Systemic Sclerosis Flashcards
What is the other word for systemic sclerosis?
Scleroderma
What are the 3 characteristics of Scleroderma?
- Vascular endothelial injury
- inflammation
- extensive activation of fibroblasts
What are the 5 most frequently affected organs in scleroderma?
- skin
- esophagus
- lungs
- heart
- kidneys
CREST?
- Calcinosis
- Raynaud’s Phenomenon
- Esophageal dysmotility
- Sclerodactyly (a scleroderma in which the fingers become thin and shiny with sclerotic skin at the tip, which is due to subcutaneous and intracutaneous calcinosis and diffused fibrosis of the collagen)
- Telangiectasias
Diffuse cutaneous SSC?
- progressive form of SSC with an EARLY onset of RAYNAUD PHENOMENON (usually within 1 year of onset of skin thickening)
- Characterized by rapid involvement of trunk, face, upper arms, and thighs
- higher propensity to develop: (CSP)
- PF (pulmonary fibrosis)
- Cardiac involvement
- SRC (scleroderma renal crisis)
antibody associated with diffuse cutaneous SSC?
anti-scleroderma 70 (antitopoisomerase-I) or anti-RNA polymerase III antibodies
Limited cutaneous SSc
characterized by a LONG pre existing history of RAYNAUD PHENOMENON and
skin changes of the extremities distal to the knee and elbow joints, including facial skin
Limited cutaneous SSC antibody?
presents with anti-centromere antibodies (50-70% of cases)
Limited cutaneous SSC is frequently associated with?
frequently associated with isolated PAH (Pulmonary arterial hypertension)
What do you call early SSC?
It is also known as undifferentiated SSc
early SSC or undifferentiated SSc is defined as?
positive RP and
at least 1 additional feature of SSc (positive nailfold capillary alterations, puffy fingers, pulmonary hypertension) and/or detectable scleroderma-associated autoantibodies without fulfilling the ACR criteria.
give the additional features of scleroderma.
1) positive nailfold capillary alterations
2)puffy fingers
3)pulmonary hypertension
and/or
detectable scleroderma-associated autoantibodies without fulfilling the ACR criteria
Define SSc sine scleroderma.
a very small proportion (1.5%) develop
- vascular (RP and/or PAH),
- immunologic (most commonly ANTICENTROMERE ANTIBODIES), and
- organ based fibrotic features of SSC
DO NOT SHOW SKIN SCLEROSIS
Raynaud phenomenon appears in what percentage of SSc patients?
appears in more than 90% of SSc patients
How do you define raynaud phenomenon?
it is defined by recurrent attacks of vasospasm of small digital arterioles/arteries at fingers and toes
usually caused by cold and/or other stimuli, for example, emotional stress
How does RP clinically appear?
RP clinically appears suddenly
It is clearly restricted and is accompanied by painful pallor/ischemia of single or several digits/toes
Followed by reactive hyperemia after reheating at the end of an attack
In some cases, cyanosis (triphasic RP) also ensues
What is a cardinal feature of SSc?
Skin involvement is a cardinal feature of SSc
Where does skin involvement in SSc usually appear?
It appears first in the fingers and hands
What kind of edema of the fingers do patients with SSc usually develop?
Patient usually develop nonpitting edema of the fingers (puffy fingers), hands and extremities followed by an increasing induration and skin thickening (sclerodactyly)
Depending on the localization of skin thickening, restricted mobility of joints (dermatogeneous contractures), and/or restricted breath excursion may be present
Depending on the localization of skin thickening, restricted mobility of joints (dermatogenous contractures), and/or restricted breath excursion may be present.
Typical facial features of SSc? (TBR)
- telangiectasias,
- a beak-shaped nose, and r
- reduced mouth aperture (microstomia)
typical facial appearance of SSc patients?
- radial furrowing around the mouth
- no expression
- a stiff and mask-like facial appearance
- sclerosis of the frenulum