connective Flashcards

(50 cards)

1
Q

What are autoimmune connective tissue disorders?

A

A group of systemic chronic diseases targeting cellular nuclei, not just connective tissue.

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2
Q

Name some autoimmune connective tissue diseases.

A

Vasculitis, dermatomyositis, scleroderma, lupus, mixed connective tissue disease, Sjogren’s, rheumatoid arthritis.

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3
Q

What type of antibodies are commonly present in these diseases?

A

Antinuclear antibodies (ANA).

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4
Q

What symptoms indicate systemic involvement?

A

Fever, arthralgia, asthenia, CRP increase.

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5
Q

Are autoimmune connective tissue disorders more common in men or women?

A

Women.

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6
Q

What is the incidence of systemic lupus erythematosus (SLE)?

A

2-6 per 100,000.

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7
Q

What is the significance of anti-dsDNA antibodies?

A

They are specific for SLE and track disease evolution.

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8
Q

What ENA marker is linked with Sjogren’s Syndrome?

A

SSA-SSB.

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9
Q

Which ENA marker is associated with systemic sclerosis?

A

Scl-70.

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10
Q

What ENA marker indicates dermatomyositis?

A

Jo-1.

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11
Q

Can healthy individuals have ANA?

A

Yes, 5-10% of healthy people.

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12
Q

What are some cutaneous manifestations of chronic cutaneous lupus erythematosus?

A

Erythema, hyperkeratosis, atrophy.

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13
Q

What is the lupus band test?

A

A DIF test showing IgG, IgA, and complement deposits.

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14
Q

What distinguishes discoid lupus from systemic lupus?

A

Discoid is localized to the skin and lacks systemic symptoms.

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15
Q

What is chilblain lupus?

A

Cold-induced violaceous lesions without hyperkeratosis.

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16
Q

What mutation is associated with familial chilblain lupus?

A

TREX1 mutation.

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17
Q

What skin findings are typical in tumidus lupus?

A

Erythematous-edematous plaques without atrophy or hyperkeratosis.

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18
Q

Describe subacute cutaneous lupus lesions.

A

Annular, psoriasiform, policyclic papulo-squamous.

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19
Q

List clinical signs of systemic lupus erythematosus (SLE).

A

Fever, joint pain, skin lesions, kidney and CNS involvement.

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20
Q

What is Gottron’s papules?

A

Violaceous papules on dorsal surfaces of joints in dermatomyositis.

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21
Q

What is poikiloderma?

A

Combination of atrophy, hypo/hyperpigmentation, and telangiectasia.

22
Q

What signs suggest dermatomyositis?

A

Heliotrope rash, Gottron papules, poikiloderma.

23
Q

What are common malignancies associated with dermatomyositis?

A

Breast, lung, GI, prostate, nasopharynx.

24
Q

What type of muscle involvement occurs in dermatomyositis?

A

Proximal symmetric muscle weakness.

25
What autoantibody is common in dermatomyositis?
Myositis-specific autoantibodies.
26
What characterizes scleroderma?
Inflammation, fibrosis, and atrophy of skin and organs.
27
What is morphea?
Localized scleroderma presenting as lilac ringed annular lesions.
28
What is linear scleroderma?
Localized sclerosis resembling a sabre stroke (en coup de sabre).
29
What is Atrophoderma of Pasini and Pierini?
Multiple atrophic plaques, a variant of morphea.
30
What are phases of systemic scleroderma skin involvement?
Edematous, sclerotic, and atrophic phases.
31
What are common facial features of scleroderma?
Taut skin, beaked nose, microstomia.
32
What autoantibody is associated with CREST syndrome?
Anti-centromere antibody (ACA).
33
What does anti-Scl70 indicate?
Diffuse systemic sclerosis with lung fibrosis.
34
What is anti-fibrillarin linked with?
Heart and lung involvement in diffuse systemic sclerosis.
35
What autoantibody indicates renal involvement in scleroderma?
Anti-RNA polymerase I and II.
36
What diagnostic tools assess systemic sclerosis?
Capillaroscopy, EMG, ECG, CT scan, pHmetry.
37
What is vasculitis?
Inflammation of blood vessels causing ischemia and tissue damage.
38
What are signs of vasculitis?
Palpable purpura, necrosis, ulceration.
39
What is Behçet’s disease?
A vasculitis with ulcers, HLA-B51 link, common along Silk Road.
40
What is a characteristic oral lesion in Behçet’s disease?
Aphthous ulcers.
41
What condition may cause lupus pernio?
Sarcoidosis.
42
What autoantibody is found in drug-induced lupus?
Anti-histone antibodies.
43
What skin change occurs in cutaneous calcinosis?
Calcium deposits in the skin.
44
What is the V-neck sign in dermatomyositis?
Photodistributed poikiloderma on the chest.
45
What is the shawl sign in dermatomyositis?
Poikiloderma on upper back.
46
What is sclerodactyly?
Thickening and tightening of the fingers in systemic sclerosis.
47
What are sausage fingers?
Swollen fingers in systemic sclerosis.
48
What is microstomia?
Reduced mouth opening due to skin tightening.
49
What distinguishes systemic from cutaneous lupus?
Systemic involves organs and has anti-dsDNA antibodies; cutaneous does not.
50
What is the CREST acronym in systemic sclerosis?
Calcinosis, Raynaud, Esophageal dysfunction, Sclerodactyly, Telangiectasia.