distribution; it can affect both genders, all races and all ages. A few studies have suggested a bimodal distribution of incidence by age, with peaks at around age 30 and 60 years, while others have pointed towards a generally increased risk of achalasia with increased age. Achalasia appears to affect males and females to largely equal extents although some investigations have detected slightly higher rates amongst females. Only one study reported a higher achalasia incidence in men. A study carried out by Mayberry et al found achalasia to be significantly more common in the Republic of Ireland in comparison to its neighbouring countries. Similarly, a study which reviewed the incidence of achalasia in New Zealand found differing incidence between ethnic groups. The Pacific Islanders had an incidence of 1.3/100000 per year in comparison to those of Maori descent having an incidence of 0.2/100000 per year. This may reflect the influence of genetic factors in achalasia aetiology.
- #15 Achalasia: A review of clinical diagnosis, epidemiology, treatment and outcomeshttps://www.wjgnet.com/1007-9327/full/v19/i35/5806.htm
Achalasia is a relatively rare condition. […] The incidence of achalasia varied between studies, with reports as low as 0.03/100000 per year in Zimbabwe to 1.63/100000 per year in Canada. […] The majority of incidence rates reported clustered between 0.5-1.2 per 100000/year. […] As shown in Figure 3, incidence rates of achalasia appear to be rising, with most reports since the 1980s exceeding rates of 0.8/100000 per year, which has doubled to 1.6/100000 per year in post-2000 studies. […] There are no distinct patterns of achalasia incidence in terms of age and sex distribution; it can affect both genders, all races and all ages. […] A few studies have suggested a bimodal distribution of incidence by age, with peaks at around age 30 and 60 years, while others have pointed towards a generally increased risk of achalasia with increased age.
- #16 Achalasia: A review of clinical diagnosis, epidemiology, treatment and outcomeshttps://pmc.ncbi.nlm.nih.gov/articles/PMC3793135/
There are no distinct patterns of achalasia incidence in terms of age and sex distribution; it can affect both genders, all races and all ages. A few studies have suggested a bimodal distribution of incidence by age, with peaks at around age 30 and 60 years, while others have pointed towards a generally increased risk of achalasia with increased age. Achalasia appears to affect males and females to largely equal extents although some investigations have detected slightly higher rates amongst females. Only one study reported a higher achalasia incidence in men. A study carried out by Mayberry et al found achalasia to be significantly more common in the Republic of Ireland in comparison to its neighbouring countries. Similarly, a study which reviewed the incidence of achalasia in New Zealand found differing incidence between ethnic groups. The Pacific Islanders had an incidence of 1.3/100000 per year in comparison to those of Maori descent having an incidence of 0.2/100000 per year. This may reflect the influence of genetic factors in achalasia aetiology.
- #17 Achalasia – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK519515/
Achalasia is very rare, occurring with an annual incidence of roughly one per 100000 people and a prevalence of 10 per 100000. […] However, a recent study showed increasing hospitalizations and associated costs for achalasia over the last 16 years in the United States with a disproportionate increase in patients under 65 years of age and racial minorities. […] Achalasia occurs with equal frequency in both males and females. The disorder typically affects people between the second to the fifth decade of life with a peak incidence between the ages of 30 to 60 years. […] Outside the United States, rates of achalasia vary from 0.1 to 1 per every 100000 people per year. […] Studies show that relapse rates are higher if the initial treatment was pneumatic dilatation. However, complications have been noted to be much higher in patients who underwent a Heller myotomy compared to those who underwent pneumatic dilatation.
- #18 Achalasia: Pathogenesis, clinical manifestations, and diagnosis – UpToDatehttps://www.uptodate.com/contents/achalasia-pathogenesis-clinical-manifestations-and-diagnosis
Achalasia has been regarded as an uncommon disorder with an annual incidence of approximately 1.6 cases per 100,000 individuals and prevalence of 10 cases per 100,000 individuals. […] Although epidemiologic data on achalasia are limited, its frequency appears to be rising, with one study suggesting that, from 2004 to 2014, the incidence and prevalence of achalasia in central Chicago were two- to threefold greater than estimates from earlier years would have predicted. […] Males and females are affected with equal frequency. The disease can occur at any age, but onset before adolescence is rare. Achalasia is usually diagnosed in patients between the ages of 25 and 60 years.
- #19 Achalasia: Pathogenesis, clinical manifestations, and diagnosis – UpToDatehttps://www.uptodate.com/contents/achalasia-pathogenesis-clinical-manifestations-and-diagnosis
Achalasia has been regarded as an uncommon disorder with an annual incidence of approximately 1.6 cases per 100,000 individuals and prevalence of 10 cases per 100,000 individuals. […] Although epidemiologic data on achalasia are limited, its frequency appears to be rising, with one study suggesting that, from 2004 to 2014, the incidence and prevalence of achalasia in central Chicago were two- to threefold greater than estimates from earlier years would have predicted. […] Males and females are affected with equal frequency. The disease can occur at any age, but onset before adolescence is rare. Achalasia is usually diagnosed in patients between the ages of 25 and 60 years.
