Yesterday we had an amazing noon conference on scleroderma with Dr Bahce! We discussed from pathophysiology to the differential diagnosis and common complications...here are some tips and pearls!
1/n (systemic sclerosis)
Although there were many reports of patient with symptoms compatible with sclerodermia, the term was coined in the beggining of the 19th century by Fantonetti and Thirial.
However, the skin was though to be the only involved organ, with no relationship with other symptoms.
2/n
Osler described it as "scleroderma is one of the
most terrible of all human ills. The disease progress until one is literally a mummy, encased in an evershrinking, slowly contracting skin of steel, is a fate not pictured in any tragedy, ancient or modern"
3/n
However, sclerodermia is an autoimmune disease with significant compromise of microvasculature and a dearranged response to the inflammatory insult, with abnormal CD4+ Th2 response...check the amazing Nature primer on this disease!
nature.com/articles/nrdp2…
4/n
Lungs, GI tract, kidney and the endotelium can be compromise, and ILD, PAH are the main causes of death systemic sclerosis (SSc)...And the degree of compromise can present a wide range, from limited, cutaneous disease involving a small area to a multysistemic disease.
5/n
...However, not everything that causes skin fibrosis is SSc. Dr Bahce presented a nice list of conditions that can present as SSc.
6/n
Not all SSc is the same. Different clinical phenotype have different clinical evolution, rate of complications, and auto antibodies detected...
7/n
Some of the basics findings to consider are sclerotic skin in hands/distal extremities, decrease in mouth aperture, loose of skin pigment (poikiloderma, don't confuse it with vitiligo!), dyspnea, crackles on auscultation and nailfold capillary changes (use a dermatoscope!).
8/n
Treatment depends on the type of SSc and can invovle MTX, Hydroxichloroquine, steroids, Cyclophosphamide (CYC) or Mycofenolate Mofetil.
Take a look at this simplified approach...
9/n
Thanks to Dr Bahce for the conference...let us know what you think!....
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