An interesting case-based review👀➡️Acute peripheral/cutaneous ischemic syndrome (APCIS), what rheumatologist should know?

APCIS is a clinical syndrome, representing the clinical appereance of skin and/or limb severe ischemia due to several etiologies.

https://pubmed.ncbi.nlm.nih.gov/33315786/ 
➡️Vessel wall abnormalities: atherosclerosis, calciphylaxis, vasculitis.
➡️Embolism: cardiac, aneurysms, cholesterol emboli.
➡️Local thrombosis: hypercoagulable states.
➡️Dysproteinemias.
➡️Venous limb gangrene: extensive venous thrombosis + compromise in arterial flow.
Clinical conditions relevant to the rheumatologist:
➡️Antiphospholipid syndrome.
➡️Vasculitis.
➡️Autoimmune diseases (SLE).
➡️Cryoproteinemias (cryoglobulinemia).
➡️Others: DADA2...
Diagnosis:
➡️ Rule out infections, emboli, atherothrombotic disorders!
➡️ Blood tests, blood smear, blood cultures, coagulation panel, autoantibodies, cryoglobulins, search for monoclonal proteins...
➡️Echocardiogram, arterial/venous ultrasound, EKG.
➡️Consider tissue biopsy.
After initial workout, in patients without evident infection or atherothrombotic/embolic disorders, suggesting an autoimmune pathology (APS, vasculitis...):
➡️ Anticoagulation + low-dose aspirin + high dose steroids (not in SSc).
➡️ Vasodilators ( prostacyclins...).
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