path q

[MudPhud20XX]

A 27-year-old woman has had increasing muscular pain, particularly involving her upper arms and legs, not related to exercise, for the past year. She has had increasing difficulty swallowing for the past 2 months. Her fingers now often become blue and painful when exposed to cold. On physical examination she has 4/5 muscle strength. She is afebrile. Which of the following light microscopic features is most likely to be present in a muscle biopsy from this woman?

A. myositis with lymphocytic infiltrate
B. Grouped atropy of myofibers
C. Extensive fibrosis and loss of fibers
D. Perivascular eosinophils
E. Coagulative necrosis
F. Increased glycogen deposition

When I first read this question, I thought it was CREST (limited scleroderma), but the pt actually has polymyositis according to the answer. So can anyone explain this? The pt seems to be having Raynaud and esohpagus dysmotility that seem to be pointing to scleroderma. So what am I missing here?

---

Members don't see this ad.

 

[Apoplexy__]

A 27-year-old woman has had increasing muscular pain, particularly involving her upper arms and legs, not related to exercise, for the past year. She has had increasing difficulty swallowing for the past 2 months. Her fingers now often become blue and painful when exposed to cold. On physical examination she has 4/5 muscle strength. She is afebrile. Which of the following light microscopic features is most likely to be present in a muscle biopsy from this woman?

A. myositis with lymphocytic infiltrate
B. Grouped atropy of myofibers
C. Extensive fibrosis and loss of fibers
D. Perivascular eosinophils
E. Coagulative necrosis
F. Increased glycogen deposition

When I first read this question, I thought it was CREST (limited scleroderma), but the pt actually has polymyositis according to the answer. So can anyone explain this? The pt seems to be having Raynaud and esohpagus dysmotility that seem to be pointing to scleroderma. So what am I missing here?

CREST is definitely a good answer. I probably would have chosen it, personally.

I think the key differentiating element here is the muscle weakness and pain. Particularly when it says upper arms and legs (i.e., the classic proximal muscle weakness).

 

[mckinsey]

Agree with kirbymiester on distinguishing between muscle weakness and pain, as well as the fact that there's proximal muscle weakness going on. Because she also has CREST sx, that leads you in the direction of autoimmune pathophysiology. She may very well have CREST syndrome. Autoimmune disorders like to flock together, so at this point you should start thinking it's polymyositis.

 

[cruCiATE]

Tricky questions since myositis is characterized my marked proximal muscle weakness as well as pain (her muscle strength is 4/5...), according to some sources 1 out of 3 patients of polymyositis do develop dysphagia but there is no where to be found that they develop Renaud Phenomenon also... I would still go with CREST but it could be one of those overlapping situations...

 

[Transposony]

What am I missing here?
Which one of the choices is pointing to CREST?

 

[cruCiATE]

C ?

 

[Transposony]

C ?

This is what FA says:

Limited scleroderma—limited skin involvement confined to fingers and face.

Here it is muscle biopsy.
I am not aware of muscle involvement in CREST syndrome.

 

[MudPhud20XX]

What am I missing here?
Which one of the choices is pointing to CREST?

The answer is A. The pt has polymyositis. I think none of the answer choice is pointing to CREST. This q is from Goljan Rapid Review Path Q bank

 

[Transposony]

The answer is A. The pt has polymyositis. I think none of the answer choice is pointing to CREST. This q is from Goljan Rapid Review Path Q bank

Thanks. I was scratching my head thinking which one of the choices was pointing to CREST.

 

[Halothane27]

Is there an explanation for what is "c"??? The fibrosis part is probably an indicator for CREST.. but the loss of fibers is totally out there.

 

[thehundredthone]

Thanks. I was scratching my head thinking which one of the choices was pointing to CREST.

And this is what should get you (not you Transposony) thinking about autoimmune muscle disorders since they're asking about muscle biopsy findings in a woman with weakness. None of the options has anything to do with CREST, so obviously they're asking about something else. Like everyone has said, if you see one autoimmune disorder, all other autoimmune disorders become fair game.

 

[mckinsey]

Is there an explanation for what is "c"??? The fibrosis part is probably an indicator for CREST.. but the loss of fibers is totally out there.

"C. extensive fibrosis and loss of fibers" = most likely getting at muscular dystrophies like Duchenne/Becker. You'll see progressive loss of muscle fibers and fibrosis/fatty replacement in those settings.

 

[Halothane27]

"C. extensive fibrosis and loss of fibers" = most likely getting at muscular dystrophies like Duchenne/Becker. You'll see progressive loss of muscle fibers and fibrosis/fatty replacement in those settings.