- #20 Achalasia: A review of clinical diagnosis, epidemiology, treatment and outcomeshttps://pmc.ncbi.nlm.nih.gov/articles/PMC3793135/
There are no distinct patterns of achalasia incidence in terms of age and sex distribution; it can affect both genders, all races and all ages. A few studies have suggested a bimodal distribution of incidence by age, with peaks at around age 30 and 60 years, while others have pointed towards a generally increased risk of achalasia with increased age. Achalasia appears to affect males and females to largely equal extents although some investigations have detected slightly higher rates amongst females. Only one study reported a higher achalasia incidence in men. A study carried out by Mayberry et al found achalasia to be significantly more common in the Republic of Ireland in comparison to its neighbouring countries. Similarly, a study which reviewed the incidence of achalasia in New Zealand found differing incidence between ethnic groups. The Pacific Islanders had an incidence of 1.3/100000 per year in comparison to those of Maori descent having an incidence of 0.2/100000 per year. This may reflect the influence of genetic factors in achalasia aetiology.
- #21 Achalasia: A review of clinical diagnosis, epidemiology, treatment and outcomeshttps://www.wjgnet.com/1007-9327/full/v19/i35/5806.htm
Achalasia is a relatively rare condition. […] The incidence of achalasia varied between studies, with reports as low as 0.03/100000 per year in Zimbabwe to 1.63/100000 per year in Canada. […] The majority of incidence rates reported clustered between 0.5-1.2 per 100000/year. […] As shown in Figure 3, incidence rates of achalasia appear to be rising, with most reports since the 1980s exceeding rates of 0.8/100000 per year, which has doubled to 1.6/100000 per year in post-2000 studies. […] There are no distinct patterns of achalasia incidence in terms of age and sex distribution; it can affect both genders, all races and all ages. […] A few studies have suggested a bimodal distribution of incidence by age, with peaks at around age 30 and 60 years, while others have pointed towards a generally increased risk of achalasia with increased age.
- #22 Achalasia: Pathogenesis, clinical manifestations, and diagnosis – UpToDatehttps://www.uptodate.com/contents/achalasia-pathogenesis-clinical-manifestations-and-diagnosis
Achalasia has been regarded as an uncommon disorder with an annual incidence of approximately 1.6 cases per 100,000 individuals and prevalence of 10 cases per 100,000 individuals. […] Although epidemiologic data on achalasia are limited, its frequency appears to be rising, with one study suggesting that, from 2004 to 2014, the incidence and prevalence of achalasia in central Chicago were two- to threefold greater than estimates from earlier years would have predicted. […] Males and females are affected with equal frequency. The disease can occur at any age, but onset before adolescence is rare. Achalasia is usually diagnosed in patients between the ages of 25 and 60 years.
- #23 Achalasia – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK519515/
Achalasia is very rare, occurring with an annual incidence of roughly one per 100000 people and a prevalence of 10 per 100000. […] However, a recent study showed increasing hospitalizations and associated costs for achalasia over the last 16 years in the United States with a disproportionate increase in patients under 65 years of age and racial minorities. […] Achalasia occurs with equal frequency in both males and females. The disorder typically affects people between the second to the fifth decade of life with a peak incidence between the ages of 30 to 60 years. […] Outside the United States, rates of achalasia vary from 0.1 to 1 per every 100000 people per year. […] Studies show that relapse rates are higher if the initial treatment was pneumatic dilatation. However, complications have been noted to be much higher in patients who underwent a Heller myotomy compared to those who underwent pneumatic dilatation.
- #24 Achalasia: A review of clinical diagnosis, epidemiology, treatment and outcomeshttps://pmc.ncbi.nlm.nih.gov/articles/PMC3793135/
There are no distinct patterns of achalasia incidence in terms of age and sex distribution; it can affect both genders, all races and all ages. A few studies have suggested a bimodal distribution of incidence by age, with peaks at around age 30 and 60 years, while others have pointed towards a generally increased risk of achalasia with increased age. Achalasia appears to affect males and females to largely equal extents although some investigations have detected slightly higher rates amongst females. Only one study reported a higher achalasia incidence in men. A study carried out by Mayberry et al found achalasia to be significantly more common in the Republic of Ireland in comparison to its neighbouring countries. Similarly, a study which reviewed the incidence of achalasia in New Zealand found differing incidence between ethnic groups. The Pacific Islanders had an incidence of 1.3/100000 per year in comparison to those of Maori descent having an incidence of 0.2/100000 per year. This may reflect the influence of genetic factors in achalasia aetiology.
- #25 Treatment and surveillance strategies in achalasia: an update | Nature Reviews Gastroenterology & Hepatologyhttps://www.nature.com/articles/nrgastro.2011.68
Controversy exists with regard to the optimal treatment for achalasia and whether surveillance for early recognition of late complications is indicated. […] Finally, long-term follow-up data in patients with achalasia support the notion that surveillance strategies might be beneficial after a disease duration of more than 10-15 years. […] Surveillance may lead to early recognition of cancer or late complications and should be considered after a disease duration of 10-15 years.
