10 - 63 - SYSTEMIC SCLEROSIS Flashcards
almost always present and are important for the early diagnosis of scleroderma
- Raynaud phenomenon,
- circulating autoantibodies,
- skin sclerosis
most frequently affected organs in scleroderma
Skin, esophagus, lung, heart, and kidneys
hallmark complications of scleroderma
- hypertensive scleroderma renal crisis (SRC),
- pulmonary arterial hypertension (PAH),
- pulmonary fibrosis (PF),
- GI dysmotility
- defined as a progressive form of SSc with an early onset of RP, usually within 1 year of onset of skin thickening
- This subset is characterized by rapid skin involvement of trunk, face, upper arms, and thighs
- there is a higher propensity to develop PF, cardiac involvement, and SRC
Diffuse cutaneous SSc
antibodies frequently positive in Diffuse cutaneous SSc
anti–scleroderma 70 (antitopoisomerase-I) or anti-RNA polymerase III antibodies
- characterized by a long preexisting history of RP and skin changes of the extremities distal to the knee and elbow joints, including facial skin
Limited cutaneous SSc
- The traditional acronym CREST (calcinosis, RP, esophageal dysmotility, sclerodactyly, and telangiectasias) is assigned to the limited form of SSc
antibodies often positive in Limited cutaneous SSc
anticentromere antibodies
subset of scleroderma that is frequently associated with isolated PAH
Limited cutaneous SSc
- defined by 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
undifferentiated SSc
A very small proportion of cases (1.5%) develop vascular (RP and/or PAH), immunologic (most commonly anticentromere antibodies), and organ-based fibrotic features of SSc, but do not show skin sclerosis
SSc sine scleroderma
Patients with features of scleroderma together with those of another autoimmune rheumatic disease
SSc overlap syndrome
disease occurring with clinical aspects of SSc (according to the ACR criteria) or main symptoms of SSc simultaneously with those of other connective tissue diseases or other autoimmune diseases, such as dermatomyositis, Sjögren syndrome, systemic lupus erythematosus, vasculitis, and polyarthritis
SSc overlap syndrome
- these patients present mostly high titers of anti-U1RNP, anti-nRNP, antifibrillarin, or anti-PmScl antibodies.
- This group of patients includes well-defined patients suffering from mixed connective tissue disease (MCTD), characterized by high titers of circulating** anti-U1RNP antibodies**
most frequent triggers of raynaud phenomenon attacks
coldness and emotional stress
the extent and severity of skin sclerosis can be assessed by what score?
modified Rodnan skin score
- Skin score at baseline correlates with disease severity and outcome in diffuse cutaneous SSc.
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
RAYNAUD PHENOMENON
- RP clinically appears suddenly and 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 a RP attack, in some cases cyanosis (triphasic RP) also ensues
cardinal feature of SSc
Skin involvement
Skin involvement is a cardinal feature of SSc and usually appears first in what areas?
fingers and hands
This is the major external feature of structural vessel disease, probably attributable to thickened intima and lumen-occluded vessels.
digital ulceration
currently the most common cause of disease-related death in SSc
PAH
most common internal organ involvement in patients suffering from both limited and diffuse SSc
GI involvement
strongest risk factor fir SSc
positive family history for SSc
histopath findings of SSc
fibrosis of the lower two-thirds of the dermis and the subcutaneous fibrous trabeculae, because of excessive deposition of extracellular matrix (ECM) proteins
The histopathology of SSc shows fibrosis of the lower two-thirds of the dermis and the subcutaneous fibrous trabeculae, because of excessive deposition of extracellular matrix (ECM) proteins, most notably what types of collagen
collagen Types I and III
Vasculopathy in SSc is an early event and is based on inappropriate vascular remodeling and repair processes.
Vascular abnormalities are characterized by what?
vasoconstriction, adventitial and intimal proliferation, inflammation, and thrombosis
key event in the development of fibrosis
induction of fibroblasts into activated myofibroblast
gold standard, but is an invasive diagnostic procedure to determine PAH
Right-heart catheterization
T/F
PAH is defined as a mean pulmonary artery pressure of ≥25 mm Hg at rest together with a p**ulmonary capillary wedge pressure of ≤15 mm Hg **as determined by right-heart catheterization
True
mainstay of therapy for critical digital ischemia
Prostacyclin derivatives by IV infusion
Key elements in the management of skin manifestations of SSc
physical therapy and regular exercise
to maintain circulation, joint mobility, and muscle strength, all aimed at improving the quality of life of SSc patients
best predictor of future decline in lung function
extent of disease by high resolution CT and a history of progressive restrictive abnormality
