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Fitz 9th > 63 - Systemic Sclerosis > Flashcards

63 - Systemic Sclerosis Flashcards

1
Q

(Women/Men) are more frequently affected by systemic sclerosis

A

Women

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2
Q

Age onset of systemic sclerosis

A

Between 30 and 50 years

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3
Q

Y/N: Systemic sclerosis has the highest case-specific mortality of any of the autoimmune rheumatic diseases

A

Yes

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4
Q

Systemic sclerosis usually starts with

A

Raynaud phenomenon

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5
Q

Hallmark complications of systemic sclerosis

A

Hypertensive scleroderma renal crisis
Pulmonary arterial hypertension
Pulmonary fibrosis
GI dysmotility

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6
Q

For the diagnosis of systemic sclerosis to be proven, the American College of Rheumatology classification (1980) requires

A

Either 1 major criterion or

At least 2 minor criteria

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7
Q

Major criterion according to the American College of Rheumatology classification (1980)

A

Scleroderma proximal to the metacarpophalangeal or metatarsophalangeal joints

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8
Q

Minor criteria according to the American College of Rheumatology classification (1980)

A

Sclerodactyly
Digital ulcerations and/or pitting digital scars
Bibasilar pulmonary fibrosis

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9
Q

The new ACR/European League Against Rheumatism criteria considered several additional criteria such as

A

Abnormal nailfold capillaries
Fingertip lesions
Autoantibodies

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10
Q

Defined as a progressive form of SSc with an early onset of RP, usually within 1 year of onset of skin thickening

A

Diffuse cutaneous SSc

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11
Q

Diffuse cutaneous SSc shows very freuqently _____ antibodies

A

Anti-scleroderma 70 (antitoposiomerase-I) or

Anti-RNA polymerase III

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12
Q

Diffuse cutaneous SSc has a higher propensity to develop

A

Pulmonary fibrosis
Cardiac involvement
Scleroderma renal crisis

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13
Q

Characterized by a long preexisting history of RP and skin changes of the extremities distal to the knee and elbow joints, including facial skin

A

Limited cutaneous SSc

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14
Q

Limited cutaneous SSc often presents with _____ antibodies

A

Anticentromere

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15
Q

Limited cutaneous SSc is frequently associated with

A

Isolated pulmonary arterial hypertension

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16
Q

CREST is a _____ form of SSc

A

Limited

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17
Q

CREST meaning

A 
Calcinosis
RP
Esophageal dysmotility
Sclerodactyly
Telangiectasias
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18
Q

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

A

Early or undifferentiated SSc

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19
Q

Develop vascular (RP and/or PAH), immunologic (most commonly anticentromere antibodies), and organ-based fibrotic features of SSc, but do not show skin sclerosis

A

SSc sine scleroderma

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20
Q

Characterized by high titers of anti-U1RNP antibodies

A

Mixed connective tissue disease

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21
Q

Y/M: MCTD has poor response to antiinflammatory/anti-immune therapy and the prognosis is clearly worse than in patients with scleroderma

A

No - good response

better than in patients with classic scleroderma

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22
Q

Usually characterized by specific Pm-Scl autoantibodies, have typical mechanic hands, and develop early intense subcutaneous calcifications

A

Patients with sclerodermatous lesions who also suffer from intense myositis

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23
Q

Y/N: Similar to MCTD patients, patients with sclerodermatous lesions who also suffer from intense myositis respond well to an early antiinflammatory treatment

A

Yes

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24
Q

The extent and severity of skin sclerosis can be assessed by the

A

Modified Rodnan skin score

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25
Q

Y/N: Skin score at baseline correlates with disease severity and outcome in diffuse cutaneous SSc

