Connective Tissue Disorders Flashcards

1
Q

Define systemic lupus erythematosus (SLE)

A

an autoimmune disease in which the body’s immune system mistakenly attacks healthy tissue. It can affect the skin, joints, kidneys, brain, and other organs.

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2
Q

Define Limited scleroderma (=CREST)

A

one subtype of scleroderma — a condition that literally means “hardened skin.” The skin changes associated with limited scleroderma typically occur only in the lower arms and legs and sometimes the face and throat. Limited scleroderma can also affect your digestive tract.

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3
Q

Define Diffuse scleroderma

A

a type of systemic sclerosis (scleroderma). Systemic scleroderma can affect any part of the body. It often affects the skin, blood vessels, and internal organs, especially the lungs, heart, kidneys, and gastrointestinal tract.

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4
Q

Define polymyositis

A

(“inflammation of many muscles”) is a type of chronic inflammation of the muscles (inflammatory myopathy) related to dermatomyositis and inclusion body myositis.

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5
Q

Define dermatomyositis

A

an uncommon inflammatory disease marked by muscle weakness and a distinctive skin rash.

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6
Q

What is the HLA associated with SLE?

A

HLA-DR3

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7
Q

What is the HLA associated with scleroderma (limited & diffuse)?

A

HLA-DR5 & DRw8

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8
Q

What is the main clinical feature of scleroderma?

A

Skin fibrosis/hard skin

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9
Q

Polymyositis & dermatomyositis is associated with?

A

Underlying malignancy

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10
Q

What hypersensitivity reaction is SLE?

A

Type III -immune complex (tissue damage caused by the activation of complement in response to antigen-antibody (immune) complexes that are deposited in tissues.)

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11
Q

What are the 4 key features of connective tissue disease?

A
Inflammation
Fibrosis/scarrign
Vasospasm (including Reynaud's)
Vascular thrombosisInflammation
Fibrosis/scarrign
Vasospasm (including Reynaud's)
Vascular thrombosis
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12
Q

Risk factors for SLE?

A

Drug-induced
Increased in classical complement deficiencies
In afrocarribean
FemalesDrug-induced
Increased in classical complement deficiencies
In afrocarribean
Females

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13
Q

Autobodies associated with SLE:

A
ANA (95%)
Anti dsDNA
Anti-Sm
Drug-induced
Anti-histoneANA (95%)
Anti dsDNA
Anti-Sm
Drug-induced
Anti-histone
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14
Q

Autobodies associated with Limited scleroderma CREST:

A

Anti-centromere

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15
Q

Autobodies associated with diffuse scleroderma:

A
Anti Scl-70
Fibrillarin
RNA pol I, II, III
PM-SclAnti Scl-70
Fibrillarin
RNA pol I, II, III
PM-Scl
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16
Q

Autobodies associated with Polymyositis & dermatomyositis:

A

Anti Jo-1 (=tRNA synthetase)

17
Q

Histolgy of Polymyositis:

A

Endomysial inflammation infiltrate

18
Q

Histolgy of dermatomyositis:

A

Drop out’ of capillaries and myofibre damage

19
Q

Histolgy of SLE:

A

LE bodies
CNS - small vessel angiopathy
Spleen - onion skin lesions
Heart - Libman-Sack Endocarditis

20
Q

Histolgy of limited scleroderma:

A

Increased collagen in skin and organs

Onion skin thickening of arterioles

21
Q

Histolgy of diffuse scleroderma:

A

Inflammation within or around muscle fibres

22
Q

Signs & Symptoms of SLE:

A

Need 4/11 ACR criteria

SOAP BRAIN MD

Serositis
Oral ulcers
Arthritis
Photosensitivity

Blood disorders (AIHA, ITP, leucopenia)
Renal involvement
ANA +ve
Immune phenomena (dsDNA, anti-Sm, Antiphospholipid Ab)
Neuro symptoms

Malar rash
Discoid rash

23
Q

Signs & Symptoms of Limited scleroderma:

A

Skin changes on face and distal to elbows and knees

CREST

Calcinosis
Raynaud's
Esophageal dysmotility
Sclerodactyly
Telangiectasia

Associated with pulmonary HTN

24
Q

Signs & Symptoms of diffuse scleroderma:

A

Skin changes can occur anywhere
Widespread organ involvement
Associated with pulmonary fibrosis

25
Q

Signs & Symptoms of Polymyositis & dermatomyositis:

A
Proximal muscle weakness
Increased CK & abnormal EMG
DM has cutaneous features:
1) Heliotrope rash
2) Gottron papules

Associated with pulmonary fibrosis