Histopath - connective tissue diseases

Question 1

Pernicious anemia - pathophys?

Answer 1

High MCV

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

Question 2

11 features of SLE

Answer 2

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

Question 3

What is the titre in the ANA test?

Answer 3

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

Question 4

3 autoantibodies seen in SLE?

Which one is most specific for SLE?

Answer 4

Anti-dsDNA
Anti-smith
Anti-histone

Question 5

Which drug can cause SLE? how?

Answer 5

Hydralazine –>anti-histone antibody formation

Question 6

2 ways to measure anti-dsDNA antibodies

Answer 6

1. Crithidia luciliae (big mitochondrion with dsDNA)

2. ELISA

Question 7

One cell type seen in SLE? what is it?

Answer 7

LE cells = neutrophils which have phagocytosed denatured nuclei

Question 8

Skin histology in malar rash of SLE?

Answer 8

lymphocyte infiltration, RBCs extravasation, vacuolisation

Question 9

Renal histology in SLE? What gives this appearance?

Answer 9

Thickened glomerular capillaires = wire loops

- Immune complex deposition in BM

Question 10

Heart involvement in SLE?

Answer 10

Libman-sacks: non infective endocarditis

Question 11

Scleroderma aka..?

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

Answer 11

Systemic sclerosis

Excess collagen + fibrosis.

Morphoea

Question 12

2 forms of scleroderma? what is the difference?

Answer 12

Diffuse and limited

Diffuse= involves the skin of the trunk

Question 13

Antibodies responsible for diffuse vs limited scleroderma

Answer 13

Limited = anticentromere antibody
Diffuse = antibodies ot DNA topoisomerase

Question 14

Features of limited form of scleroderma

Answer 14

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

Question 15

Why Oesophageal dysmotility in scleroderma?

Answer 15

Fibrosis in submucosa

Question 16

What is seen on screening in suspected scleroderma

Answer 16

ANA test - nucleolar immunofluorescence

Question 17

Nail changes in scleroderma

Answer 17

Nail-fold capillary dilatation

Question 18

Histology in scleroderma?

Answer 18

Onion skin appearance of small arteries = intimal thickening

Question 19

What is mixed connective tissue disease?

Answer 19

SLE
Scleroderma
Polymyositis
Dermatomyositis

Question 20

What is seen in screening for mixed connective tissue disease?

Answer 20

Speckled pattern

Question 21

Abnormal Ix result in polymyositis + dermatomyositis

Answer 21

Raised CK

Question 22

Cutaneous feature of polymyositis

Answer 22

Gottron’s papules

Question 23

Most commonly involved organ in sarcoidosis? manifestations?

Skin manifestations?

Answer 23

Lung involvement (fibrosis + BHL + lymphocytosis)

Lupus pernio, erythema nodosum

Question 24

Sarcoidosis - histology?

Answer 24

Non-Caseating (non necrotic) granulomas

+ schaumann and asteroid bodies

Question 25

Eye and CNS and gland involvement in sarcoidosis?

Answer 25

Uveitis, keratoconjunctivitis Meningitis, CN lesions Bilateral parotid gland enlargement

Question 26

3 abnormal investigation results seen in sarcoidosis

Answer 26

hypergammaglobulinaemia Raised ACE Hypercalcemia (Vit D hydroxylation from macrophages)

Question 27

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

Answer 27

Polyarteritis nodosa Hep B Gut ischemia

Question 28

Weird Ix with temporal arteritis

Answer 28

Raised ESR

Question 29

Characteristic rash in vasculitis?

Answer 29

Purpuric + PALPABLE

Question 30

Kawasaki's disease Features? Main concern

Answer 30

``` Persistent fever >5days Conjunctivitis Lymphadenopathy Desquamation of palms and soles Mucous membranes (strawberry tongue) ``` Coronary artery involvement --> aneurysms

Question 31

Features of polyarteritis nodosa? Appearance on angiography

Answer 31

Necrotising Inflammation is focal + well demarcated = mostly renal + mesenteric arteries Nodular appearance (micro aneurysms)

Question 32

Wegener's aka...?

Answer 32

Granulomatosis with polyangiitis

Question 33

Granulomatosis with polyangiitis = 3 main things? Useful Ix?

Answer 33

ENT Lungs Kidney cANCA directed against proteinase 3

Question 34

Chung Strauss - 3 main things? useful Ix?

Answer 34

Asthma Eosinophilia Vasculitis pANCA against myeloperoxidase