- #26 Treatment and surveillance strategies in achalasia: an update. – Document – Gale Academic OneFilehttps://go.gale.com/ps/i.do?id=GALE%7CA259078543&sid=googleScholar&v=2.1&it=r&linkaccess=abs&issn=17595045&p=AONE&sw=w
Controversy exists with regard to the optimal treatment for achalasia and whether surveillance for early recognition of late complications is indicated. […] Finally, long-term follow-up data in patients with achalasia support the notion that surveillance strategies might be beneficial after a disease duration of more than 10-15 years.
- #27 Treatment and surveillance strategies in achalasia: an update | Nature Reviews Gastroenterology & Hepatologyhttps://www.nature.com/articles/nrgastro.2011.68
Controversy exists with regard to the optimal treatment for achalasia and whether surveillance for early recognition of late complications is indicated. […] Finally, long-term follow-up data in patients with achalasia support the notion that surveillance strategies might be beneficial after a disease duration of more than 10-15 years. […] Surveillance may lead to early recognition of cancer or late complications and should be considered after a disease duration of 10-15 years.
- #28 Treatment and surveillance strategies in achalasia: an update. – Document – Gale Academic OneFilehttps://go.gale.com/ps/i.do?id=GALE%7CA259078543&sid=googleScholar&v=2.1&it=r&linkaccess=abs&issn=17595045&p=AONE&sw=w
Controversy exists with regard to the optimal treatment for achalasia and whether surveillance for early recognition of late complications is indicated. […] Finally, long-term follow-up data in patients with achalasia support the notion that surveillance strategies might be beneficial after a disease duration of more than 10-15 years.
- #29 Achalasia and cancer prevention and surveillance – Del Grande – Annals of Esophagushttps://aoe.amegroups.org/article/view/5391/html
Achalasia is a well-known risk factor for esophageal cancer (EC). However, the incidence of EC in patients with achalasia is elusive. […] The patient must be informed about the risk of EC especially after 10 years, even treated. […] Surveillance may be considered in patients with more than 10 years of disease, especially if associated with other risk factors.
- #30 Role of endoscopy in patients with achalasiahttps://www.e-ce.org/journal/view.php?number=7793
Many studies have suggested an association between esophageal squamous cell carcinoma (SCC) and achalasia, with 16- to 28-fold increased risks of esophageal carcinoma. […] Although remains no established consensus regarding the surveillance of patients with achalasia, there is a growing consensus regarding regular endoscopic surveillance for cancer screening and posttreatment symptom assessment.
- #31https://link.springer.com/article/10.1007/s00535-022-01916-1
Achalasia is a primary esophageal motility disorder, and it has been reported to be associated with ECs. However, details of achalasia-related ECs are not well investigated since achalasia is a rare disease with unknown etiology. […] Although the relative risk of ECs in achalasia patients is considered higher, the absolute risk is still low with the incidence of EC about 0.59 per 100 person-year. Therefore, many guidelines reported insufficient evidence to support routine screening in achalasia patients. […] This article by Sato H et al. pointed that endoscopic surveillance is important in patients with a long history of achalasia, advanced age (more than 40 years), male sex, and regular alcohol intake. […] Therefore, based on the current evidence, we suggest routine endoscopic surveillance for ECs in achalasia patients in high-risk factors, including male sex, 40 years of age, with a long history of achalasia symptoms onset over 10 years, BE, with chronic GERD, and regular alcohol intake and smoking.
- #32 Achalasia: A review of clinical diagnosis, epidemiology, treatment and outcomeshttps://www.wjgnet.com/1007-9327/full/v19/i35/5806.htm
A Canadian population-based study also considered the prevalence and survival rates of patients with achalasia. […] The rise in prevalence was seen in both genders but was noted to be more pronounced in males, reflecting the fact that achalasia is a slowly progressive disease. […] However, others have discerned that the majority of deaths in achalasia patients result from unrelated causes, leading to an equivalent life expectancy to the general population. […] Despite the relative risk being increased, the absolute risk of cancer in patients with achalasia is still small and there would be a large number of examinations required to detect a single cancer. […] The most recent guidelines indicate that surveillance endoscopy is not indicated.
- #33 Role of endoscopy in patients with achalasiahttps://www.e-ce.org/journal/view.php?number=7793
Achalasia is an esophageal motility disorder characterized by impaired lower esophageal sphincter relaxation and peristalsis of the esophageal body. […] With the increasing prevalence of achalasia, interest in the role of endoscopy in its diagnosis, treatment, and monitoring is also growing. […] The reported incidence and prevalence of achalasia vary by studies, with an annual incidence of 0.39 to 1.63 individuals per 100,000 and a prevalence of 7.8 to 10.82 per 100,000. […] Recent studies have noted an increase in the overall prevalence, probably due to the chronic nature of the disease, increased awareness, and improvements in diagnostic methods, especially in the application of high-resolution manometry (HRM). […] Despite the well-documented risk of esophageal cancer in patients with achalasia, the most recent American Society of Gastrointestinal Endoscopy guidelines concluded that routine endoscopic surveillance is insufficient because the overall absolute number of cancer incidences is low, as more than 400 endoscopies are required to detect one cancer.
