Re: Survival Rate Question

[Guest guest]

Barbara Webber wrote:

> I'm sorry to even ASK this question, but when I looked at this article

> it said longest record of survival of patients with PSC - is 15

> years ... is that correct?

>

Well, I've had PSC for at least 14 years and I'm not planning on dying

this year! There are others here who've had it even longer.

So no, 15 years isn't the longest survival record. Didn't someone post

an article about some who finally had a transplant after 40 years or so

of PSC?

It's hard to say exactly what's " normal " because the course of the

disease can be so variable, especially since it's often diagnosed at

very different stages of progression. Someone here can probably dig up

an average survival time from diagnosis to transplant, but those of us

who've been here for a while have seen some need a transplant a few

months after diagnosis and others who've gone many, many years, and done OK.

Not sure if that helps! We've all got to just take things as they come

I guess!

athan

[Guest guest]

Barbara Webber wrote:

> I'm sorry to even ASK this question, but when I looked at this article

> it said longest record of survival of patients with PSC - is 15

> years ... is that correct?

>

Well, I've had PSC for at least 14 years and I'm not planning on dying

this year! There are others here who've had it even longer.

So no, 15 years isn't the longest survival record. Didn't someone post

an article about some who finally had a transplant after 40 years or so

of PSC?

It's hard to say exactly what's " normal " because the course of the

disease can be so variable, especially since it's often diagnosed at

very different stages of progression. Someone here can probably dig up

an average survival time from diagnosis to transplant, but those of us

who've been here for a while have seen some need a transplant a few

months after diagnosis and others who've gone many, many years, and done OK.

Not sure if that helps! We've all got to just take things as they come

I guess!

athan

[Guest guest]

Barbara Webber wrote:

> I'm sorry to even ASK this question, but when I looked at this article

> it said longest record of survival of patients with PSC - is 15

> years ... is that correct?

>

Well, I've had PSC for at least 14 years and I'm not planning on dying

this year! There are others here who've had it even longer.

So no, 15 years isn't the longest survival record. Didn't someone post

an article about some who finally had a transplant after 40 years or so

of PSC?

It's hard to say exactly what's " normal " because the course of the

disease can be so variable, especially since it's often diagnosed at

very different stages of progression. Someone here can probably dig up

an average survival time from diagnosis to transplant, but those of us

who've been here for a while have seen some need a transplant a few

months after diagnosis and others who've gone many, many years, and done OK.

Not sure if that helps! We've all got to just take things as they come

I guess!

athan

[Guest guest]

Dear Barbara;

The statement about the longest record of survival of patients with

PSC being 15 years is derived from a study in 1981:

______________________

Dig Dis Sci. 1981 Sep;26(9):778-82.

Long-standing asymptomatic primary sclerosing cholangitis: report of

three cases.

Chapman RW, Burroughs AK, Bass NM, Sherlock S.

Three patients, two males and one female, with asymptomatic primary

sclerosing cholangitis (PSC) are described. The diagnosis was made

in each case by endoscopic retrograde cholangiography after

investigation of persistent elevation of the serum alkaline

phosphatase. All three have remained completely well without any

medical or surgical treatment for 3, 7, and 15 years, respectively,

despite extensive involvement of the biliary tree. Follow-up liver

biopsies in two have shown no histological evidence of progression

to secondary biliary cirrhosis. PSC may occur more frequently and

may follow a less severe clinical course than previously recognized.

Publication Types:

Case Reports

PMID: 7285744

______________________

It's important to note that in the last 25 years there has been much

progress in detecting PSC much earlier in the course of the disease,

and back in 1981 there were fewer treatments.

In 1989, the " median " survival time was estimated to be about 12

years:

____________________

Hepatology. 1989 Oct;10(4):430-6.

Primary sclerosing cholangitis: natural history, prognostic factors

and survival analysis.

Wiesner RH, Grambsch PM, Dickson ER, Ludwig J, MacCarty RL, Hunter

EB, Fleming TR, Fisher LD, Beaver SJ, LaRusso NF.

Department of Diagnostic Radiology, Mayo Clinic, Rochester,

Minnesota 55905.

