CECIL SCLERO Flashcards
EPIDEMIO
Middle-aged women
30-50a
Organs commonly affected
Hallmark - Skin
Lungs
GI tract
Kidneys
Heart
4 Conditions with Scleroderma-like skin induration
Paraneoplastic
Chronic graft-versus-host disease
Morphea - localized
Schulman disease - diffuse fasciitis with eosinophilia
In limited cutaneous SSC, which parts of the body are spared?
Proximal extremities
Trunk
In LcSSC, which manifestations precedes all others?
Raynaud phenomenon
Often severe
Critical ischemia
CREST syndrome
Calcinosis cutis Raynaud phenomenon Esophageal dysmotility Sclerodactyly Teleangiectasia
Relatively good prognosis
Subset of lcSSC patients
Mixed Connective Tissue Disorder is a combination of which three disorders? Which specific autoantibody is present in this disease? Better or worse prognosis compared to SSC?
SLE+SSC+Myositis
U1-RNP
Better
When the lcSSC occurs as single or multiple solitary patches it is called…
Morphea
2 problems related to linear scleroderma in children
Growth retardation
Joint contracture
Strongest risk factor
Family history
The most common histologic pattern in SSC-associated lung disease is
Nonspecific interstitial pneumonitis
Replacement of the normal gut architecture leads to …(4)
Disordered peristaltic activity
GE refluxand dysmotility
Gastroparesis
Small bowel obstruction
Centromere autoantibodies are related to which subset of the disease
Limited Cutaneous disease
Topoisomerase-I is related to which subset…
Diffuse cutaneous
RNA polymerase III as well
6 initial clinical manifestations of DcSSC? Which ones ensue that?
Soft tissue swelling Erythema Pruritus Fatigue Stiffness Malaise
Arthralgia Muscle weakness Carpal tunnel syndrome Raynaud - later in the disease Skin induration Hyperpigmentation Loss of body hair Impaired sweating
Which manifestations are more pronounced in lcSSC than in DcSSC?
Vascular manifesations
Digital ischemia
Cutaneous teleangiextasia
PAH
What sort of deposits may occur and where?
Calcium
Finger pads
Extensor surfaces of the forearms
Olecranon
Prepatellar bursae
They may produce drainage of chalky white material
What may happen on the skin of dark-skinned individuals?
Vitiligo-like hypopigmentation
Salt-and-pepper changes
What sort of examination is useful in distinguishing primary from secondary Raynaud phenomenon?
Nailfold capillaroscopy
Most frequently GI organ affected
Esophagus
3 symptoms of delayed gastric emptying
Early satiety
Abdominal distention
Affravated reflux symptoms
GAVE - gastric vascular ectasia - iron deficiency anemia
What might impaired small bowel motility cause?
Chronic diarrhea due to bacterial overgrowth
Malabsorption may be diagnosed by…
Hydrogen breath test
14C D-xylose test
serum prealbumin (transthyretin) is usegul to monitor malnutrition
In late-stage SSc which GI emergency may occur
Wide-mouth colonic sacculations may perfurate and bleed
Two major forms of lung involvement in SSC
Interstitial lung disease
PAH
An autoantibody that is a risk factor for intestitial lung disease
Topoisomerase-I autoantibodies
Which intervention is required to confirm the dx of PAH, assess its severity and evaluate ventricular dysfunction?
RIght heart catheterization
Harbingers of impending scleroderma renal crisis (3)
Thrombocytopenia
Early-onset anemia
Pericardial effusion
GC use is associated with a more than 10-fold increase risk
Musculoskeletal complication that may be a presenting manifestation of SSC
Carpal tunnel syndrome
Which cancers are timely related to the dx of SSc? Which autoantibody is associated with this scenario?
Breast
Lung
Ovarian carcinoma
Lymphoma
Anti-RNA polymerase III
2 lab markers that are useful to be monitored in patients with small bowel bacterial overgrowth and malabsorption
Vitamin K
Prealbumin
Which autoantibody are present in virtually all patients with SSc
ANA
Wich antibodies are associated with PAH
Anticentromere
Single drug that significantly alters a part of the natural history of SSc
ACEi in Scleroderma renal crisis
Which class of drugs should be avoided if possible for its increased risk for scleroderma renal crisis?
Corticosteroids
To improve skin involvement and stabilize lung disease
Mycophenolate mofetil
To reduce progression of symptomatic interstitial lung disease in early SSc
Cyclophosphamide
Antifibrotic therapy
D-Penicillamine
Imatinib in clinical trials
Refractory hypomotility of the small bowel may respond to subcutaneous
octreotide injections
Patients with severe Raynaud phenomenon require
alpha1 adrenergic receptor blockers
Prazosin
Reduces development of new ichemic ulcers
Bosentan
Endothelin-1
All patients with SSc should be screened for…at initial evaluation? Tx for this condition?
PAH
Bosentan
5-PDE inhibitor
Pxs with early-stage SSc and progressive skin involvement are at highest risk of scleroderma renal crisis, therefore they need to…
Tx of scleroderma renal crisis
Monitor their BP daily and repot significant alterations immediately
ACEi
Visceral organ involvement develops and progresses most rapidly during the intitial..
2 to 4 years of the disease
Clinical hallmark of nephrogenic systemic fibrosis
Thickening and woody tightness of skin over the lower extremities
