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You actually point out indirectly an important point. A biopsy, nor a CT

nor any other test can give a diagnosis of IPF. They can give one of UIP

or any of the other conditions as defined by pathology. UIP, NSIP, and

many others. However, determining cause is a matter of clinical

diagnosis and IPF is the clinical diagnosis for UIP of undetermined

cause. Often IPF is nothing but a temporary diagnosis as more

information becomes available. But, as you point out, a cause was later

determined for yours and that was a connective tissue disease. Similarly

NSIP is a pathology diagnosis that may be determined later to have a

specific cause such as a connective tissue disease. The moment causes

for any of these are determined then the treatment plan may change.

Whereas many physicians would like to depend simply on a CT or on a

biopsy to make a diagnosis, that isn't possible. Diagnosis is a

combination of pathology and clinical evaluation and includes a

patient's history, all their blood labs and other conditions, their

PFT's, and even their response to medications in some cases. As new

evidence becomes apparent then that diagnosis might change. IPF is too

often a catch-all term with a large number of IPF diagnoses changing

over time. The process of diagnosing an Interstitial Lung Disease is

never complete in my mind. You make the diagnosis based on the

information available. But more information may become available.

Underlying connective tissue diseases may be slow to manifest

themselves.

As to my second opinion on the biopsy which also came from Mayo, I

imagine it reads much like yours. " These changes are compatible with

usual interstitial pneumonia (UIP) and may correspond to clinical

idiopathic pulmonary fibrosis (IPF). " All my diagnoses to this point

read UIP and some read IPF while others say UIP with likelihood of

underlying connective tissue disease such as undifferentiated connective

tissue disease. Connective tissue disease may manifest itself in the

future.

So, basically, a biopsy may define the pathology and the medical

condition but still cannot determine the cause. In some diseases, cause

might not be important, but in Interstitial Lung Diseases it is quite

important and the same pathology but with varying causes can be a very

different situation.

>

> I just want to add my 2 cents here. I had the VATS done in 2/07 . They

also sent it to the Mayo for a 2nd opinion. They said I had Ipf.WRONG

Then a year later I find out I have an ILD due to my several auto immune

diseases . So would I have it again HELL NO. They could see from my ct

scan I had a problem so why put yourself through it. It is a surgery and

it took a long time to get better. I couldn't even think about wearing a

bra ,even now every once in a while I am bothered.In the end it is your

choice.

> IPF 2/07 IL Scleroderma Raynauds Sjogrens

>

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Bruce,

Being new to all this I am still trying to sort all this out. Does a diagnosis of IPF mean that you have either UIP or NSIP and can it only be confirmed which one it is from a biopsy sent to pathology?

Therefore is an IPF diagnosis just used when there is no definitive information, and are there cases when a biopsy is done and it will still not confirm UIP or NSIP?

Patti Indianapolis IPF 2007

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Patti,

This is all very confusing isn't it? In general it is said that all IPF is UIP. That's why you see the designation IPF/UIP. However not all UIP is IPF. In other words If you've got UIP and they know the cause then you don't have IPF.

For example, it's possible to have hypersensitivity pneumonitis that was caused by say mold exposure that looks like UIP on the ct scan or the biopsy. In that case that individual has HP/UIP. In other words they have UIP but the cause is known so it's not IPF.

Or you could have UIP caused by a connective tissue disease so in that case you would have something like UIP/Lupus.

The only time something is clinically designated as IPF is when the cause is unknown. That's what idiopathic means. IPF literally means lung scarring of unknown origin. Once a cause is known you move away from the IPF label.

It may seem like this is all picky and insignificant but it does matter when it comes to treatment. Determining a cause for someone's fibrosis opens up the possibility of more and potentially more effective forms of treatment.

I hope this helps a bit.

Beth

Moderator

Fibrotic NSIP 06/06 Dermatomyositis 11/08

To: Breathe-Support Sent: Sat, January 9, 2010 7:59:41 AMSubject: Re: Re VATS

Bruce,

Being new to all this I am still trying to sort all this out. Does a diagnosis of IPF mean that you have either UIP or NSIP and can it only be confirmed which one it is from a biopsy sent to pathology?

Therefore is an IPF diagnosis just used when there is no definitive information, and are there cases when a biopsy is done and it will still not confirm UIP or NSIP?

Patti Indianapolis IPF 2007

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Patti,

I neglected to mention that you can have more than one type of fibrosis at the same time. We have members who have both UIP and NSIP and there can be other combinations.

