Histopathology 5s - Immune disorders/ connective tissue disease

Question 1

cANCA directed against?

Answer 1

proteinase 3

Question 2

pANCA directed against?

Answer 2

Myeloperoxidase

Question 3

SLE: SOAPBRAINMD

Answer 3

Serositis
Oral ulcers
Arthritis
Photosensitivity
Blood (all counts low)
Renal (proteinuria)
ANA
Immunological (anti ds-DNA)
Neurological (psych, seizures)
Malar rash 
Discoid rash

Question 4

Ro + La = ?

Answer 4

Sjrogens

Question 5

anti-SCL70 (anti-topoisomerase)

Answer 5

Diffuse cutaneous systemic sclerosis

Question 6

Anti-centromere antibodies

Answer 6

Limited cutaneous systemic sclerosis (CREST)

Question 7

anti-Jo1/anti-Mi2

Answer 7

Myositis

Question 8

Most specific SLE antibody

Answer 8

Anti-dsDNA

Question 9

Antibody in drug related SLE = ?

Answer 9

Anti-histone

Question 10

Which drug can cause SLE

Answer 10

Hydralazine taken for high BP

Question 11

Which other ANA markers are positive in patients with SLE?

Answer 11

All the ENAs are +Ve e.g. Ro, La, Sm, U1RNP

Question 12

hOW DO YOU MEASURE ANTI-DSdna?

Answer 12

ELISA

Question 13

What causes the rash in SLE?

Answer 13

Extravasation of RBCs

Question 14

What will immunofluorescence of the skin of an SLE patient show?

Answer 14

IgG immune complex deposition at the epidermis-dermis junction

Question 15

SLE associated endocarditis, what is it called?

Answer 15

Libman-Sacks endocarditis, non-infective cause of endocarditis

Question 16

What are the two types of systemic sclerosis?

Answer 16

Diffuse and limited

Question 17

Difference between distribution of diffuse and limited

Answer 17

Limited does NOT involve the trunk

Question 18

Limited systemic sclerosis/CREST antibody

Answer 18

Anti-centromere

Question 19

Diffuse systemic sclerosis antibody

Answer 19

Anti-topoisomerase/anti-scl70

Question 20

Pattern of immunofluorescence in diffuse systemic sclerosis = ?

Answer 20

NUCLEOLAR PATTERN

Question 21

Skin histology in systemic sclerosis

Answer 21

Increased depth and amount of collagen which reduces elasticity

Question 22

Another name for systemic sclerosis

Answer 22

Scleroderma

Question 23

What is mixed connective tissue disease?

Answer 23

Overlap of several connective tissue diseases: SLE, scleroderma, myositis

Question 24

Pattern of ANA in mixed connective tissue disease

Answer 24

SPECKLED

Question 25

Classification of granuloma seen in sarcoidosis

Answer 25

Non-caseating i.e. the core is not necrotic

Question 26

2 examples of medium vessel vasculitis

Answer 26

Polyarteritis nodosa | KAWASAKI DISEASE

Question 27

Large vessel vasculitis example

Answer 27

Takayasu arteritis | Giant cell arteritis

Question 28

2 examples of immune complex associated small vessel vasculitis

Answer 28

Anti-GBM and IgA vasculitis (HSP)

Question 29

Examples of ANCA-mediated small vessel vasculitis

Answer 29

GPA, eGPA., microscopic polyangiitis

Question 30

Polyarteritis nodosa associations

Answer 30

HBV | Rosary bead appearance on angiogram (aneurysms)

Question 31

Investigations for temporal arteritis

Answer 31

ESR and biopsy (needed for diagnosis)

Question 32

In temporal arteritis, which layer of the blood vessel is involved?

Answer 32

The tunica media NOT intima, lymphocytic infiltration

Question 33

The three hallmarks of Granulomatosis with polyangiitis

Answer 33

Upper respiratory tract (nosebleeds, saddle nose, sinusitis) Lower respiratory (lungs --> haemoptysis, SOB) Kidneys (haematuria)

Question 34

3 hallmarks of eGPA

Answer 34

Eosinophils Asthma Vasculitis

Question 35

name the 4 ribonucleoprotein (RNP) ANAs

Answer 35

Ro, La, Sm, U1RNP

Question 36

Anti-dsDNA staining pattern on ELISA

Answer 36

Homogenous

Question 37

Anti-ENA staining pattern on ELISA

Answer 37

Speckled