- #34 Role of endoscopy in patients with achalasiahttps://www.e-ce.org/journal/view.php?number=7793
Many studies have suggested an association between esophageal squamous cell carcinoma (SCC) and achalasia, with 16- to 28-fold increased risks of esophageal carcinoma. […] Although remains no established consensus regarding the surveillance of patients with achalasia, there is a growing consensus regarding regular endoscopic surveillance for cancer screening and posttreatment symptom assessment.
- #35 PTU-156â Assessing Current UK Practice for Endoscopic Surveillance in Achalasia | Guthttps://gut.bmj.com/content/65/Suppl_1/A134.1
There are no established guidelines on achalasia surveillance from the British Society of Gastroenterology(BSG)or the American Society for Gastrointestinal Endoscopy(ASGE). […] The aim of this study was to document current clinical practice among UK endoscopists regarding achalasia surveillance. […] Despite the high levels of interest, 79% of the respondents did not undertake cancer surveillance in achalasia. […] The majority of specialists do not endorse cancer surveillance in achalasia based on a very low perceived risk and anecdotal experience. […] There is a need for establishing a national database to study the natural history, long-term management and cost-effectiveness of surveillance. […] It would be helpful for the BSG to recommend against surveillance, highlighting the lack of sufficient evidence and enthusiasm as judged by current clinical practice.
- #36https://link.springer.com/article/10.1007/s00535-022-01916-1
Achalasia is a primary esophageal motility disorder, and it has been reported to be associated with ECs. However, details of achalasia-related ECs are not well investigated since achalasia is a rare disease with unknown etiology. […] Although the relative risk of ECs in achalasia patients is considered higher, the absolute risk is still low with the incidence of EC about 0.59 per 100 person-year. Therefore, many guidelines reported insufficient evidence to support routine screening in achalasia patients. […] This article by Sato H et al. pointed that endoscopic surveillance is important in patients with a long history of achalasia, advanced age (more than 40 years), male sex, and regular alcohol intake. […] Therefore, based on the current evidence, we suggest routine endoscopic surveillance for ECs in achalasia patients in high-risk factors, including male sex, 40 years of age, with a long history of achalasia symptoms onset over 10 years, BE, with chronic GERD, and regular alcohol intake and smoking.
- #37 Achalasia and cancer prevention and surveillance – Del Grande – Annals of Esophagushttps://aoe.amegroups.org/article/view/5391/html
Achalasia is a well-known risk factor for esophageal cancer (EC). However, the incidence of EC in patients with achalasia is elusive. […] The patient must be informed about the risk of EC especially after 10 years, even treated. […] Surveillance may be considered in patients with more than 10 years of disease, especially if associated with other risk factors.
- #38 Diagnosis and Management of Achalasia: Updates of the Last Two Yearshttps://www.mdpi.com/2077-0383/10/16/3607
Achalasia is a rare disease, with an estimated incidence of 0.03 to 1.63 per 100,000 persons per year and a prevalence of 10 per 100,000. […] When successfully treated, the quality of life almost returns to near normal for a long time; on the other hand, when untreated, the course is usually progressive, leading to esophageal lumen dilatation, which, over time, leads to a burned-out, decompensated sigmoid esophagus with its clinical related consequences, including malnutrition. […] Longstanding achalasia is a significant risk factor for esophageal adenocarcinoma (50 folds) and esophageal squamous cell carcinoma, even when achalasia is adequately managed. […] Nonetheless, no formal practical guidelines recommend endoscopic surveillance in achalasia patients. However, an endoscopy every three years is considered an acceptable practical surveillance approach for esophageal cancer in longstanding achalasia. […] Therefore, continuing endoscopic surveillance is required for the detection of malignancy at an early stage.
- #39 Achalasia and cancer prevention and surveillance – Del Grande – Annals of Esophagushttps://aoe.amegroups.org/article/view/5391/html
Achalasia is a well-known risk factor for esophageal cancer (EC). However, the incidence of EC in patients with achalasia is elusive. […] The patient must be informed about the risk of EC especially after 10 years, even treated. […] Surveillance may be considered in patients with more than 10 years of disease, especially if associated with other risk factors.
- #40 Treatment and surveillance strategies in achalasia: an update | Nature Reviews Gastroenterology & Hepatologyhttps://www.nature.com/articles/nrgastro.2011.68
Controversy exists with regard to the optimal treatment for achalasia and whether surveillance for early recognition of late complications is indicated. […] Finally, long-term follow-up data in patients with achalasia support the notion that surveillance strategies might be beneficial after a disease duration of more than 10-15 years. […] Surveillance may lead to early recognition of cancer or late complications and should be considered after a disease duration of 10-15 years.
