Histopath - connective tissue diseases Flashcards

1
Q

Pernicious anemia - pathophys?

A

High MCV

Anti-parietal cell antibodies, thus low IF –> poor B12 absorption

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2
Q

11 features of SLE

A
SOAP-BRAIN MD 
Serositis
Oral ulcers
Arthritis
Photosensitivity
Blood (pancytopenia, AIHA, ITP)
Renal involvement
ANA +ve
Immune(anti-dsDNA, anti-smith, anti-histone)
Neuro (psychosis)

Malar rash
Discoid rash

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3
Q

What is the titre in the ANA test?

A

The highest dilution of pt serum at which the test is still positive

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4
Q

3 autoantibodies seen in SLE?

Which one is most specific for SLE?

A

Anti-dsDNA
Anti-smith
Anti-histone

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5
Q

Which drug can cause SLE? how?

A

Hydralazine –>anti-histone antibody formation

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6
Q

2 ways to measure anti-dsDNA antibodies

A
  1. Crithidia luciliae (big mitochondrion with dsDNA)

2. ELISA

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7
Q

One cell type seen in SLE? what is it?

A

LE cells = neutrophils which have phagocytosed denatured nuclei

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8
Q

Skin histology in malar rash of SLE?

A

lymphocyte infiltration, RBCs extravasation, vacuolisation

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9
Q

Renal histology in SLE? What gives this appearance?

A

Thickened glomerular capillaires = wire loops

- Immune complex deposition in BM

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10
Q

Heart involvement in SLE?

A

Libman-sacks: non infective endocarditis

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11
Q

Scleroderma aka..?

What causes the tight skin? What is the localised form called?

A

Systemic sclerosis

Excess collagen + fibrosis.

Morphoea

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12
Q

2 forms of scleroderma? what is the difference?

A

Diffuse and limited

Diffuse= involves the skin of the trunk

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13
Q

Antibodies responsible for diffuse vs limited scleroderma

A
Limited = anticentromere antibody
Diffuse = antibodies ot DNA topoisomerase
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14
Q

Features of limited form of scleroderma

A
CREST
Calcinoshs (painful Ca deposits in fingertips)
Raynauds
Oesophageal dysmotility
Sclerodactyly
Telangiectasia
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15
Q

Why Oesophageal dysmotility in scleroderma?

A

Fibrosis in submucosa

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16
Q

What is seen on screening in suspected scleroderma

A

ANA test - nucleolar immunofluorescence

17
Q

Nail changes in scleroderma

A

Nail-fold capillary dilatation

18
Q

Histology in scleroderma?

A

Onion skin appearance of small arteries = intimal thickening

19
Q

What is mixed connective tissue disease?

A

SLE
Scleroderma
Polymyositis
Dermatomyositis

20
Q

What is seen in screening for mixed connective tissue disease?

A

Speckled pattern

21
Q

Abnormal Ix result in polymyositis + dermatomyositis

A

Raised CK

22
Q

Cutaneous feature of polymyositis

A

Gottron’s papules

23
Q

Most commonly involved organ in sarcoidosis? manifestations?

Skin manifestations?

A

Lung involvement (fibrosis + BHL + lymphocytosis)

Lupus pernio, erythema nodosum

24
Q

Sarcoidosis - histology?

A

Non-Caseating (non necrotic) granulomas

+ schaumann and asteroid bodies

25
Q

Eye and CNS and gland involvement in sarcoidosis?

A

Uveitis, keratoconjunctivitis

Meningitis, CN lesions

Bilateral parotid gland enlargement

26
Q

3 abnormal investigation results seen in sarcoidosis

A

hypergammaglobulinaemia

Raised ACE

Hypercalcemia (Vit D hydroxylation from macrophages)

27
Q

Which vasculitis is assoc with a certain virus? how does it sometimes present?

A

Polyarteritis nodosa
Hep B

Gut ischemia

28
Q

Weird Ix with temporal arteritis

A

Raised ESR

29
Q

Characteristic rash in vasculitis?

A

Purpuric + PALPABLE

30
Q

Kawasaki’s disease
Features?
Main concern

A
Persistent fever >5days
Conjunctivitis
Lymphadenopathy
Desquamation of palms and soles
Mucous membranes (strawberry tongue)

Coronary artery involvement –> aneurysms

31
Q

Features of polyarteritis nodosa?

Appearance on angiography

A

Necrotising
Inflammation is focal + well demarcated = mostly renal + mesenteric arteries

Nodular appearance (micro aneurysms)

32
Q

Wegener’s aka…?

A

Granulomatosis with polyangiitis

33
Q

Granulomatosis with polyangiitis = 3 main things?

Useful Ix?

A

ENT
Lungs
Kidney

cANCA directed against proteinase 3

34
Q

Chung Strauss - 3 main things? useful Ix?

A

Asthma
Eosinophilia
Vasculitis

pANCA against myeloperoxidase