The natural history of primary sclerosing cholangitis was assessed

in 174 patients; 37 were asymptomatic and 137 had symptoms related

to underlying liver disease. At the time of diagnosis, the mean age

was 39.9 years, 66% of the primary sclerosing cholangitis patients

were male and 71% had associated inflammatory bowel disease, most

commonly chronic ulcerative colitis. Long-term follow-up (mean: 6.0

years; range: 2.7 to 15.5 years) was available in all patients.

During follow-up, 59 (34%) of the patients died: 55 in the

symptomatic group and four in the asymptomatic group. Median

survival from the time of diagnosis of primary sclerosing

cholangitis at the Mayo Clinic was 11.9 years. Survival in the

asymptomatic group was significantly decreased compared with that in

a control population matched for age, race and sex. Multivariate

analysis ( proportional hazards regression modeling) revealed

that age, serum bilirubin concentration, blood hemoglobin

concentration, presence or absence of inflammatory bowel disease and

histologic stage on liver biopsy were independent predictors of high

risk of dying. The development of a multivariate statistical

survival model is a major step in identifying individual primary

sclerosing cholangitis patients at low, moderate and high risk of

dying. Such models will be useful for stratifying patients in

therapeutic trials, in patient counseling and in patient selection

and timing of liver transplantation. PMID: 2777204

_____________________________

In a study in The Netherlands in 2002, the median survival time was

18 years:

______________________________

Gut. 2002 Oct;51(4):562-6.

Natural history of primary sclerosing cholangitis and prognostic

value of cholangiography in a Dutch population.

Ponsioen CY, Vrouenraets SM, Prawirodirdjo W, Rajaram R, Rauws EA,

Mulder CJ, Reitsma JB, Heisterkamp SH, Tytgat GN.

Department of Gastroenterology and Hepatology, Academic Medical

Centre, Amsterdam, The Netherlands. cponsioen@...

BACKGROUND: Median survival of patients with primary sclerosing

cholangitis (PSC) has been estimated to be 12 years. Cholangiography

is the gold standard for diagnosis but is rarely used in estimating

prognosis. AIMS: To assess the natural history of Dutch PSC patients

and to evaluate the prognostic value of a cholangiographic

classification system. PATIENTS: A total of 174 patients with

established PSC attending a university hospital and three teaching

hospitals from 1970 to 1999. METHODS: Charts were reviewed for

validity and time of diagnosis, concurrent inflammatory bowel

disease, interventions, liver transplantation, occurrence of

cholangiocarcinoma, and death. Follow up data were obtained from the

charts and from the attending clinician or family physician. Median

follow up was 76 months (range 1-300). The earliest available

cholangiography was scored using a radiological classification

system for the severity of sclerosis, developed in our institution.

Survival curves were computed by the Kaplan-Meier method.

Cholangiographic staging was used to construct a prognostic model,

applying proportional hazards analysis. RESULTS: The estimated

median survival from time of diagnosis to death from liver disease

or liver transplantation was 18 years. Cholangiocarcinoma was found

in 18 (10%) patients. Fourteen patients (8%) underwent liver

transplantation. Cholangiographic scoring was inversely correlated

with survival. A combination of intrahepatic and extrahepatic

scoring, together with age at endoscopic retrograde

cholangiopancreatography, proved strongly predictive of survival.

CONCLUSIONS: The observed survival was considerably better than

reported in earlier series from Sweden, the UK, and the USA.

Classification and staging of cholangiographic abnormalities has

prognostic value. PMID: 12235081

______________________

Perhaps high-dose ursodeoxycholic acid given much earlier in the

course of the disease will help further prolong survival?

Best regards,

Dave

(father of (21); PSC 07/03; UC 08/03)

>

> I'm sorry to even ASK this question, but when I looked at this

article it said longest record of survival of patients with PSC - is

15 years ... is that correct? I see this case the patient is

alive much longer (29 years), but what is the norm/standard ...

does anyone know?

[Guest guest]

Dear Barbara;

The statement about the longest record of survival of patients with

PSC being 15 years is derived from a study in 1981:

______________________

Dig Dis Sci. 1981 Sep;26(9):778-82.

Long-standing asymptomatic primary sclerosing cholangitis: report of

three cases.

Chapman RW, Burroughs AK, Bass NM, Sherlock S.

