253 Scleroderma Flashcards
distinguished hallmark of systemic scleroderma
thick and indurated skin
what is in CREST syndrome
calcinosis cutis, Raynaud’s phenomenon, eosphageal dysmotility, sclerodactyly and telangiectasia
True or false. Systemic Scleroderma shows a strong female preponderanc which is most pronounced in child bearing years
True.
What are the scleroderma subtypes
limited and diffuse
three cardinal pathomechanistic processes underlie the protean clinical manifestations of Ssc
- diffuse microangiopathy 2. inflammation and autoimmunity, 3. visceral and vascular fibrosis
an early and possibly primary pathogenic event in Ssc that leads to protein clinical manifestation of small vessel vaculopathy
vascular injury
most common lung manifestation of Ssc
nonspecific interstitial pneumonia
most frequent extracutaneous complication of Ssc
Reynauds phenomenon
can be helpful in differentiating primary from secondary Raynauds phenomenon
nailfold capilaroscopy
nailfold capillaroscopy between primary and secondary Raynaud’s phenomenon
primary: evenly spaced parallel vascular loops while in secondary widened and irregular loops, dilated lumen, microhemorrhages and areas of vascular dropout
hallamark of Ssc that distinguishes it from other connective tissue disorder
bilateral symmetrical skin thickening
presents with accelerated hypertension accompanied by acute kidney injury and progressive failure
scleroderma renal crisis
True or false. Clinical cardiac involvement is more frequent in diffuse Scleroderma than in LcSSc
True.
True or false. Immunosuppresive agents used in other autoimmune diseases have generally shown modest to no benefit in Ssc.
True.
leading cause of death in patients with Ssc
Interstitial lung disease