- #41 Diagnosis and Management of Achalasia: Updates of the Last Two Yearshttps://www.mdpi.com/2077-0383/10/16/3607
Achalasia is a rare disease, with an estimated incidence of 0.03 to 1.63 per 100,000 persons per year and a prevalence of 10 per 100,000. […] When successfully treated, the quality of life almost returns to near normal for a long time; on the other hand, when untreated, the course is usually progressive, leading to esophageal lumen dilatation, which, over time, leads to a burned-out, decompensated sigmoid esophagus with its clinical related consequences, including malnutrition. […] Longstanding achalasia is a significant risk factor for esophageal adenocarcinoma (50 folds) and esophageal squamous cell carcinoma, even when achalasia is adequately managed. […] Nonetheless, no formal practical guidelines recommend endoscopic surveillance in achalasia patients. However, an endoscopy every three years is considered an acceptable practical surveillance approach for esophageal cancer in longstanding achalasia. […] Therefore, continuing endoscopic surveillance is required for the detection of malignancy at an early stage.
- #42 Achalasia | 5-Minute Clinical Consulthttps://www.unboundmedicine.com/5minute/view/5-Minute-Clinical-Consult/816837/all/Achalasia
Incidence: 1 to 2/100,000 population/year (1) […] Age of peak incidence: 30 to 60 years […] Prevalence: 10/100,000 population (2) […] Male = female […] No racial or ethnic preference […] American Society for Gastrointestinal Endoscopy (ASGE) favors radiographic or endoscopic surveillance for cancer every 3 years if achalasia has been present for 10 to 15 years (3)[C].
- #43 Achalasia and esophageal cancer: risks and links | CEGhttps://www.dovepress.com/achalasia-and-esophageal-cancer-risks-and-links-peer-reviewed-fulltext-article-CEG
Once the risks and links between ESCC/EA and achalasia are established, it is logical to think of strategies to prevent the development of those neoplasias. Although a regular surveillance program for esophageal cancer as standard practice in patients with achalasia is controversial, clinicians who treat patients with achalasia should be keenly aware of the association to detect cancer in its early stages. […] Even though surgical treatment for achalasia has been successful, the risk of ESCC and EA can persist, raising the question of whether patients who have been operated on for achalasia should still be in a surveillance program. Therefore, it is suggested that even though surgery for achalasia usually improves passage symptoms, esophageal cancer still arises in some cases, and the number of tumors developing after many years is not negligible.
- #44 Endoscopic approach to achalasiahttps://www.wjgnet.com/1948-5190/full/v5/i8/379.htm
Even if the latest American Society of Gastrointestinal Endoscopy guidelines correctly state that there are still insufficient data to support routine endoscopic (cancer) surveillance for patients with achalasia, endoscopic surveillance might be beneficial in particular if one considers that cancer is not the only late complication of this disease. Therefore, most experts favor some form of endoscopic surveillance in patients with achalasia if the disease has been present for more than 10-15 years. […] However, further studies are needed to determine whether such surveillance strategies will improve the overall outcome.
- #45 PTU-156â Assessing Current UK Practice for Endoscopic Surveillance in Achalasia | Guthttps://gut.bmj.com/content/65/Suppl_1/A134.1
There are no established guidelines on achalasia surveillance from the British Society of Gastroenterology(BSG)or the American Society for Gastrointestinal Endoscopy(ASGE). […] The aim of this study was to document current clinical practice among UK endoscopists regarding achalasia surveillance. […] Despite the high levels of interest, 79% of the respondents did not undertake cancer surveillance in achalasia. […] The majority of specialists do not endorse cancer surveillance in achalasia based on a very low perceived risk and anecdotal experience. […] There is a need for establishing a national database to study the natural history, long-term management and cost-effectiveness of surveillance. […] It would be helpful for the BSG to recommend against surveillance, highlighting the lack of sufficient evidence and enthusiasm as judged by current clinical practice.
- #46 :: JKMS :: Journal of Korean Medical Sciencehttps://jkms.org/DOIx.php?id=10.3346/jkms.2014.29.4.576
Owing to the rarity of the disease, epidemiologic information on achalasia is limited. […] This study aimed to investigate the epidemiology and treatment patterns of achalasia in the population of Korea using a national healthcare database. […] A total of 3,105 patients with achalasia (1,447 men; mean age, 52.5 yr) were identified between 2007 and 2011, indicating a prevalence of 6.29/100,000 (95% confidence interval [CI], 4.94-7.66) during this 5-yr period. […] A total of 191 incident cases of achalasia (82 men; mean age, 49.5 yr), which were not diagnosed as achalasia in the previous 4 yr, were detected in 2011, indicating an incidence of 0.39/100,000 (95% CI, 0.15-0.63) for that year. […] This is the first population-based epidemiologic study of achalasia in Korea. […] The present study is the first epidemiologic study of achalasia in the general population of Korea, revealing the incidence of achalasia in Korea to be 0.39/100,000/yr.