Three patients, two males and one female, with asymptomatic primary

sclerosing cholangitis (PSC) are described. The diagnosis was made

in each case by endoscopic retrograde cholangiography after

investigation of persistent elevation of the serum alkaline

phosphatase. All three have remained completely well without any

medical or surgical treatment for 3, 7, and 15 years, respectively,

despite extensive involvement of the biliary tree. Follow-up liver

biopsies in two have shown no histological evidence of progression

to secondary biliary cirrhosis. PSC may occur more frequently and

may follow a less severe clinical course than previously recognized.

Publication Types:

Case Reports

PMID: 7285744

______________________

It's important to note that in the last 25 years there has been much

progress in detecting PSC much earlier in the course of the disease,

and back in 1981 there were fewer treatments.

In 1989, the " median " survival time was estimated to be about 12

years:

____________________

Hepatology. 1989 Oct;10(4):430-6.

Primary sclerosing cholangitis: natural history, prognostic factors

and survival analysis.

Wiesner RH, Grambsch PM, Dickson ER, Ludwig J, MacCarty RL, Hunter

EB, Fleming TR, Fisher LD, Beaver SJ, LaRusso NF.

Department of Diagnostic Radiology, Mayo Clinic, Rochester,

Minnesota 55905.

The natural history of primary sclerosing cholangitis was assessed

in 174 patients; 37 were asymptomatic and 137 had symptoms related

to underlying liver disease. At the time of diagnosis, the mean age

was 39.9 years, 66% of the primary sclerosing cholangitis patients

were male and 71% had associated inflammatory bowel disease, most

commonly chronic ulcerative colitis. Long-term follow-up (mean: 6.0

years; range: 2.7 to 15.5 years) was available in all patients.

During follow-up, 59 (34%) of the patients died: 55 in the

symptomatic group and four in the asymptomatic group. Median

survival from the time of diagnosis of primary sclerosing

cholangitis at the Mayo Clinic was 11.9 years. Survival in the

asymptomatic group was significantly decreased compared with that in

a control population matched for age, race and sex. Multivariate

analysis ( proportional hazards regression modeling) revealed

that age, serum bilirubin concentration, blood hemoglobin

concentration, presence or absence of inflammatory bowel disease and

histologic stage on liver biopsy were independent predictors of high

risk of dying. The development of a multivariate statistical

survival model is a major step in identifying individual primary

sclerosing cholangitis patients at low, moderate and high risk of

dying. Such models will be useful for stratifying patients in

therapeutic trials, in patient counseling and in patient selection

and timing of liver transplantation. PMID: 2777204

_____________________________

In a study in The Netherlands in 2002, the median survival time was

18 years:

______________________________

Gut. 2002 Oct;51(4):562-6.

Natural history of primary sclerosing cholangitis and prognostic

value of cholangiography in a Dutch population.

Ponsioen CY, Vrouenraets SM, Prawirodirdjo W, Rajaram R, Rauws EA,

Mulder CJ, Reitsma JB, Heisterkamp SH, Tytgat GN.

Department of Gastroenterology and Hepatology, Academic Medical

Centre, Amsterdam, The Netherlands. cponsioen@...

BACKGROUND: Median survival of patients with primary sclerosing

cholangitis (PSC) has been estimated to be 12 years. Cholangiography

is the gold standard for diagnosis but is rarely used in estimating

prognosis. AIMS: To assess the natural history of Dutch PSC patients

and to evaluate the prognostic value of a cholangiographic

classification system. PATIENTS: A total of 174 patients with

established PSC attending a university hospital and three teaching

hospitals from 1970 to 1999. METHODS: Charts were reviewed for

validity and time of diagnosis, concurrent inflammatory bowel

disease, interventions, liver transplantation, occurrence of

cholangiocarcinoma, and death. Follow up data were obtained from the

charts and from the attending clinician or family physician. Median

follow up was 76 months (range 1-300). The earliest available

cholangiography was scored using a radiological classification

system for the severity of sclerosis, developed in our institution.

Survival curves were computed by the Kaplan-Meier method.

