Rheum

Question 1

What is ANCA?

Answer 1

ANCA = anti-neutrophil cytoplasm abs
Auto abs that bind to 2 proteins in the neutrophil cytoplasm PR3 + MPO

The binding of ANCA to N causes a toxic release of substances which causes inflammation to vessels - the N this migrate through the vessel wall causing release of pro-inflammatory cytokines

Question 2

What is granulomatosis polyangitis?

Answer 2

GPA = wegeners
Characterised by necrotising granuloma of upper and lower respiratory tract with focal glomerulonephritis
ELK
ENT/Lungs/Kidneys

Question 3

How might someone present with granulomatosis polyangitis?

Answer 3

E - hearing loss, saddle nose, strawberry gingivitis + mouth ulcers
L - SOB, CP, haemoptysis (pulmonary nodules)
K - haematuria, oedema, HTN

Heart - pericarditis
Arthritis
Skin - sc nodules at the elbow
NS - numbness and weakness

Question 4

What can help you confirm the diagnosis?

Answer 4

ANCA testing (90% are ANCA +ve) 
Indirect immunofluorescence IIF // ELISA

Question 5

How can you treat granulomatosis polyangitis?

Answer 5

Immunosuppression
Non-life threatening = IV Methyl pred -> oral pred + methotrexate
Life-threatening = IV methyl pred -> PO pred + cyclophosphamide

Maintaining remission = PO pred + methotrexate + folic acid
?Rituximab

Question 6

What is Churg-strauss disease?

Answer 6

Eosinophilic granulomatosis with polyangitis
= vascalitis in a person with atopy

1) Asthma
2) Eosinophilia
3) vasculitis

Question 7

What causes Henoch-Schonlein purpura?

Answer 7

IgA immune complexes deposited in small vessels - inflammation and bleeding in the small vessels of the skin, joints, intestines and the kidneys

Question 8

How does Henoch-Schonlein purpura present?

Answer 8

Arthralgia 
Abdo pain (V)
Purpuric rash (buttocks/LL) 
\+ low grade fever 
40% have nephrotic syndrome (proteinuria RBC, casts)

Question 9

A 7 year old boy presents with a purpuric rash on his buttock and back of legs. He has a 2/7 hx of vomiting, abdominal pain and fever. His mother says he has also been complaining of knee pain.
What are your main differentials?

Answer 9

Henoch-Schonlein purpura 
Meningitis 
Thrombocytopenia 
SLE 
Intussusception (occurs in 2-3% of HSP patients)

Question 10

What investigation work up would you do in someone with suspected HSP?

Answer 10

Bloods: FBC (high WCC + eosinophilia), ESR, CR is renal involvement, IgA, auto AB screen,
Urine: urinalysis (haematuria/proteinuria)
Imaging: Abdo USS

Question 11

How would you manage a patient with HSP?

Answer 11

Supportive (self-limiting disease with no therapy showing to shorten the length of disease)

• NSAIDS (arthralgia but mind renal impairment)
• Corticosteroids

Question 12

What is Good-Pastures?

Answer 12

AKA Anti-GBM disease
Its is a pulmonary-renal disease with auto-abs to alpha-3 chain of type 4 collagen found in the BM of alveoli and glomeruli
(2 organs but 4 collagen)

Question 13

A 55yr old man presents who gets regular dialysis presents with haemoptysis and chest pain.
What are you differentials?

Answer 13

(pulmonary haemorrhage with renal failure)
SLE, RA
Wegener’s granulomatosis
Idiopathic rapidly progressive glomerulonephritis

Question 14

What type of anaemia would a person with Goodpastures have and why?

Answer 14

Iron-deficiency anaemia from intrapulmonary bleeding

Question 15

A 45yr old Asian man presents complaining of a purpuric rash which has now started to ulcerate and abdo and testicular pain.
He also has symptoms of fever, weight loss, headache and myalgia. He has recently been diagnosed with HTN.
What is your main differential?

Answer 15

Polyarteritis Nodosa

Skin - purpura, s/c nodules and livedo reticularis
GI - pain from ischaemia
NS - mononeuritis multiplex

(tends to spare lung and renal arteries)

Question 16

What is polyarteritis nodosa?

Answer 16

A necrotising arteritis with microaneurysm formation, thrombus and infarction
It usually occurs in places where Hep B is common
ANCA -ve
Rosary sign on arteriography
Small artery biopsy will show necrotising inflam

Question 17

How would Kawasaki disease present?

Answer 17

CRASH + BURN // Warm Cream 
Conjunctivitis 
Rash - erythema/maculopapular 
Adenopathy - cervical LN 
Strawberry tongue/cracked lips 
Hands and feet - erythema and desquamation

Question 18

What is a complication of Kawasaki’s disease?

Answer 18

Coronary artery aneurysms

Do ECHO after 6 weeks

Question 19

How do you treat Kawasaki’s disease?

Answer 19

IVIG + high dose aspirin

Question 20

What is Kawasaki’s disease?

Answer 20

It is a mucocutaneous LN disease - AI mediated, self-limiting, small/medium vessel vasculitis

Question 21

Why is GCA seen as a medical emergency?

Answer 21

It can lead to inflammation of the optic artery –> anterior ishaemic optic neuritis –> sudden loss of vision and blindness

Question 22

Apart from the temporal arteries, GCA can affect which other artey?

Answer 22

Aorta (15%) - do aortic arch angiography

Question 23

A 50year old lady presents with vision problems - she has blurred vision with diplopia in her left eye. She has also had a sudden headache over the past hour.
What is your differential?
What other symptoms would you ask about?

Answer 23

GCA
Headache in the temporal region that is worse at night
Scalp tenderness
Jaw claudication - when talking or eating
Systemic symptoms - anorexia, weight loss, fever, sweats

Question 24

What are the differentials to GCA?

Answer 24

Migraine
Tension headache
Trigeminal neuralgia
Polyarteritis nodosa

Question 25

What are the 6A’s of association with Ank Spond?

Answer 25

Anterior uveitis 
Aortic regurgitation
AVN block  
Apical fibrosis 
Achiles tendonitis 
Amyloidosis