- #47 :: JKMS :: Journal of Korean Medical Sciencehttps://jkms.org/DOIx.php?id=10.3346/jkms.2014.29.4.576
We identified 3,105 cases of achalasia during the 5-yr period of the study, leading to a prevalence of 6.29/100,000 persons. […] Assuming the duration of disease for achalasia is 20 yr and its incidence is 0.39/100,000/yr, the prevalence of this disease in Korea is calculated to be 7.8/100,000 persons. […] The epidemiologic studies reported are based on hospital records from local areas, and most of the studies were from Western countries. […] The source of the current study’s data, Korea’s accessible, well-organized national health insurance system’s database, conferred several advantages. […] In conclusion, this is the first population-based epidemiologic study of achalasia in Korea, revealing its incidence, estimated prevalence, demographic features, and treatment patterns.
- #48 :: JKMS :: Journal of Korean Medical Sciencehttps://jkms.org/DOIx.php?id=10.3346/jkms.2014.29.4.576
We identified 3,105 cases of achalasia during the 5-yr period of the study, leading to a prevalence of 6.29/100,000 persons. […] Assuming the duration of disease for achalasia is 20 yr and its incidence is 0.39/100,000/yr, the prevalence of this disease in Korea is calculated to be 7.8/100,000 persons. […] The epidemiologic studies reported are based on hospital records from local areas, and most of the studies were from Western countries. […] The source of the current study’s data, Korea’s accessible, well-organized national health insurance system’s database, conferred several advantages. […] In conclusion, this is the first population-based epidemiologic study of achalasia in Korea, revealing its incidence, estimated prevalence, demographic features, and treatment patterns.
- #49 Achalasia Cardia: A Comprehensive Review | EMJ Reviewshttps://www.emjreviews.com/gastroenterology/article/achalasia-cardia-a-comprehensive-review/
Achalasia is equally common in both sexes. Most commonly diagnosed between 40 and 60 years of age, achalasia can present in any age group. Most of the epidemiological data is derived from retrospective studies as population-based studies are scarce because of the rarity of achalasia. Because of its rarity and chronicity, the prevalence is much higher than the incidence of achalasia. According to the Dutch healthcare insurance data from 2018, the incidence and prevalence of achalasia were 2.2 per 100,000 population per year and 15.3 per 100,000 population, respectively. Similarly, Asian data from Korea showed incidence and prevalence of 0.4 per 100,000 population per year and 6.3 per 100,000 population, respectively, in 2014. According to these studies, the incidence of achalasia is increasing.
- #50https://journals.lww.com/jcma/fulltext/9900/epidemiology_and_practice_patterns_of_achalasia_in.492.aspx
Achalasia is a rare disease of gastrointestinal motility characterized by impaired esophageal peristalsis and reduced esophageal sphincter relaxation. However, data on its epidemiology and outcomes in Taiwan are limited. This study aimed to assess the incidence, characteristics, and clinical management of achalasia in Taiwan. […] In total, 206 new achalasia cases were identified. The mean annual incidence in Taiwan was 1.64 (95% confidence interval 1.22-2.05) per 100,000 persons. […] This is the first population-based epidemiological study on achalasia in Taiwan, revealing the incidence of achalasia before the era of high-resolution manometry. Clinicians should be vigilant about the development of esophageal cancer and mortality during long-term follow-ups. There is also room to enhance the utilization of various diagnostic tools for achalasia.
- #51https://journals.lww.com/ajg/abstract/2019/10001/463_the_prevalence_and_epidemiology_of_achalasia.463.aspx
Achalasia is a rare and benign neurodegenerative disorder characterized by failure of relaxation of the lower esophageal sphincter (LES) and loss of peristalsis in the distal esophagus. A substantial increase has been reported in the number of hospitalizations for achalasia and its associated costs over the last few decades and the etiology is still unknown. Epidemiological studies of this disease have been limited by small sample size. We aim to investigate further the epidemiology of achalasia using a large database. […] Of the 36,750,450 individuals in the database, we identified 19,840 individuals with achalasia with an overall prevalence of 0.05%. […] This is one of the largest epidemiological studies investigating the prevalence and epidemiology of achalasia. The estimated prevalence rate of achalasia was 0.05%. Further studies are needed to confirm these findings.
- #52 Achalasia – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK519515/
Achalasia is very rare, occurring with an annual incidence of roughly one per 100000 people and a prevalence of 10 per 100000. […] However, a recent study showed increasing hospitalizations and associated costs for achalasia over the last 16 years in the United States with a disproportionate increase in patients under 65 years of age and racial minorities. […] Achalasia occurs with equal frequency in both males and females. The disorder typically affects people between the second to the fifth decade of life with a peak incidence between the ages of 30 to 60 years. […] Outside the United States, rates of achalasia vary from 0.1 to 1 per every 100000 people per year. […] Studies show that relapse rates are higher if the initial treatment was pneumatic dilatation. However, complications have been noted to be much higher in patients who underwent a Heller myotomy compared to those who underwent pneumatic dilatation.