Cholangiographic staging was used to construct a prognostic model,

applying proportional hazards analysis. RESULTS: The estimated

median survival from time of diagnosis to death from liver disease

or liver transplantation was 18 years. Cholangiocarcinoma was found

in 18 (10%) patients. Fourteen patients (8%) underwent liver

transplantation. Cholangiographic scoring was inversely correlated

with survival. A combination of intrahepatic and extrahepatic

scoring, together with age at endoscopic retrograde

cholangiopancreatography, proved strongly predictive of survival.

CONCLUSIONS: The observed survival was considerably better than

reported in earlier series from Sweden, the UK, and the USA.

Classification and staging of cholangiographic abnormalities has

prognostic value. PMID: 12235081

______________________

Perhaps high-dose ursodeoxycholic acid given much earlier in the

course of the disease will help further prolong survival?

Best regards,

Dave

(father of (21); PSC 07/03; UC 08/03)

>

> I'm sorry to even ASK this question, but when I looked at this

article it said longest record of survival of patients with PSC - is

15 years ... is that correct? I see this case the patient is

alive much longer (29 years), but what is the norm/standard ...

does anyone know?

[Guest guest]

Dear Barbara;

The statement about the longest record of survival of patients with

PSC being 15 years is derived from a study in 1981:

______________________

Dig Dis Sci. 1981 Sep;26(9):778-82.

Long-standing asymptomatic primary sclerosing cholangitis: report of

three cases.

Chapman RW, Burroughs AK, Bass NM, Sherlock S.

Three patients, two males and one female, with asymptomatic primary

sclerosing cholangitis (PSC) are described. The diagnosis was made

in each case by endoscopic retrograde cholangiography after

investigation of persistent elevation of the serum alkaline

phosphatase. All three have remained completely well without any

medical or surgical treatment for 3, 7, and 15 years, respectively,

despite extensive involvement of the biliary tree. Follow-up liver

biopsies in two have shown no histological evidence of progression

to secondary biliary cirrhosis. PSC may occur more frequently and

may follow a less severe clinical course than previously recognized.

Publication Types:

Case Reports

PMID: 7285744

______________________

It's important to note that in the last 25 years there has been much

progress in detecting PSC much earlier in the course of the disease,

and back in 1981 there were fewer treatments.

In 1989, the " median " survival time was estimated to be about 12

years:

____________________

Hepatology. 1989 Oct;10(4):430-6.

Primary sclerosing cholangitis: natural history, prognostic factors

and survival analysis.

Wiesner RH, Grambsch PM, Dickson ER, Ludwig J, MacCarty RL, Hunter

EB, Fleming TR, Fisher LD, Beaver SJ, LaRusso NF.

Department of Diagnostic Radiology, Mayo Clinic, Rochester,

Minnesota 55905.

The natural history of primary sclerosing cholangitis was assessed

in 174 patients; 37 were asymptomatic and 137 had symptoms related

to underlying liver disease. At the time of diagnosis, the mean age

was 39.9 years, 66% of the primary sclerosing cholangitis patients

were male and 71% had associated inflammatory bowel disease, most

commonly chronic ulcerative colitis. Long-term follow-up (mean: 6.0

years; range: 2.7 to 15.5 years) was available in all patients.

During follow-up, 59 (34%) of the patients died: 55 in the

symptomatic group and four in the asymptomatic group. Median

survival from the time of diagnosis of primary sclerosing

cholangitis at the Mayo Clinic was 11.9 years. Survival in the

asymptomatic group was significantly decreased compared with that in

a control population matched for age, race and sex. Multivariate

analysis ( proportional hazards regression modeling) revealed

that age, serum bilirubin concentration, blood hemoglobin

concentration, presence or absence of inflammatory bowel disease and

histologic stage on liver biopsy were independent predictors of high

risk of dying. The development of a multivariate statistical

survival model is a major step in identifying individual primary

sclerosing cholangitis patients at low, moderate and high risk of

dying. Such models will be useful for stratifying patients in

therapeutic trials, in patient counseling and in patient selection

and timing of liver transplantation. PMID: 2777204

_____________________________

In a study in The Netherlands in 2002, the median survival time was

18 years:

______________________________

Gut. 2002 Oct;51(4):562-6.

Natural history of primary sclerosing cholangitis and prognostic

value of cholangiography in a Dutch population.

