Wegener's

[tasar1898]

Hey guys , I was studying Pulmonology for my rotation from Step Up to medicine and both Wegener's / Goodpasture's in the lungs result in interstitial lung disease with hemoptysis / dyspnea + a restrictive pattern of PFT's .. So I was wondering about pathophysiology and I am asking in this forum since you all are studying that stuff anyways

I have this thought: We get the granulomatous vasculitis in the lung vessels , so we get inflammation ,all this cells , protein ,blood leaking in the alveolus , maybe some pain and that results in hemoptysis/dyspnea . Eventually all this ''process'' heals , scars off and we get fibrosis , traction in the alveoli that results in a low compliance lung with fibrosed alveoli that are very hard to pop open... Kinda same thing with Goodpasture's but inflammation is type II HS , instead of type IV in Wegener's

Is my thought process correct or are there other forces in play in this disease process??

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[PaxRoma]

Your reasoning sounds good. The whole mechanism of this disease is still being worked out and I'm glad someone else is doing it.

 

[Phloston]

Hey guys , I was studying Pulmonology for my rotation from Step Up to medicine and both Wegener's / Goodpasture's in the lungs result in interstitial lung disease with hemoptysis / dyspnea + a restrictive pattern of PFT's .. So I was wondering about pathophysiology and I am asking in this forum since you all are studying that stuff anyways

I have this thought: We get the granulomatous vasculitis in the lung vessels , so we get inflammation ,all this cells , protein ,blood leaking in the alveolus , maybe some pain and that results in hemoptysis/dyspnea . Eventually all this ''process'' heals , scars off and we get fibrosis , traction in the alveoli that results in a low compliance lung with fibrosed alveoli that are very hard to pop open... Kinda same thing with Goodpasture's but inflammation is type II HS , instead of type IV in Wegener's

Is my thought process correct or are there other forces in play in this disease process??

GP syndrome is 2-3-4 - i.e., type-II HS against the alpha-3 chains of type-IV collagen, aka anti-GBM antibodies. So you get basement membrane disease, which shows up as linear on immunofluorescence. Wegener is a vasculitis, which is inflammation of blood vessels, and does not target the basement membrane specifically like GP syndrome does. It happens to be the case that much of the time there's cANCA positivity in active Wegener, although pANCA and ANCA(-) Wegener exist.

 

[tasar1898]

@Phloston Yeah of course , but after all that hypersensitivity , granulomas , vasculitis and collagen stuff is said and done , does the pathophysiology , in regards to the clinical manifestations in the lungs , sound correct?

 

[Transposony]

Goodpasture syndrome clinically may present very similar to Granulomatosis with polyangiitis.
Both of them can lead to Diffuse alveolar hemorrhage due to injury to arterioles, venules, and the alveolar septal (alveolar wall or interstitial) capillaries resulting hemoptysis secondary to disruption of the alveolar-capillary basement membrane.

 

[Phloston]

@Phloston Yeah of course , but after all that hypersensitivity , granulomas , vasculitis and collagen stuff is said and done , does the pathophysiology , in regards to the clinical manifestations in the lungs , sound correct?

Clinically GP syndrome and Wegener can present very similarly (i.e., haematuria and/or haemoptysis), but Wegener will usually have the added "head-itis," i.e. mastoiditis, sinusitis, otitis, etc. If you're asking about specific histologic descriptions, I associate "necrotizing" and "granulomatous" with Wegener (as transposony has pointed out the new name for this is cumbersomely granulomatosis with polyangiitis). For GP syndrome, it's usually a male 20-40 with disease limited to the kidneys and lungs, and they usually give the answer away by mentioning anti-GBS or linear immunofluorescence. For Wegener they can mention cANCA/anti-proteinase 3, or mononeuritis multiplex (a non-specific finding seen frequently in different vasculitides).

 

[tasar1898]

@Phloston @Transposony Thanks a lot to both of you! All this DDx info is very useful .

No I don't really care about histology/path , thankfully all this step 1 stuff is behind me . I just wanted to know if the basic pathophys link is correct in my thoughts , i.e

Inflammation in the alveolar/capillary general area due to w/e reason (wegener's , goodpasture's , microscopic polyangitis and others) leads to disruption of the interface and leaking of stuff( blood , protein , fluid , all this junk ) into the alveolus and insterstitial area ---> Hemoptysis , Dyspnea , maybe some hypoxemia due to reduced DLCO since alveoli are filled up

Then this guy gets steroids or it kinda ''heals'' on its own via scarring /fibrosis ---> Fibrotic ILD ( Interstitial Lung Disease ) with reduced compliance , lung volumes ( Restrictive PFT's)

And this process keeps repeating itself until your lungs are 2 balls of fibrotic junk.

So I just wanted to know if the above sounds correct. Any input will be greatly appreciated !

 

[thehundredthone]

Inflammation in the alveolar/capillary general area due to w/e reason (wegener's , goodpasture's , microscopic polyangitis and others) leads to disruption of the interface and leaking of stuff( blood , protein , fluid , all this junk ) into the alveolus and insterstitial area ---> Hemoptysis , Dyspnea , maybe some hypoxemia due to reduced DLCO since alveoli are filled up

Then this guy gets steroids or it kinda ''heals'' on its own via scarring /fibrosis ---> Fibrotic ILD ( Interstitial Lung Disease ) with reduced compliance , lung volumes ( Restrictive PFT's)

And this process keeps repeating itself until your lungs are 2 balls of fibrotic junk.

Or it causes alveolar hemorrhage, cavitation, atelectasis, or post-obstructive pneumonia. Take your pick. All of these things are possibilities. ILD is not a guaranteed end result, but yes, granulomatous vasculitis is a cause of ILD although MPA is more commonly associated than is GPA.

 

[tasar1898]

@thehundredthone Ok , I get it , thanks a lot!