PSC & Autoimmune Hepatitis

[Guest guest]

Hi All

Just to let you all know that around! I have had a complete

reassessment of my condition due to serious jaundice, pain and weight

loss as well as elevated cholestorol and iron levels. PSC was

confirmed and existence of autoimmune hepatitis discovered. This is

the classic overlap syndrome where both conditions exist side by side

and present similar symptoms. Just a note of caution to all to

investigate every possibility if you are concerned. The autoimmune

hepatitis was discovered because of unusual smooth muscle antibody

(SMA) test results. It can be very serious if not treated leading to

cirrhosis and liver failure. Unfortunately, it has to be treated

agressively with cortisone and other immune suppressing drugs and one

has to just live with the side effects. So please don't ever dismiss

any symptoms that concern you. Have it checked out thoroughly! Rather

safe than sorry!

[Guest guest]

Hi Meena;

I am really glad for you that your doctors discovered this

assciation. I hope you respond quickly to the steroids. The following

paper came out today on this rare condition, and reiterates your

point that any sudden change of symptoms deserves careful

investigation:

J Clin Gastroenterol. 2004 Nov;38(10):906-909.

Rapid Progression of Autoimmune Hepatitis in the Background of

Primary Sclerosing Cholangitis.

Hong-Curtis J, Yeh MM, Jain D, Lee JH.

From the Section of Digestive Diseases, Department of Internal

Medicine, and Department of Pathology, Yale University School of

Medicine, New Haven, CT.

" Overlap syndromes " have been reported among various autoimmune liver

diseases, particularly between primary biliary cirrhosis and

autoimmune hepatitis (AIH) in adults and between AIH and autoimmune

cholangitis in children. The overlap syndrome of AIH and primary

sclerosing cholangitis (PSC), however, has been scarcely reported.

Furthermore, in most of the reported cases of AIH/PSC overlap

syndrome, PSC and AIH were believed to occur simultaneously. We

report a case of a 34-year-old woman who has ulcerative colitis and

PSC (diagnosed by colonoscopy, histology, and cholangiogram) and 7

years later develops rapidly progressive liver failure and hemolytic

anemia from AIH. Liver biopsy showed dense portal lymphoplasmacytic

infiltrate with interface hepatitis and acidophil bodies confirming

AIH. She responded well to immunosuppressive therapy with steroids,

both with respect to her liver disease and her autoimmune hemolytic

anemia. Additionally, her clinical symptoms of fatigue, jaundice, and

pruritus improved markedly and quickly. Overlap or " crossover "

syndrome should be considered in all patients with PSC when they

present with sudden deterioration of the liver function and changes

in liver enzymes. By making the diagnosis of AIH in a patient with

well-established PSC, appropriate treatment can be initiated,

resulting in the patient's prompt recovery.

PMID: 15492611

Best regards,

Dave

(father of (19); PSC 07/03; UC 08/03)

[Guest guest]

Hi

Thanks for the information. I will pass it on to my doctor as well.

Will keep you informed about any progress on my side.

Regards

Meena

>

> Hi Meena;

>

> I am really glad for you that your doctors discovered this

> assciation. I hope you respond quickly to the steroids. The

following

> paper came out today on this rare condition, and reiterates your

> point that any sudden change of symptoms deserves careful

> investigation:

>

> J Clin Gastroenterol. 2004 Nov;38(10):906-909.

>

> Rapid Progression of Autoimmune Hepatitis in the Background of

> Primary Sclerosing Cholangitis.

>

> Hong-Curtis J, Yeh MM, Jain D, Lee JH.

>

> From the Section of Digestive Diseases, Department of Internal

> Medicine, and Department of Pathology, Yale University School of

> Medicine, New Haven, CT.

>

> " Overlap syndromes " have been reported among various autoimmune

liver

> diseases, particularly between primary biliary cirrhosis and

> autoimmune hepatitis (AIH) in adults and between AIH and autoimmune

> cholangitis in children. The overlap syndrome of AIH and primary

> sclerosing cholangitis (PSC), however, has been scarcely reported.

> Furthermore, in most of the reported cases of AIH/PSC overlap

> syndrome, PSC and AIH were believed to occur simultaneously. We

> report a case of a 34-year-old woman who has ulcerative colitis and

> PSC (diagnosed by colonoscopy, histology, and cholangiogram) and 7

> years later develops rapidly progressive liver failure and

hemolytic

> anemia from AIH. Liver biopsy showed dense portal lymphoplasmacytic

> infiltrate with interface hepatitis and acidophil bodies confirming

> AIH. She responded well to immunosuppressive therapy with steroids,

> both with respect to her liver disease and her autoimmune hemolytic

> anemia. Additionally, her clinical symptoms of fatigue, jaundice,

and

> pruritus improved markedly and quickly. Overlap or " crossover "

> syndrome should be considered in all patients with PSC when they

> present with sudden deterioration of the liver function and changes

> in liver enzymes. By making the diagnosis of AIH in a patient with

> well-established PSC, appropriate treatment can be initiated,

> resulting in the patient's prompt recovery.

>

> PMID: 15492611

>

> Best regards,

>

> Dave

> (father of (19); PSC 07/03; UC 08/03)