CECIL SCLERO

Question 1

EPIDEMIO

Answer 1

Middle-aged women

30-50a

Question 2

Organs commonly affected

Answer 2

Hallmark - Skin

Lungs
GI tract
Kidneys
Heart

Question 3

4 Conditions with Scleroderma-like skin induration

Answer 3

Paraneoplastic
Chronic graft-versus-host disease
Morphea - localized
Schulman disease - diffuse fasciitis with eosinophilia

Question 4

In limited cutaneous SSC, which parts of the body are spared?

Answer 4

Proximal extremities

Trunk

Question 5

In LcSSC, which manifestations precedes all others?

Answer 5

Raynaud phenomenon
Often severe
Critical ischemia

Question 6

CREST syndrome

Answer 6

Calcinosis cutis
Raynaud phenomenon
Esophageal dysmotility
Sclerodactyly
Teleangiectasia

Relatively good prognosis
Subset of lcSSC patients

Question 7

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?

Answer 7

SLE+SSC+Myositis

U1-RNP

Better

Question 8

When the lcSSC occurs as single or multiple solitary patches it is called…

Answer 8

Morphea

Question 9

2 problems related to linear scleroderma in children

Answer 9

Growth retardation

Joint contracture

Question 10

Strongest risk factor

Answer 10

Family history

Question 11

The most common histologic pattern in SSC-associated lung disease is

Answer 11

Nonspecific interstitial pneumonitis

Question 12

Replacement of the normal gut architecture leads to …(4)

Answer 12

Disordered peristaltic activity
GE refluxand dysmotility
Gastroparesis
Small bowel obstruction

Question 13

Centromere autoantibodies are related to which subset of the disease

Answer 13

Limited Cutaneous disease

Question 14

Topoisomerase-I is related to which subset…

Answer 14

Diffuse cutaneous

RNA polymerase III as well

Question 15

6 initial clinical manifestations of DcSSC? Which ones ensue that?

Answer 15

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

Question 16

Which manifestations are more pronounced in lcSSC than in DcSSC?

Answer 16

Vascular manifesations

Digital ischemia
Cutaneous teleangiextasia
PAH

Question 17

What sort of deposits may occur and where?

Answer 17

Calcium

Finger pads
Extensor surfaces of the forearms
Olecranon
Prepatellar bursae

They may produce drainage of chalky white material

Question 18

What may happen on the skin of dark-skinned individuals?

Answer 18

Vitiligo-like hypopigmentation

Salt-and-pepper changes

Question 19

What sort of examination is useful in distinguishing primary from secondary Raynaud phenomenon?

Answer 19

Nailfold capillaroscopy

Question 20

Most frequently GI organ affected

Answer 20

Esophagus

Question 21

3 symptoms of delayed gastric emptying

Answer 21

Early satiety
Abdominal distention
Affravated reflux symptoms

GAVE - gastric vascular ectasia - iron deficiency anemia

Question 22

What might impaired small bowel motility cause?

Answer 22

Chronic diarrhea due to bacterial overgrowth

Question 23

Malabsorption may be diagnosed by…

Answer 23

Hydrogen breath test
14C D-xylose test

serum prealbumin (transthyretin) is usegul to monitor malnutrition

Question 24

In late-stage SSc which GI emergency may occur

Answer 24

Wide-mouth colonic sacculations may perfurate and bleed

Question 25

Two major forms of lung involvement in SSC

Answer 25

Interstitial lung disease | PAH

Question 26

An autoantibody that is a risk factor for intestitial lung disease

Answer 26

Topoisomerase-I autoantibodies

Question 27

Which intervention is required to confirm the dx of PAH, assess its severity and evaluate ventricular dysfunction?

Answer 27

RIght heart catheterization

Question 28

Harbingers of impending scleroderma renal crisis (3)

Answer 28

Thrombocytopenia Early-onset anemia Pericardial effusion GC use is associated with a more than 10-fold increase risk

Question 29

Musculoskeletal complication that may be a presenting manifestation of SSC

Answer 29

Carpal tunnel syndrome

Question 30

Which cancers are timely related to the dx of SSc? Which autoantibody is associated with this scenario?

Answer 30

Breast Lung Ovarian carcinoma Lymphoma Anti-RNA polymerase III

Question 31

2 lab markers that are useful to be monitored in patients with small bowel bacterial overgrowth and malabsorption

Answer 31

Vitamin K | Prealbumin

Question 32

Which autoantibody are present in virtually all patients with SSc

Answer 32

ANA

Question 33

Wich antibodies are associated with PAH

Answer 33

Anticentromere

Question 34

Single drug that significantly alters a part of the natural history of SSc

Answer 34

ACEi in Scleroderma renal crisis

Question 35

Which class of drugs should be avoided if possible for its increased risk for scleroderma renal crisis?

Answer 35

Corticosteroids

Question 36

To improve skin involvement and stabilize lung disease

Answer 36

Mycophenolate mofetil

Question 37

To reduce progression of symptomatic interstitial lung disease in early SSc

Answer 37

Cyclophosphamide

Question 38

Antifibrotic therapy

Answer 38

D-Penicillamine Imatinib in clinical trials

Question 39

Refractory hypomotility of the small bowel may respond to subcutaneous

Answer 39

octreotide injections

Question 40

Patients with severe Raynaud phenomenon require

Answer 40

alpha1 adrenergic receptor blockers Prazosin

Question 41

Reduces development of new ichemic ulcers

Answer 41

Bosentan | Endothelin-1

Question 42

All patients with SSc should be screened for...at initial evaluation? Tx for this condition?

Answer 42

PAH Bosentan 5-PDE inhibitor

Question 43

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

Answer 43

Monitor their BP daily and repot significant alterations immediately ACEi

Question 44

Visceral organ involvement develops and progresses most rapidly during the intitial..

Answer 44

2 to 4 years of the disease

Question 45

Clinical hallmark of nephrogenic systemic fibrosis

Answer 45

Thickening and woody tightness of skin over the lower extremities