Connective Tissue Disorders Flashcards

1
Q

Sjogrens Syndrome (SS)

A

Chronic inflammatory disorder of the lacrimal and salivary glands.
Dry mouth, dry eyes
Mostly primary

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2
Q

Sicca complex

A

Dry eyes

Dry mouth

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3
Q

Sjogrens Epidemiology

A

All age groups

Women (9:1) in 4th and 5th decade

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4
Q

Sjogrens patho

A

Lymphocytic infiltrate into glands and impair function

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5
Q

Sjogrens extraglandular involvement

A

Vaginal dryness
Interstitial nephritis
Rhinitis, sinusitis
Dry cough

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6
Q

Antibodies for SS

A

Anti-Ro/SSA (best) and

Anti-La/SSB

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7
Q

Scleroderma

A

Thickened, hardened skin, commonly associated w/ systemic involvement

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8
Q

Cutaneous manifestations tx

A

Nothing can reverse the sclerosis.

Antihistamines for pruritis

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9
Q

Most common initial manifestation of scleroderma?`

A

Raynauds Phenomenon

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10
Q

Raynauds Phenomenon (RP)

A

Vasoconstriction from cold or emotional stress.

Can be white or blue

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11
Q

Tx for raynauds?

A

CCB
Prevention
Alpha blockers
low dose ASA

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12
Q

Systemic Sclerosis GI

A

GERD like sx

Tx with PPI

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13
Q

Systemic Sclerosis Musculoskeletal

A

Erosive, inflammatory arthritis

Methotrex to tx

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14
Q

Pulm manifestations of systemic sclerosis

A

Lung fibrosis common
Ground glass pattern on CT
Pulm HTN, lung CA
Cyclophosphamide to tx

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15
Q

Which meds should not be used for renal sx in systemic sclerosis pts?

A

DONT use diuretics

Use ACE

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16
Q

CREST

A
Calcinosis
Raynauds
Esophageal dysmotility
Sclerodactyly
Telangiectasia
17
Q

CREST syndrome

A

Refers to limited cutaneous form of scleroderma

Pts may go on to develop pulm HTN and CHF

18
Q

Polymyositis

A

Inflammatory muscle dz without a cutaneous eruption

19
Q

Dermatomyocytis

A

Inflammatory muscle dx WITH cutaneous eruption.

20
Q

Poly/Dermatomyositis

A

Inflammatory damage of myocytes leads to proximal muscle weakness (most common presenting feature)

21
Q

Gottrons Sign

A

Rash over PIP and MCP joints

22
Q

Poly/Dermatomyositis Eyelid rash

A

Purple heliotrope eruption over eyelids.

23
Q

Shawl sign

A

Rash over upper anterior and around shoulders

24
Q

Poly/Dermatomyositis S/S

A

Insidious onset of symmetric, proximal muscle weakness.

Myalgias and muscle tenderness

25
Q

Poly/Dermatomyositis Labs

A

CK, LDH, ASl, ALT

ANA in 80%

26
Q

Where are the cellular infiltrates in Poly/dermatomyositis

A

Dermato: around vessels
Poly: within the fascicle

27
Q

Poly/Dermatomyositis tx

A

Steroids