1b Lupus - Other Connective Tissue Problems Flashcards

SAID ON PPT WILL NOT BE TESTED IN EXAMS

1
Q

What is Sjogren’s syndrome?

A

Autoimmune exocrinopathy - lymphocytic infiltration of especially exocrine glands and sometimes other organs

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2
Q

What does exocrine gland pathology result in in Sjogrens syndrome?

A

Dry eyes (xerophthalmia)
Dry mouth (xerostomia)
Parotid gland enlargement

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3
Q

What will a salivary gland biopsy show in Sjogrens syndrome?

A

Salivary gland biopsy will show lymphocytic infiltration predominantly CD4 helper T cells and to lesser extent B lymphocytes

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4
Q

What is inflammatory muscle disease?

A

Proximal muscle weakness due to autoimmune-mediated inflammation either with (dermatomyositis) or without (polymyositis) a rash

Skin changes with polymyositis

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5
Q

What are the skin changes seen in inflammatory muscle disease?

A

Lilac-coloured (heliotrope) rash on eyelids, malar region and naso-labial folds
Red or purple flat or raised lesions on knuckles (Gottron’s papules)
Subcutaneous calcinosis
Mechanic’s hands (fissuring and cracking of skin over finger pads)

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6
Q

What biochemical test results are seen in inflammatory muscle disease? (some features for both types, some are type-specific)

A

Elevated CPK, abnormal electromyography, abnormal muscle biopsy (polymyositis = CD8 T cells; dermatomyositis = CD4 T cells in addition to B cells)

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7
Q

What are the key manifestations of systemic sclerosis?

A

thickened skin with raynaud’s phenomenon

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8
Q

What are the changes seen in systemic sclerosis?

A

Thickened skin with Raynaud’s phenomenon
Dermal fibrosis, cutaneous calcinosis and telangiectasia

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9
Q

What is CREST?

A

CREST describes a sub-type of limited systemic sclerosis.
It stands for Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasia

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10
Q

What is overlap syndrome?

A

When features of more than 1 connective tissue disorder are present e.g. SLE and inflammatory muscle disease we can use the term overlap syndrome

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11
Q

what antibody confirms the presence of Mixed Connective tissue disease?

A

Anti-U1-RNP antibody*

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