The thing to remember is that there is tremendous change going on in this field. The terminology shifts around and it can be difficult to keep a handle on the research and changes. By coming here and pooling our knowledge we help each other.

Beth

Moderator

Fibrotic NSIP 06/06 Dermatomyositis 11/08

To: Breathe-Support Sent: Sat, January 9, 2010 8:42:00 AMSubject: Re: Re: Re VATS

Patti,

This is all very confusing isn't it? In general it is said that all IPF is UIP. That's why you see the designation IPF/UIP. However not all UIP is IPF. In other words If you've got UIP and they know the cause then you don't have IPF.

For example, it's possible to have hypersensitivity pneumonitis that was caused by say mold exposure that looks like UIP on the ct scan or the biopsy. In that case that individual has HP/UIP. In other words they have UIP but the cause is known so it's not IPF.

Or you could have UIP caused by a connective tissue disease so in that case you would have something like UIP/Lupus.

The only time something is clinically designated as IPF is when the cause is unknown. That's what idiopathic means. IPF literally means lung scarring of unknown origin. Once a cause is known you move away from the IPF label.

It may seem like this is all picky and insignificant but it does matter when it comes to treatment. Determining a cause for someone's fibrosis opens up the possibility of more and potentially more effective forms of treatment.

I hope this helps a bit.

Beth

Moderator

Fibrotic NSIP 06/06 Dermatomyositis 11/08

From: Patti <napa73 (AT) comcast (DOT) net>To: Breathe-Support@ yahoogroups. comSent: Sat, January 9, 2010 7:59:41 AMSubject: Re: Re VATS

Bruce,

Being new to all this I am still trying to sort all this out. Does a diagnosis of IPF mean that you have either UIP or NSIP and can it only be confirmed which one it is from a biopsy sent to pathology?

Therefore is an IPF diagnosis just used when there is no definitive information, and are there cases when a biopsy is done and it will still not confirm UIP or NSIP?

Patti Indianapolis IPF 2007

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Beth,Does that mean if you have UIP with something else it is then treatable? I guess that's where I'm confused. And how do they detirmine if you do have something else going on? When I go to Columbia Presp. Hosp. next month will they do more searching?To: Breathe-Support Sent: Sat, January 9, 2010 9:06:49 AMSubject: Re: Re: Re VATS

Patti,

I neglected to mention that you can have more than one type of fibrosis at the same time. We have members who have both UIP and NSIP and there can be other combinations.

The thing to remember is that there is tremendous change going on in this field. The terminology shifts around and it can be difficult to keep a handle on the research and changes. By coming here and pooling our knowledge we help each other.

Beth

Moderator

Fibrotic NSIP 06/06 Dermatomyositis 11/08

From: Beth <mbmurtha (AT) yahoo (DOT) com>To: Breathe-Support@ yahoogroups. comSent: Sat, January 9, 2010 8:42:00 AMSubject: Re: Re: Re VATS

Patti,

This is all very confusing isn't it? In general it is said that all IPF is UIP. That's why you see the designation IPF/UIP. However not all UIP is IPF. In other words If you've got UIP and they know the cause then you don't have IPF.

For example, it's possible to have hypersensitivity pneumonitis that was caused by say mold exposure that looks like UIP on the ct scan or the biopsy. In that case that individual has HP/UIP. In other words they have UIP but the cause is known so it's not IPF.

Or you could have UIP caused by a connective tissue disease so in that case you would have something like UIP/Lupus.

The only time something is clinically designated as IPF is when the cause is unknown. That's what idiopathic means.. IPF literally means lung scarring of unknown origin. Once a cause is known you move away from the IPF label.

It may seem like this is all picky and insignificant but it does matter when it comes to treatment. Determining a cause for someone's fibrosis opens up the possibility of more and potentially more effective forms of treatment.

I hope this helps a bit.

Beth

Moderator

Fibrotic NSIP 06/06 Dermatomyositis 11/08

From: Patti <napa73 (AT) comcast (DOT) net>To: Breathe-Support@ yahoogroups. comSent: Sat, January 9, 2010 7:59:41 AMSubject: Re: Re VATS

Bruce,

Being new to all this I am still trying to sort all this out. Does a diagnosis of IPF mean that you have either UIP or NSIP and can it only be confirmed which one it is from a biopsy sent to pathology?

Therefore is an IPF diagnosis just used when there is no definitive information, and are there cases when a biopsy is done and it will still not confirm UIP or NSIP?

Patti Indianapolis IPF 2007

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Patti

No, IPF means you have UIP of undetermined cause. Now, many are using it

to mean other things and to use before they know, but technically that

is the definition.