- #53 Esophageal Achalasia in the Veneto Region: Epidemiology and Treatment – SEARCHhttps://primo.qatar-weill.cornell.edu/discovery/fulldisplay/cdi_proquest_journals_1095652306/974WCMCIQ_INST:VU1
Achalasia is a rare esophageal motility disorder, incurable but amenable to palliative treatments to relieve dysphagia. Given the rarity of the disease, there is a paucity of data from population-based studies on incidence and outcome of the two treatments most commonly used in clinical practice, i.e., endoscopic pneumatic dilation (PD) and surgical myotomy (SM). A retrospective longitudinal study was conducted on the Veneto region, in north-eastern Italy. All patients with achalasia as their primary diagnosis between 2001 and 2005 were identified and their demographics and treatment details obtained. The overall incidence of achalasia was 1.59 cases/100,000/year. Achalasia patients were mainly seen at University Hospitals. Fifty-five percent of the patients received treatment, 23.3% SM and 31.8% PD. The cumulative risk of any subsequent intervention for achalasia was 20% in treated patients (29.7% in patients treated primarily with PD and 4% in patients treated with SM first). The epidemiology of achalasia in the Veneto Region is in line with the situation reported elsewhere and did not change between 2001 and 2005. Achalasia patients are mostly seen at University Hospitals. We observed a greater risk of subsequent intervention for patients previously treated with PD compared with SM.
- #54 Achalasia: Practice Essentials, Background, Pathophysiologyhttps://emedicine.medscape.com/article/169974-overview
The incidence of achalasia is estimated to be approximately 1.6 per 100,000 people per year. It is likely higher owing to misdiagnoses of heartburn and chest pain. […] The incidence of esophageal dysmotility appears to be increased in patients with spinal cord injury (SCI). […] In a retrospective study (1990-2013) from the Netherlands, the mean incidence of achalasia in children was 0.1 per 100,000 people per year. […] The male-to-female ratio of achalasia is 1:1. […] Achalasia typically occurs in adults aged 25-60 years. Less than 5% of cases occur in children.
- #55 Achalasia: Practice Essentials, Background, Pathophysiologyhttps://emedicine.medscape.com/article/169974-overview
The incidence of achalasia is estimated to be approximately 1.6 per 100,000 people per year. It is likely higher owing to misdiagnoses of heartburn and chest pain. […] The incidence of esophageal dysmotility appears to be increased in patients with spinal cord injury (SCI). […] In a retrospective study (1990-2013) from the Netherlands, the mean incidence of achalasia in children was 0.1 per 100,000 people per year. […] The male-to-female ratio of achalasia is 1:1. […] Achalasia typically occurs in adults aged 25-60 years. Less than 5% of cases occur in children.
- #56 Achalasia (Causes, Symptoms, and Treatment)https://patient.info/doctor/achalasia-pro
Achalasia tends to present in adult life. A Canadian study reported a mean age at diagnosis of 53.1 years. The same study reported an annual incidence of 1.63/100,000. […] Achalasia in children is rare but the incidence is rising. A UK study reported an annual incidence of 0.18/100,000. […] Endoscopic surveillance is controversial but might be worthwhile in patients with long-standing achalasia.
- #57 Epidemiology, Diagnosis, and Medical Management of Achalasia – Clinical Treehttps://clinicalpub.com/epidemiology-diagnosis-and-medical-management-of-achalasia/
Achalasia is a rare esophageal disorder presenting primarily with dysphagia, characterized by well-defined esophageal motor abnormalities. […] Epidemiologic data originate mostly from Western populations and vary among studies. The incidence rate ranges from a low of 0.03/100,000 per year to 1.63/100,000 per year, with the majority of rates clustering between 0.5 and 1.2/100,000 per year, with prevalence rates of approximately 10/100,000. Hospitalizations for achalasia increase in frequency with age, with a peak incidence in those older than 65 years, reaching a high of 37/100,000 in patients older than 85 years. The incidence of achalasia is comparable among males and females, and it can affect adults of all age groups. […] A genetic etiology is documented in a fraction of patients with achalasia. The triple-A syndrome (Allgrove disease) is a rare condition presenting with achalasia, alacrima, adrenocorticotropic hormone (ACTH) resistant adrenal insufficiency, and neurologic disturbances. A few familial cases have been described. Polymorphisms in the nitric oxide synthase gene have been investigated, but data are conflicting. No difference in polymorphisms was found between patients with achalasia and controls; however, a recent report described two siblings with infant-onset achalasia that were homozygous for a premature stop codon in the gene encoding nitric oxide synthase. Idiopathic achalasia was also found to be associated with class II human leukocyte antigens (HLAs).