Ponsioen CY, Vrouenraets SM, Prawirodirdjo W, Rajaram R, Rauws EA,

Mulder CJ, Reitsma JB, Heisterkamp SH, Tytgat GN.

Department of Gastroenterology and Hepatology, Academic Medical

Centre, Amsterdam, The Netherlands. cponsioen@...

BACKGROUND: Median survival of patients with primary sclerosing

cholangitis (PSC) has been estimated to be 12 years. Cholangiography

is the gold standard for diagnosis but is rarely used in estimating

prognosis. AIMS: To assess the natural history of Dutch PSC patients

and to evaluate the prognostic value of a cholangiographic

classification system. PATIENTS: A total of 174 patients with

established PSC attending a university hospital and three teaching

hospitals from 1970 to 1999. METHODS: Charts were reviewed for

validity and time of diagnosis, concurrent inflammatory bowel

disease, interventions, liver transplantation, occurrence of

cholangiocarcinoma, and death. Follow up data were obtained from the

charts and from the attending clinician or family physician. Median

follow up was 76 months (range 1-300). The earliest available

cholangiography was scored using a radiological classification

system for the severity of sclerosis, developed in our institution.

Survival curves were computed by the Kaplan-Meier method.

Cholangiographic staging was used to construct a prognostic model,

applying proportional hazards analysis. RESULTS: The estimated

median survival from time of diagnosis to death from liver disease

or liver transplantation was 18 years. Cholangiocarcinoma was found

in 18 (10%) patients. Fourteen patients (8%) underwent liver

transplantation. Cholangiographic scoring was inversely correlated

with survival. A combination of intrahepatic and extrahepatic

scoring, together with age at endoscopic retrograde

cholangiopancreatography, proved strongly predictive of survival.

CONCLUSIONS: The observed survival was considerably better than

reported in earlier series from Sweden, the UK, and the USA.

Classification and staging of cholangiographic abnormalities has

prognostic value. PMID: 12235081

______________________

Perhaps high-dose ursodeoxycholic acid given much earlier in the

course of the disease will help further prolong survival?

Best regards,

Dave

(father of (21); PSC 07/03; UC 08/03)

>

> I'm sorry to even ASK this question, but when I looked at this

article it said longest record of survival of patients with PSC - is

15 years ... is that correct? I see this case the patient is

alive much longer (29 years), but what is the norm/standard ...

does anyone know?

[Guest guest]

>

> > I'm sorry to even ASK this question, but when I looked at this

article

> > it said longest record of survival of patients with PSC - is 15

> > years ... is that correct?

> >

>

>

>

>

> Well, I've had PSC for at least 14 years and I'm not planning on

dying

> this year! There are others here who've had it even longer.

>

> So no, 15 years isn't the longest survival record. Didn't someone

post

> an article about some who finally had a transplant after 40 years

or so

> of PSC?

>

In reading Barbara's post I wanted to make sure everyone understood

what the term " survival " meant. Survival does not necessarily mean

death but also can mean time to transplant. When you see studies

citing " survival " statistics that can either mean time to liver

failure requiring transplantation or less likely death. Most

patients receive a transplant and continue on well beyond 15 years.

in Seattle

UC 1991, PSC 2001

[Guest guest]

Barbara,

As pointed out that paper was very dated. Published the same

year (1983) that cyclosporine allowed transplantation to change from

an experimental procedure to a standard of treatment. The following

year the National Organ Transplant Act (NOTA) passed, which authorized

the Organ Procurement and Transplantation Network (OPTN) (established

in 1986). Even the previous long term PSC survivor at that time of 15

years had only been diagnosed 1 year after the first successful liver

transplant, which was done in 1967.

The developments over the last 40 years in endoscopy, transplantation,

radiology and pharmacology have made a huge difference in our lives

and the prospect of survival beyond end stage liver disease. As MizKit

implied, we can look forward to more " miracles " in the future.

Tim R

>

> I'm sorry to even ASK this question, but when I looked at this

> article it said longest record of survival of patients with PSC - is

> 15 years ... is that correct? I see this case the patient is

> alive much longer (29 years), but what is the norm/standard ...

> does anyone know?

>