Biopsy can never confirm IPF. It can only confirm UIP or NSIP or

Sarcoidosis or many of the other 200 forms of PF. But IPF is confirmed

by the combination of biopsy and clinical information.

Yes, there are cases where biopsy does not give a definitive answer.

This is more common in the open biopsy where only one wedge is taken

than it most VATS where three slices are taken from different sections.

But there is still a roughly 4% error on determinations by biopsy as to

the specific form.

>

> Bruce,

> Being new to all this I am still trying to sort all this out. Does a

diagnosis of IPF mean that you have either UIP or NSIP and can it only

be confirmed which one it is from a biopsy sent to pathology?

> Therefore is an IPF diagnosis just used when there is no definitive

information, and are there cases when a biopsy is done and it will still

not confirm UIP or NSIP?

>

> Patti Indianapolis IPF 2007

>

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Bruce, even though it may be only 4% chance on an error of biopsy I sometimes feel encouraged. I have been tested for connective diseases more than once and once my dr. (rheumotologist ? )said that even though the test came back negative he couldn't rule out Lupus. And here I am with UIP.I am going to NYC Columbia Presp. Hosp next month for transplant eval. so I'm hoping they will look at my biopsy again.From: Bruce

To: Breathe-Support Sent: Sat, January 9, 2010 10:22:57 AMSubject: Re: Re VATS

If you have UIP caused by something determinable then by treating the

underlying cause, the progression of your UIP may be slowed or even

stopped for a while.

>

> Beth,

> Does that mean if you have UIP with something else it is then

treatable? I guess that's where I'm confused. And how do they detirmine

if you do have something else going on? When I go to Columbia Presp.

Hosp. next month will they do more searching?

>

>

>

>

>

> ____________ _________ _________ __

> From: Beth mbmurtha@...

> To: Breathe-Support@ yahoogroups. com

> Sent: Sat, January 9, 2010 9:06:49 AM

> Subject: Re: Re: Re VATS

>

>

> Patti,

> I neglected to mention that you can have more than one type of

fibrosis at the same time. We have members who have both UIP and NSIP

and there can be other combinations.

>

> The thing to remember is that there is tremendous change going on in

this field. The terminology shifts around and it can be difficult to

keep a handle on the research and changes. By coming here and pooling

our knowledge we help each other.

>

> Beth

> Moderator

> Fibrotic NSIP 06/06 Dermatomyositis 11/08

>

>

>

>

>

>

> ____________ _________ _________ __

> From: Beth mbmurtha (AT) yahoo (DOT) com>

> To: Breathe-Support@ yahoogroups. com

> Sent: Sat, January 9, 2010 8:42:00 AM

> Subject: Re: Re: Re VATS

>

>

> Patti,

> This is all very confusing isn't it? In general it is said that all

IPF is UIP. That's why you see the designation IPF/UIP. However not all

UIP is IPF. In other words If you've got UIP and they know the cause

then you don't have IPF.

> For example, it's possible to have hypersensitivity pneumonitis that

was caused by say mold exposure that looks like UIP on the ct scan or

the biopsy. In that case that individual has HP/UIP. In other words they

have UIP but the cause is known so it's not IPF.

> Or you could have UIP caused by a connective tissue disease so in that

case you would have something like UIP/Lupus.

>

> The only time something is clinically designated as IPF is when the

cause is unknown. That's what idiopathic means. IPF literally means lung

scarring of unknown origin. Once a cause is known you move away from the

IPF label.

>

> It may seem like this is all picky and insignificant but it does

matter when it comes to treatment. Determining a cause for someone's

fibrosis opens up the possibility of more and potentially more effective

forms of treatment.

>

> I hope this helps a bit.

>

> Beth

> Moderator

> Fibrotic NSIP 06/06 Dermatomyositis 11/08

>

>

>

>

>

>

> ____________ _________ _________ __

> From: Patti napa73 (AT) comcast (DOT) net>

> To: Breathe-Support@ yahoogroups. com

> Sent: Sat, January 9, 2010 7:59:41 AM

> Subject: Re: Re VATS

>

>

> Bruce,

> Being new to all this I am still trying to sort all this out. Does a

diagnosis of IPF mean that you have either UIP or NSIP and can it only

be confirmed which one it is from a biopsy sent to pathology?

> Therefore is an IPF diagnosis just used when there is no definitive

information, and are there cases when a biopsy is done and it will still

not confirm UIP or NSIP?

>

> Patti Indianapolis IPF 2007

>

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