- #58 :: JKMS :: Journal of Korean Medical Sciencehttps://jkms.org/DOIx.php?id=10.3346/jkms.2014.29.4.576
Owing to the rarity of the disease, epidemiologic information on achalasia is limited. […] This study aimed to investigate the epidemiology and treatment patterns of achalasia in the population of Korea using a national healthcare database. […] A total of 3,105 patients with achalasia (1,447 men; mean age, 52.5 yr) were identified between 2007 and 2011, indicating a prevalence of 6.29/100,000 (95% confidence interval [CI], 4.94-7.66) during this 5-yr period. […] A total of 191 incident cases of achalasia (82 men; mean age, 49.5 yr), which were not diagnosed as achalasia in the previous 4 yr, were detected in 2011, indicating an incidence of 0.39/100,000 (95% CI, 0.15-0.63) for that year. […] This is the first population-based epidemiologic study of achalasia in Korea. […] The present study is the first epidemiologic study of achalasia in the general population of Korea, revealing the incidence of achalasia in Korea to be 0.39/100,000/yr.
- #59 :: JKMS :: Journal of Korean Medical Sciencehttps://jkms.org/DOIx.php?id=10.3346/jkms.2014.29.4.576
We identified 3,105 cases of achalasia during the 5-yr period of the study, leading to a prevalence of 6.29/100,000 persons. […] Assuming the duration of disease for achalasia is 20 yr and its incidence is 0.39/100,000/yr, the prevalence of this disease in Korea is calculated to be 7.8/100,000 persons. […] The epidemiologic studies reported are based on hospital records from local areas, and most of the studies were from Western countries. […] The source of the current study’s data, Korea’s accessible, well-organized national health insurance system’s database, conferred several advantages. […] In conclusion, this is the first population-based epidemiologic study of achalasia in Korea, revealing its incidence, estimated prevalence, demographic features, and treatment patterns.
- #60 Advances in the management of oesophageal motility disorders in the era of high-resolution manometry: a focus on achalasia syndromes | Nature Reviews Gastroenterology & Hepatologyhttps://www.nature.com/articles/nrgastro.2017.132
A common observation among early adopters of HRM was increased detection of achalasia, leading to the suspicion that the disease was more common than previously reported. […] Several factors make it difficult to obtain accurate figures on the incidence and prevalence of achalasia. […] Consequently, available estimates, reporting annual incidence and prevalence values of about 1 per 100,000 and 10 per 100,000, respectively, are based on the assumption that all cases are detected. […] An accurate estimate of the incidence and prevalence of achalasia, as defined by HRM, would require a very large population-based HRM study. […] Nonetheless, the estimated incidence of achalasia averaged 2.92 per 100,000 during a 10-year period and the prevalence progressively increased from 15.64 to 32.58 per 100,000 over the same period, with no plateau evident.
- #61 Advances in the management of oesophageal motility disorders in the era of high-resolution manometry: a focus on achalasia syndromes | Nature Reviews Gastroenterology & Hepatologyhttps://www.nature.com/articles/nrgastro.2017.132
A common observation among early adopters of HRM was increased detection of achalasia, leading to the suspicion that the disease was more common than previously reported. […] Several factors make it difficult to obtain accurate figures on the incidence and prevalence of achalasia. […] Consequently, available estimates, reporting annual incidence and prevalence values of about 1 per 100,000 and 10 per 100,000, respectively, are based on the assumption that all cases are detected. […] An accurate estimate of the incidence and prevalence of achalasia, as defined by HRM, would require a very large population-based HRM study. […] Nonetheless, the estimated incidence of achalasia averaged 2.92 per 100,000 during a 10-year period and the prevalence progressively increased from 15.64 to 32.58 per 100,000 over the same period, with no plateau evident.
- #62 Achalasia: The Current Clinical Dilemma and Possible Pathogenesishttps://www.jnmjournal.org/view.html?uid=1836&vmd=Full
Achalasia used to be considered as a rare motility disease, with an annual incidence and prevalence of 1.63/100 000 and 10.82/100 000, respectively. However, the data might be significantly underestimated due to difficulties in identifying patients with achalasia. […] A recent American epidemiological survey conducted in areas with a high prevalence of high-resolution manometry (HRM) found that the prevalence and annual incidence of achalasia were 162.1/100 000 and 26.0/100 000 respectively, which were at least 2- to 3-fold greater than previously estimated. […] Therefore, given the poor penetration of the most advanced diagnostic criteria and technology, it is safe to say that the actual number of achalasia patients worldwide remains unknown.
- #63 :: JKMS :: Journal of Korean Medical Sciencehttps://jkms.org/DOIx.php?id=10.3346/jkms.2014.29.4.576
We identified 3,105 cases of achalasia during the 5-yr period of the study, leading to a prevalence of 6.29/100,000 persons. […] Assuming the duration of disease for achalasia is 20 yr and its incidence is 0.39/100,000/yr, the prevalence of this disease in Korea is calculated to be 7.8/100,000 persons. […] The epidemiologic studies reported are based on hospital records from local areas, and most of the studies were from Western countries. […] The source of the current study’s data, Korea’s accessible, well-organized national health insurance system’s database, conferred several advantages. […] In conclusion, this is the first population-based epidemiologic study of achalasia in Korea, revealing its incidence, estimated prevalence, demographic features, and treatment patterns.