Thanks!!!!

 

[MudPhud20XX]

So here is the full explanation.

Option A (Myositis with lymphocytic infiltrates) is correct. Polymyositis is treatable with corticosteroid therapy. Jo-1 antibody may be present. The disease is probably mediated by cytotoxic CD8 lymphocytes.

Option B (Grouped atrophy of myofibers) is incorrect. Grouped atrophy is a feature of neurogenic muscular diseases such as amyotrophic lateral sclerosis.

Option C (Extensive fibrosis and loss of fibers) is incorrect. Extensive fibrosis can be seen in the end stage of many muscular diseases, particularly dystrophic muscular diseases.

Option D (Perivascular eosinophils) is incorrect. Eosinophilia suggests an allergic reaction or parasitic infestation. In muscle, it is most characteristic for trichinosis.

Option E (Coagulative necrosis) is incorrect. The vascular supply to muscle is generally good. Infarction is most likely to occur with a compartment syndrome in a focal region. With peripheral vascular disease, gangrenous necrosis is more likely to encompass multiple tissues in an extremity, not just muscle.

Option F (Increased glycogen deposition) is incorrect. Sarcolemmal glycogen deposition is a feature of McArdle syndrome from myophosphorylase deficiency.

 

[cruCiATE]

Ok so just to be clear, if the patient where to have CREST (again just to be clear) what would a good answer choice be ? Something like, Extensive fibrosis and collagen deposition limited to skin ?
And what would a classic vignette be like ? (Calcinosis, Rayunad Phenom, Dysphagia, Sclerodactyly and the Anti Centromere AB) and nothing about muscle I supposed

 

[MudPhud20XX]

Ok so just to be clear, if the patient where to have CREST (again just to be clear) what would a good answer choice be ? Something like, Extensive fibrosis and collagen deposition limited to skin ?
And what would a classic vignette be like ? (Calcinosis, Rayunad Phenom, Dysphagia, Sclerodactyly and the Anti Centromere AB) and nothing about muscle I supposed

I haven't encountered any from Q banks, but I will post one if I see one.

In general, CREST syndrome is a localized variant of systemic sclerosis. In both conditions, smooth muscle of the esophagus is replaced by normal collagen causing aperistalsis and dysphagia for solids and liquids, which is not present in the patient.

Systemic sclerosis targets more organs than CREST syndrome, which is a limited variant of systemic sclerosis. Esophageal findings are similar to the findings described for CREST syndrome.

 

[MudPhud20XX]

alright, here is a clinical vignette from UW.

A 45 yr old Caucasian female suffers from recurrent nonpitting edema of the hands. She often wears warm gloves indoors b/c her fingers turn blue when they are cold. She also complains of retrosternal burning and regurgitaion, especially when supine. Which of the following antibodies is most likely to be found in this pt?
A. Anti-dsDNA
B. Anti-centromere
C. Anti-histone
D. Anti-SSA and SSB
E. Anti-phospholipid

 

[SBR249]

Edit: oops this is what I get for not reading the whole thread. Obviously the person has CREST since it's what the thread's been talking about.

 

[thehundredthone]

Ok so just to be clear, if the patient where to have CREST (again just to be clear) what would a good answer choice be ? Something like, Extensive fibrosis and collagen deposition limited to skin ?

...characteristically shows excessive ground substance and odd-looking endothelial cells in the dermis and later deposits of collagen. The epidermis is usually atrophic.

alright, here is a clinical vignette from UW.

A 45 yr old Caucasian female suffers from recurrent nonpitting edema of the hands. She often wears warm gloves indoors b/c her fingers turn blue when they are cold. She also complains of retrosternal burning and regurgitaion, especially when supine. Which of the following antibodies is most likely to be found in this pt?
A. Anti-dsDNA
B. Anti-centromere
C. Anti-histone
D. Anti-SSA and SSB
E. Anti-phospholipid

Are you looking for the answer to this one? Or is it an example for the previous poster, because this is quite straightforward.

 

[MudPhud20XX]

...characteristically shows excessive ground substance and odd-looking endothelial cells in the dermis and later deposits of collagen. The epidermis is usually atrophic.


Are you looking for the answer to this one? Or is it an example for the previous poster, because this is quite straightforward.

thank you. I wanted to show an example of CREST clinical vignette.

 

[cruCiATE]

Yeah I saw this on uW as well, just to clarify see how there is nothing about muscle pain in this vignette. thanks for the post Mud