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REUMATO > CECIL SCLERO > Flashcards

CECIL SCLERO Flashcards

1
Q

EPIDEMIO

A

Middle-aged women

30-50a

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2
Q

Organs commonly affected

A

Hallmark - Skin

Lungs
GI tract
Kidneys
Heart

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3
Q

4 Conditions with Scleroderma-like skin induration

A

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

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4
Q

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

A

Proximal extremities

Trunk

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5
Q

In LcSSC, which manifestations precedes all others?

A

Raynaud phenomenon
Often severe
Critical ischemia

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6
Q

CREST syndrome

A 
Calcinosis cutis
Raynaud phenomenon
Esophageal dysmotility
Sclerodactyly
Teleangiectasia

Relatively good prognosis
Subset of lcSSC patients

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7
Q

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?

A

SLE+SSC+Myositis

U1-RNP

Better

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8
Q

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

A

Morphea

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9
Q

2 problems related to linear scleroderma in children

A

Growth retardation

Joint contracture

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10
Q

Strongest risk factor

A

Family history

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11
Q

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

A

Nonspecific interstitial pneumonitis

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12
Q

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

A

Disordered peristaltic activity
GE refluxand dysmotility
Gastroparesis
Small bowel obstruction

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13
Q

Centromere autoantibodies are related to which subset of the disease

A

Limited Cutaneous disease

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14
Q

Topoisomerase-I is related to which subset…

A

Diffuse cutaneous

RNA polymerase III as well

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15
Q

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

A 
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
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16
Q

Which manifestations are more pronounced in lcSSC than in DcSSC?

A

Vascular manifesations

Digital ischemia
Cutaneous teleangiextasia
PAH

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17
Q

What sort of deposits may occur and where?

A

Calcium

Finger pads
Extensor surfaces of the forearms
Olecranon
Prepatellar bursae

They may produce drainage of chalky white material

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18
Q

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

A

Vitiligo-like hypopigmentation

Salt-and-pepper changes

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19
Q

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

A

Nailfold capillaroscopy

20
Q

Most frequently GI organ affected

A

Esophagus

21
Q

3 symptoms of delayed gastric emptying

A

Early satiety
Abdominal distention
Affravated reflux symptoms

GAVE - gastric vascular ectasia - iron deficiency anemia

22
Q

What might impaired small bowel motility cause?

A

Chronic diarrhea due to bacterial overgrowth

23
Q

Malabsorption may be diagnosed by…

A

Hydrogen breath test
14C D-xylose test

serum prealbumin (transthyretin) is usegul to monitor malnutrition

24
Q

In late-stage SSc which GI emergency may occur

A

Wide-mouth colonic sacculations may perfurate and bleed

25
Q

Two major forms of lung involvement in SSC

A

Interstitial lung disease

PAH

26
Q

An autoantibody that is a risk factor for intestitial lung disease

A

Topoisomerase-I autoantibodies

27
Q

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

A

RIght heart catheterization

28
Q

Harbingers of impending scleroderma renal crisis (3)

A

Thrombocytopenia
Early-onset anemia
Pericardial effusion

GC use is associated with a more than 10-fold increase risk

29
Q

Musculoskeletal complication that may be a presenting manifestation of SSC

A

Carpal tunnel syndrome

30
Q

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

A

Breast
Lung
Ovarian carcinoma
Lymphoma

Anti-RNA polymerase III

31
Q

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

A

Vitamin K

Prealbumin

32
Q

Which autoantibody are present in virtually all patients with SSc

A

ANA

33
Q

Wich antibodies are associated with PAH

A

Anticentromere

34
Q

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

A

ACEi in Scleroderma renal crisis

35
Q

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

A

Corticosteroids

36
Q

To improve skin involvement and stabilize lung disease

A

Mycophenolate mofetil

37
Q

To reduce progression of symptomatic interstitial lung disease in early SSc

A

Cyclophosphamide

38
Q

Antifibrotic therapy

A

D-Penicillamine

Imatinib in clinical trials

39
Q

Refractory hypomotility of the small bowel may respond to subcutaneous

A

octreotide injections

40
Q

Patients with severe Raynaud phenomenon require

A

alpha1 adrenergic receptor blockers

Prazosin

41
Q

Reduces development of new ichemic ulcers

A

Bosentan

Endothelin-1

42
Q

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

A

PAH

Bosentan
5-PDE inhibitor

43
Q

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

A

Monitor their BP daily and repot significant alterations immediately

ACEi

44
Q

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

A

2 to 4 years of the disease

45
Q

Clinical hallmark of nephrogenic systemic fibrosis

A

Thickening and woody tightness of skin over the lower extremities

REUMATO (6 decks)

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