Thread by @MohitHarshMD

Hey #MedTwitter fam!

Time for the inaugural #MedDermTwagTeam #tweetorial on #Scleroderma

This is a diffuse (get it?) topic so @RashDecisionz & I will try to keep it focused

I’ll tweet on illness script

S/O to @CPSolvers @MedEdTwagTeam and many others for the inspiration!
1/12

Who else gets confused with the terminology? I sure do

The confusion may arise because scleroderma is a spectrum of disorders. Also, people say Scleroderma interchangeably with #SystemicSclerosis

The subtype is based on extent of skin & organ involvement

2/12

Let’s focus on Systemic Sclerosis (SSc)

SSc is a multi-organ, progressive vasculopathy and fibrosing condition with skin being main target

Earliest sign is Raynaud phenomenon, occurs in 95% of pts

Skin thickening w/o Raynauds makes SSc unlikely

nature.com/articles/s4158…

3/12

MSK in SSc💪🏾

Joint involvement occurs in up to 65% of SSc pts and is hand/wrist predominant, can impact DIP

SSc associated myositis occurs in 10-15% pts and impacts prox muscles. There is a strong association w/ myositis and myocardial involvement

pubmed.ncbi.nlm.nih.gov/7236323/

4/12

GI in SSc💩

~80% of pts have esophageal dysmotility +/- GERD

Esophageal dysmotility is common in all forms of SSc

Dysmotility can occur in small intestine and colon.

Interestingly, esophageal involvement may contribute to pathogenesis of ILD

ncbi.nlm.nih.gov/pmc/articles/P…

5/12

Kidney in SSc

Scleroderma Renal Crisis affects 5% of pts and previously was main cause of mortality

Presentation includes HTN emergency, possible MAHA, proteinuria

Risk factors: Diffuse SSc, anti-RNA polymerase III Ab, use of high dose steroids

ncbi.nlm.nih.gov/books/NBK48242…

6/12

Lung in SSc

ILD is leading cause of mortality

90% of SSc pts develop interstitial changes on HRCT, 70% w/ ILD

Highest risk w/ anti-Scl-70 ab and limited cutaneous systemic sclerosis

Pulm HTN occurs 2/2 ILD or can be isolated PAH in 13% of pts

ncbi.nlm.nih.gov/pmc/articles/P…

7/12

❤️ in SSc

More common in men, with pericarditis occurring in 10% of pts

Myocardial fibrosis can occur from vascular vasospasm and myocardial ischemia

8/12

How about those pesky Ab?

ANA + in 95% of pts, so if - think other Dx

Anti-scl-70 + higher risk for ILD in diffuse cutaneous SSc

Anti-RNA polymerase III + higher risk for renal crisis and worse skin dz

Anti-centromere + usually in limited cutaneous SSc

All 95% specific

9/12

With any rheum condition, can’t hang your hat on antibodies alone.

There was a consensus classification created for research purposes by ACR that can be helpful.

The sensitivity and specificity reaches close to 92% when the total score = 9

ncbi.nlm.nih.gov/pmc/articles/P…

10/12

Conceptually, I think about scleroderma as tightening and fibrosis of organs

Fibrosis of vasculature - raynauds, ulceration, HTN

Fibrosis of lungs - ILD

Tightening of GI tract - dysmotility, stricture

The obvious tightening of skin, etc

Early eval by rheum is key!

11/12

This ends the overview of systemic sclerosis spectrum of disease. I hope this helped create your own illness script

Always welcome to feedback for improvement

Up next is @RashDecisionz who will discuss more specifics on skin manifestations of the disease

#Eam2LearnMo

12/12

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