Re: atypical pANCA

[Guest guest]

I was tested for pANCA, but not for PSC. It was about 7 years ago, and I was

receiving Remicade and Imuran for CD. I developed some rheumatic symptoms and

went to see a rheumatologist. She did several tests and diagnosed 'drug induced

Lupus' which is an occasional side effect of Remicade treatment. I was not aware

that the pANCA, which was positive, did not support that diagnosis, nor have

anything to do with it. It was when I was being diagnosed with PSC last year,

and a very astute NP decided to dog through my old labs, that the result was

re-discovered, and of course fits right in with the PSC diagnosis. My PSC was

missed by both the rheumatologist and my old GI. Hindsight is 20/20, because the

indicators have been there for years. So to answer your question, my doctor's NP

explained to me that PSC patients usually have a positive pANCA, however this

positive result can appear in many other conditions.

ee

>

> Have any of you been tested for this ? I was just looking at my latest immune

tests and I was tested for it and it was negative- the

[Guest guest]

hi ee- thanks for your response. Was it your pANCA that was tested or

*atypical* pANCA ? I didn't even know there were two seperate tests until I

reviewed my own recent immune testing- I know my son was only tested for pANCA

and not the atypical pANCA that my lab report (from LabCorp) explanation section

say are an indicator for PSC.

The labs report info section under pANCA talks about PR3 and MPO-ANCA enzyme

immunoassays being needed for follow up testing- but doesn't talk about PSC or

UC at all.

Lori

lucky mom blessed with triplets

" I was tested for pANCA, but not for PSC. "

[Guest guest]

hi ee- thanks for your response. Was it your pANCA that was tested or

*atypical* pANCA ? I didn't even know there were two seperate tests until I

reviewed my own recent immune testing- I know my son was only tested for pANCA

and not the atypical pANCA that my lab report (from LabCorp) explanation section

say are an indicator for PSC.

The labs report info section under pANCA talks about PR3 and MPO-ANCA enzyme

immunoassays being needed for follow up testing- but doesn't talk about PSC or

UC at all.

Lori

lucky mom blessed with triplets

" I was tested for pANCA, but not for PSC. "

[Guest guest]

Dear Lori;

I'm not sure whether you got a satisfactory answer on your atypical p-ANCA

question? As far as I know, absence of atypical p-ANCA does not rule out PSC

because the specificity and sensitivity of this test is very low:

________________________

J. Gastroenterol. Hepatol. 15: 437-442 (2000)

Anti-neutrophil cytoplasmic antibodies in patients with chronic liver diseases:

prevalence, antigen specificity and predictive value for diagnosis of autoimmune

liver disease. Swedish Internal Medicine Liver Club (SILK).

Lindgren S, Nilsson S, Nässberger L, Verbaan H, Wieslander J

Department of Medicine, University Hospital, Malmö, Sweden.

Stefan.Lindgren@...

BACKGROUND: Anti-neutrophil cytoplasmic antibodies (ANCA) against proteinase 3

are diagnostic of Wegener's granulomatosis, but ANCA occur also in patients with

other inflammatory disorders, such as ulcerative colitis, primary sclerosing

cholangitis (PSC) and autoimmune hepatitis. As their predictive value for

autoimmune liver disease remains unknown, we analysed the prevalence and antigen

specificity of ANCA in patients with various chronic liver diseases (CLD).

METHODS: We studied sera from 100 patients with primary biliary cirrhosis (PBC),

from 76 with PSC and from 279 with various CLD, consecutively drawn during a

5-year period at the time of liver biopsy. The ANCA were detected by indirect

immunofluorescence (IIF) while the antigen specificity was characterized by

ELISA by using lactoferrin, neutrophil elastase, cathepsin G and BPI

(bactericidal/permeability increasing protein) as antigens. RESULTS: In PBC,

ANCA were detected by IIF in 39 patients (39%). The antigen reactivity by ELISA

was lactoferrin in seven, elastase in 15, BPI in 20 and cathepsin G in four

patients. Four patients had reactivity against more than one antigen. In PSC,

IIF demonstrated ANCA in 49 patients (65%). The antigen reactivity was

lactoferrin in 17, elastase in 14, BPI in 20 and cathepsin G in four patients.

Twelve patients showed reactivity against more than one antigen. In CLD, ANCA

were observed in sera from 55 patients (20%). Nineteen of 45 patients (42%) with

autoimmune liver disease were ANCA positive versus 36/234 (15%) with

non-autoimmune liver disease (P = 0.0002). Among IIF-positive patients, antibody

reactivity against lactoferrin was noted in 14, elastase in 28, BPI in 25 and

cathepsin G in five patients. Twenty-one patients had reactivity against more

than one antigen. Elastase and BPI antibodies occurred more frequently in

patients with autoimmune compared to non-autoimmune liver disease (P < 0.01).

CONCLUSIONS: Anti-neutrophil cytoplasmic antibodies are prevalent in patients

with chronic liver diseases, but although they occur more frequently in patients

with autoimmune liver disease their specificity and sensitivity for autoimmune

liver disease is low. The predominant antigens are lactoferrin, elastase and

BPI, but the correlation between IIF findings and ELISA reactivity against these

antigens is weak. PMID: 10824890.

________________________

In other words, absence of a positive p-ANCA does not mean that autoimmune liver

disease is not present; ERCP, MRCP and biopsy are much better indicators of

this.

Best regards,

Dave

(father of (23); PSC 07/03' UC 08/03)

>

> Have any of you been tested for this ? I was just looking at my latest immune

tests and I was tested for it and it was negative- the notes on my labs says-

> The atypical pANCA pattern has been observed in a significant percentage of

patients with UC, PSC and autoimmune hepatitis.

>

> I know Braden hasn't been tested for this just the P ANCA and one dr was

convinced he didn't have PSC because the test was negative- but it sounds like

he ordered the wrong test ? I don't think you have to have a positive test for P

ANCA to be diagnosed with PSC-it's just an indicator ?? That one doctor still

keeps looking for indicators that Braden might not have PSC- even though he was

the first to suggest he had it ?!? I don't know why he is looking for signs that

he doesn't have PSC now.....but Braden seems to be stable with vancomycin and

monthly IVIG infusions but i think it would be a HUGE mistake to try and dismiss

all the evidence and now say- he doesn't have PSC KWIM ?

> Braden has small duct PSC (early in the dx process) and increased EOS per

biopsy , intrahepatic beading, repeated strictures after many dilations and

stents for 3 years, thickening of intrahepatic duct walls, persistantly

thickened common bile duct walls , classic LFT elevations that stabilized

somewhat with urso, and classic PSC indicators on all of his MRCPs and ERCPs.

>

> Lori

> lucky mom blessed with triplets

>