IgG4–related systemic disease...someone who knows about this?


As I said ... my mother after pancreatitis attacks... she get Whipple surgery for pseudocyst in the head of pancreas...some months after she diagnosed with retroperitoneal fibrosis ... before Christmas after liver biopsy diagnosed with PSC ... next week we will go to an endocrinologist because there are indications of abnormalities in the thyroid gland...maybe is hashimoto's thyroiditis...according to the rheumatologist ... all this autoimmunity (retroperitoneal fibrosis, PSC and maybe thyroiditis) ... derive from an antibody called IGG4...and which causes a syndrome of diseases causing fibrosis in various organs... in the case of the PSC ...the IgG4-related sclerosing cholangitis...is a different clinical entity from the "classical" PSC with better prognosis and response to immunosuppression with steroids and azathioprine (for all the manifestations of the syndrome the treatment is the same)...but unfortunately we have clinical manifestations but do not have an official diagnosis ... because when they did the liver biopsy they didn't do histopathological and immunohistochemical staining for IGG4 antibody...this according to the rheumatologist...the hepatologist disagrees with this case...he thinks that it is a simple retroperitoneal fibrosis who affect liver...but from April my mother started azathioprine ...and liver enzymes fell more...that's all for know....!

I haven’t had these issues…yet?! But I did have pancreatitis and had the Whipple (found malignant cells in pancreatic duct) and 6 months later dx with PBC but a year after that dx with PSC. My Dr has Sclerosing Cholangitis, Autoimmune Cholangitis plus the PBC and the PSC all listed as my dx on my chart. Gets very confusing at times because they are similar and different at the same time! Is it because they really don’t know…?! What were some of her symptoms for them to look towards that direction of thyroid and such and are they new symptoms, ect?
Thanks Lori

I had hoped docs were being more careful these days before doing Whipple procedures as sometimes they are done needlessly when in fact the patient has autoimmune pancreatitis (an IgG4 disease) which is treated with prednisone. I know someone who recently refused Whipple surgery by a specialist of fine repute, the patient did not believe there was carcinoma and it turned out he was indeed correct - it's autoimmune pancreatitis (AIP) and he is doing quite well on prednisone. Most people will respond to a 2-3 month round or two of prednisone and are then ok.

I have AIP which fortunately was diagnosed correctly by IgG4 8x higher than nrl and endoscopic ultrasound. Unfortunately the prednisone (several 3 month rounds of it) only provided very temporary relief. Have been on azathiaprine since 2009. IgG4 has been reasonably decent, though the past year seems to be slowly rising. My PSC is actually secondary sclerosing cholangitis (SCC) to the AIP but is essentially doing a fine impression of PSC.

Lori, so often these autoimmune diseases overlap and it does get mired down and confusing and the dx's can change like the weather.

Thanks for your response! well ...Alix...the pseudocyst is a complication of chronic pancreatitis ... or in our case of autoimmune pancreatitis ... and when it is at the head of the pancreas symptoms are similar to those of a tumor ... my mother had jaundice .. . lost many pounds ... amylase was high ... but tumor markers were normal, and imaging tests showed no malignancy ... in gastroenterological department tried to make puncture to that "mass"...but did not make it ... and then my mother transferred to the surgical department ... the surgeon did not take the responsibility to say if it was malignancy or not ... until the surgery and biopsy are done (LOT OF STRESS!!!!)... and biopsy of the pancreas reportes that there is necrosis, fibrosis and thickening of the wall of the pseudocyst ... so surgery was unavoidable...Anyway ... this belongs to the past ... it was major... but successful surgery ...Now the important thing is the continuation of immunosuppression ... and unfortunately my mother stopped azathioprine because of a urinary infection ... we do tests to find the right antibiotic...to be able to restart azathioprine...lori...my mother has no symptoms of thyroid gland...except high thyroid peroxidase antibody (TPO)...generally important blood tests for the thyroid gland are: T3, T4, TSH, TPO and TG antibodies... and ultrasound of course...the problem with the thyroid started when began to decrease cortisone ...so cortisone is important and for this issue...my mother is generally in good condition ... blood tests with azathioprine was perfect ... goes to work ... hates doctors as usual ...and i have to solve the issue with the urinary tract infection ...!!!


Stephen thanks for your help!!!!!!

Let me share my minimal knowledge (which might display my ignorance). Ig stands for ImmunoGlobulin, I presume. After receiving a liver transplant which cured my PSC, I was diagnosed with Hypogammaglobulinemia, Common Variable Immunodeficiency (CVID). Long, complex words that mean my body is not producing A, G and M, immune cells. I now receive infusions of human immune cells to protect me from infections. Science has not established a link between CVID and PSC. My immunologist works at Northwestern Memorial Hospital in Chicago, which is where I received my transplant. I don't think my condition would have been diagnosed if I had gone to a lesser facility. Immune problems and PSC go together but scientists just don't know how. I was persistent describing my symptoms to my hepatologist, who tested me every which way for 6 months. When he saw the strong likelihood that I had some kind of immune disease that was causing my gut to be upset, he referred me to my immunologist who said the testing was complete and I clearly had CVID.

In their database that is shared with other hospitals, he found one other PSC patient with a transplant that had CVID. I don't know who it is or where they are, but both doctors applied for funds to do research on both of us. Our treatment goal is to protect us from infections. Yours will be different. I can't help but think your condition is linked to the immune problems I have. They just don't know much about immune system diseases. I wish you good luck with treatment.

Γεια σου Κώστα. Τι κάνετε? Πώς είναι η υγεία της μητέρας σου? Μια

που είμαστε και οι δύο από Ελλάδα μπορούμε να ανταλλάξουμε εμπειρία από τα ελληνικά δεδομένα.


vango said:

Γεια σου Κώστα. Τι κάνετε? Πώς είναι η υγεία της μητέρας σου? Μια

που είμαστε και οι δύο από Ελλάδα μπορούμε να ανταλλάξουμε εμπειρία από τα ελληνικά δεδομένα.

very kind of yours sparkles