A

Yes

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26
Q

Raynaud phenomenon appears in more than _____% of SSc patients

A

90

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27
Q

Skin involvement is a cardinal feature of SSc and usually appears first in the

A

Fingers and hands

28
Q

Nonpitting edema of the fingers

A

Puffy fingers

29
Q

Induration and skin thickening

A

Sclerodactyly

30
Q

Restricted mobility of joints

A

Dermatogenous contractures

31
Q

Reduced mouth aperture

A

Microstomia

32
Q

Abnormal deposition of cutaneous and/or subcutaneous calcium

A

Calcinosis cutis

33
Q

Calcinosis cutis next to joints

A

Thibierge-Weissenbach syndrome

34
Q

Hypopigmented and hyperpigmented skin

A

Salt-and-pepper

35
Q

Loss of hair follicles and sweat glands

A

Hypohidrosis/anhidrosis

36
Q

Most common cause of disease-related death in SSc

A

Pulmonary arterial hypertension

37
Q

PAH occurs in both limited and diffuse cutaneous subsets, although the most typical cases are those of

A

Limited SSc associated with isolated PAH

38
Q

Most cases have PAH, but there are some patients with late-stage extensive _____ in SSc that develop a true secondary pulmonary hypertension

A

Interstitial lung fibrosis

39
Q

Most common internal organ involvement in SSc

A

GI

40
Q

Drugs that should be avoided in patients suffering from SSc

A

Nephrotoxic drugs

High-dose prednisolone (>7.5 mg/day)

41
Q

Several studies suggest that _____ is the strongest risk factor for SSc

A

Positive family history

42
Q

Scleroderma-like syndromes have been reported in association with

A

Solvents (vinyl chloride, benzene, toluene, epoxy resins)
Drugs (bleomycin, carbidopa, pentazocine, cocaine, docetaxel, metaphenylenediamine)
Coal, gold, silicon

43
Q

Vasculopathy in SSc is a/an (early/late) event and is based on inappropriate vascular remodeling and repair processes

A

Early

44
Q

Early lesions in the microcirculation because of structural damage are initially seen in

A

Nailfold capillaries

Vasospastic responses in RP

45
Q

The first inflammatory infiltrates in lesional skin are predominantly

A

Cells of the monocyte lineage (T cells, macrophages, B cells, mast cells)

46
Q

T cells are predominantly _____, and show a predominant _____ phenotype

A

CD4+

T-helper 2

47
Q

Several studies suggest that B cells are able to induce ECM production through secretion of

A

IL-6

TGF-beta

48
Q

Patients presenting only with RP should be studied for

A

Capillary alterations
Autoantibody status

*Predictors for the development of SSc

49
Q

Number of sites assessed in the modified Rodnan score

A

17

50
Q

Most important techniques to determine possible cardiopulmonary involvement

A

Pulmonary functions tests

51
Q

To determine the presence of interstitial lung involvement, _____ should be used

A

HRCT and/or

Thoracic radiography

52
Q

Gold standard to determine PAH

A

Right-heart catheterization

53
Q

Used to detect right ventricular impairment

A

N-terminal brain natriuretic peptide

54
Q

The best evidence available for general immunosuppression is for

A

Cyclophosphomide

55
Q

Y/N: At present, there is no proven antifibrotic agent

A

Yes

56
Q

Should be considered first-line therapy for RP

A

Calcium channel blockers

Angiotensin II receptor antagonists

57
Q

Mainstay of therapy for critical digital ischemia

Help to heal digital ulcers and may prevent recurrent lesions

A

Parenteral prostacyclin derivatives (iloprost)

58
Q

Also used especially in critical digital ischemia

A

Antiplatelet agents (aspirin, clopidogrel)

59
Q

Therapies that are effective for PAH and digital vasculopathy

A

Oral dual-specificity endothelin receptor antagonist (bosentan)
Phosphodiesterase type 5 inhibitors (sildenafil, tadalafil)

60
Q

Surgical treatments include ____, which can benefit single fingers with refractory ulcers

A

Digital microarteriolysis

61
Q

May be helpful for lower-limb RP or ulceration

A

Lumbar sympathectomy

62
Q

Key elements in the management of skin manifestations of SSc

A

Physical therapy and regular exercise

63
Q

Ultraviolet _____ phototherapy appears to inhibit fibrotic and inflammatory processes and reduce the amount of sclerotic skin

A

A1

64
Q

High-dose treatment with domperidone is associated with increased risk of

A

Cardiac arrhythmia

65
Q

Distinguishes SRC from other causes of end-stage renal failure

A

Possibility of late recovery

66
Q

Routine therapy for SRC

A

Angiotensin-converting enzyme inhibitors

Fitz 9th (108 decks)

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