Scleroderma Flashcards
define scleroderma or systemic sclerosis
autoimmune connective tissue disorder characterized by fibrosis
etiology/RF of scleroderma
genetics
female
infectious: parvovirus and EBV
exposure: silica
subtypes of scleroderma
limited scleroderma: derm symptoms to the distal extremities (trunk sparing)
- CREST syndrome
diffuse scleroderma: whole body involvement + internal organs
population scleroderma
females
10x more rare vs lupus
age of onset 50-60Y
patho of scleroderma
- unknown trigger (viral, environmental, autoantibody, enzymes, cytokines)
- Activation of immune system vs the soft tissue
- Activated B cells → autoantibodies → anti-topoisomerase I (anti-Scl-70)
Cytokine and GF signaling - Leukocyte recruitment and ↑ fibroblasts
- Deposition of collagen and ECM proteins into tissues
- Systemic sclerosis → small vessel vasculopathy + multi-organ interstitial fibrosis
cardinal s/s scleroderma
C - calcinosis – calcium deposits underneath the skin (typically at pressure points)
R - Raynauld phenomenon
E - esophageal dysmotility
S - sclerodactyly
T - telangiectasia (spider veins)
sclerodermal renal crisis: raynauld of the kidney ➔ decrease organ perfusion ➔ kidney thinks body needs to increase BP ➔ increase renin ➔ HTN emergency
other organ involvement
- lungs: pulmonary arterial HTN and interstitial lung disease
- esophageal: barrets esophagus, strictures
- watermelon stomach – dilated blood vessels in the stomach ➔ source of bleeding
ix scleroderma
ANA
anti-Scl70 - diffuse scleroderma
anti-centromere - limited scleroderma
tx scleroderma
steroids
immunosupressants
tx based on s/s
- Raynauld ➔ CCB/viagra + avoid cold
- renal - ACEi
- derm issues ➔ emolliants
what main cx are we concerned about for scleroderma?
lung disease - leading cause of mortality ➔ pulmonary arterial HTN and interstital lung disease
renal crisis ➔ hypertensive emergency
