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  #1  
Old 07-08-2012
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Question Systemic Sclerosis or Scleroderma!

A 49-year-old African American female complains of progressive exertional dyspnea. She also complains of swelling and pain in her finger joints over the last 6 months. She has been treated for severe heartburn with pantoprazole and for hypertension with lisinopril and hydrochlorothiazide. Her blood pressure is 145/100 mmHg and her heart rate is 80/min. Physical examination reveals prominent jugular venous distention. Her lungs are clear to auscultation, and no murmurs are present on cardiac auscultation. Her skin is thickened and discolored over the fingertips and interphalangeal joints. There is mild pitting ankle edema.

Systemic sclerosis or scleroderma! Please explain.
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systemic sclerosis since she has JVD secondary to Pulmonary HT >>>Right Heart Failure.
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Originally Posted by Novobiocin View Post
systemic sclerosis since she has JVD secondary to Pulmonary HT >>>Right Heart Failure.
as per my kno, PHTN can occur in scleroderma too. so ?


from wiki
Quote:
Additionally, pulmonary arterial hypertension may occur in up to one-third of patients and is the most serious complication for this form of scleroderma.
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Quote:
Originally Posted by tyagee View Post
as per my kno, PHTN can occur in scleroderma too. so ?
There is a lot of confusion regarding the terms Systemic Sclerosis and Scleroderma. Even MTBs refer to them as same disease. However, Medstudy explains it best.
Strictly speaking (by definition) Scleroderma means involvement of skin only (aka Morphea). Also linear Scleroderma is classified under this category.

Systemic Sclerosis (aka Systemic scleroderma) means Scleroderma with systemic (organ) involvement and it is divided into two:

Diffuse systemic sclerosis is rapidly progressing and affects a large area of the skin and one or more internal organs, frequently the kidneys, esophagus, heart, and lungs. It is characterized by skin thickening that involves areas proximal to the elbows and/or knees. Interstitial lung disease is more common in this form.

Limited systemic sclerosis involves cutaneous manifestations that mainly affect the hands, arms, and face. Limited cutaneous systemic sclerosis (lcSSc) is characterized by skin disease that does not progress proximal to the elbows or knees. A subset of which is called CREST syndrome. Pulmonary arterial hypertension occurs in this form.

Hope this clears things up!

Last edited by Novobiocin; 07-08-2012 at 06:06 PM.
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Old 07-09-2012
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What's the ans. ?


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Old 07-09-2012
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Originally Posted by tyagee View Post
What's the ans. ?
answer is obvious
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systemic sclerosis since she has JVD secondary to Pulmonary HT >>>Right Heart Failure.
are you saying systemic sclerosis ?
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Originally Posted by tyagee View Post
are you saying systemic sclerosis ?
Yes, as highlighted in red in my previous post.

Quote:
Systemic Sclerosis (aka Systemic scleroderma) means Scleroderma with systemic (organ) involvement and it is divided into two:

Diffuse systemic sclerosis is rapidly progressing and affects a large area of the skin and one or more internal organs, frequently the kidneys, esophagus, heart, and lungs. It is characterized by skin thickening that involves areas proximal to the elbows and/or knees. Interstitial lung disease is more common in this form.

Limited systemic sclerosis involves cutaneous manifestations that mainly affect the hands, arms, and face. Limited cutaneous systemic sclerosis (lcSSc) is characterized by skin disease that does not progress proximal to the elbows or knees. A subset of which is called CREST syndrome. Pulmonary arterial hypertension occurs in this form.
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Originally Posted by Novobiocin View Post
Yes, as highlighted in red in my previous post.
what is the cause of dyspnea in this patient ? is it PAHTN or Interstitial lung disease ? first you said it is PAHTN ! if that is hte cause, ans should be limited scleroderma...
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Originally Posted by tyagee View Post
what is the cause of dyspnea in this patient ? is it PAHTN or Interstitial lung disease ? first you said it is PAHTN ! if that is hte cause, ans should be limited scleroderma...
I have explained in detail in my previous post but you are not trying to understand............read it again
It is Systemic sclerosis (aka Systemic scleroderma) which has two types Diffuse and Limited.
Scleroderma is just scleroderma (without any systemic manifestations) while systemic sclerosis is features of scleroderma with systemic manifestations.
Read Medstudy if you still cannot understand.

You really need some break tyagee

Quote:
Quote:
Originally Posted by Novobiocin View Post
systemic sclerosis since she has JVD secondary to Pulmonary HT >>>Right Heart Failure.

Last edited by Novobiocin; 07-10-2012 at 01:28 PM.
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Originally Posted by Novobiocin View Post
systemic sclerosis since she has JVD secondary to Pulmonary HT >>>Right Heart Failure.
what i get from this is ... patient has ss because patient has Pulmonary HT.
what i know is this...pulmonary HT points more to scleroderma rather than ss.

damn! one and for all,

please disregard all previous post and give reason why this question has systemic sclerosis. God bless you.:sorry:
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Quote:
Originally Posted by tyagee View Post
what i get from this is ... patient has ss because patient has Pulmonary HT.
what i know is this...pulmonary HT points more to scleroderma rather than ss.

damn! one and for all,

please disregard all previous post and give reason why this question has systemic sclerosis. God bless you.:sorry:
I am sorry...........I can't make you understand..........I give up!

I suggest you read Medstudy if you want to understand.
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Make it simple guys ! First the most common cause of death in systemic sclerosis which is also called diffused sclerosis is cor pulmonale which occurs due to pulmonary fibrosis whenever there is pulmonary fibrosis there is pulmonary hypertension and due to that there is cor pulmonale( right heart failure due to pulmonary dysfunction) so as JVP is distended and edema and clear lung auscultation ( clear auscultation shows that this right heart failure is not due to left heart failure- so it's cor pulmonale) and colour changes at fingers are raynaud and drugs for heartburn that he was using because of esophageal dysmotility and swelling of fingers are due to calcinosis around the fingers !
Hope this all makes sense !
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i can explain this question through scleroderma

scleroderma->Pulmonary HTN ->Cor pulmonale

rest of findings are part of CREST syndrome.

i am worried because this is uworld question and decision was made if you knw patient has scleroderma or ss.

please any1 who can throw more light and clear up the doubt.
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Quote:
Originally Posted by tyagee View Post
i can explain this question through scleroderma

scleroderma->Pulmonary HTN ->Cor pulmonale

rest of findings are part of CREST syndrome.

i am worried because this is uworld question and decision was made if you knw patient has scleroderma or ss.

please any1 who can throw more light and clear up the doubt.

U knw when crest involves lung causing pulmonary fibrosis we call it diffuse/systemic sclerosis !
If there is only crest then it's known as limited sclerosis
Hope this helps
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please any1 who can throw more light and clear up the doubt.
Read the attachment.
Attached Files
File Type: pdf Scleroderma and Systemic Sclerosis.pdf (630.7 KB)
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  #17  
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Read the attachment.
thanks. i confused scleroderma with limited sclerosis.

there are actually 3 things...scleroderma..limited and diffuse ss.

thanks
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