MCRP Notes Rheum

Question 1

New name for Wegener’s

Answer 1

Granulomatosis with polyangitis

Question 2

What is Granulomatosis with polyangitis

Answer 2

Autoimmune condition associated with a necrotizing granulomatous vasculitis

Question 3

What does Granulomatosis with polyangitis affect?

Answer 3

Affects both the upper and lower respiratory tract as well as the kidneys

Question 4

Main symptoms of Granulomatosis with polyangitis (GPA)?

Answer 4

Upper resp tract - epistaxis, sinusitis, nasal crusting.
Lower resp tract - dyspnoea and haemoptysis
Kidneys - rapidly progressive glomerulonephritis

Question 5

Type of glomerulonephritis in GPA

Answer 5

Pauci-immune in 80%. Minimal hypersensitivity seen when staining with immunofluorescence for IgG

Question 6

Extra symptoms of GPA

Answer 6

Saddle shaped nose deformity. Cranial nerve lesions. Eye involvement - proptosis. Vasculitic rash

Question 7

Antibodies present in GPA

Answer 7

c-ANCA in over 95%, p-ANCA in 25%

Question 8

What is seen in investigations in GPA

Answer 8

CXR and CT chest - cavitating lesions

Renal biopsy - epithelial crescents in bowmans capsule

Question 9

Management of GPA

Answer 9

Steroids, cyclophosphamide (response in 90%), plasma exchange

Question 10

Mean survival GPA

Answer 10

8-9 years

Question 11

Similarities between GPA and Churg-Strauss syndrome

Answer 11

vasculitis, sinusitis, dyspnoea

Question 12

Differences between GPA and Churg-Strauss syndrome

Answer 12

Churg-strauss = asthma, eosinophilia + pANCA
GPA = c-anca, renal, epistaxis/haemoptysis

Question 13

New name for Churg-Strauss syndrome

Answer 13

Eosinophilic granulomatosis with polyangitis

Question 14

Organs affected by Churg-strauss

Answer 14

Skin, lungs, bowels, heart, nerves and kidneys

Question 15

Features/stages of churg-strauss

Answer 15

Allergic phase - occurs 3-9years before the rest, asthma/allergic rhinitis, can get nasal polyps
Eosinophilic phase - high levels of eosinophils, esp in lungs and bowels. Get weight loss, night sweats, cough, abdo pain, GI bleeding
Vasculitic phase - worsening of all previous symptoms plus vessel disease/vasculitis. Can get blood clots and infarction of organs including severe heart disease

Question 16

Treatment of churg-strauss

Answer 16

prednisolone, azathioprine, cyclophosphamide

Question 17

Age of churg-strauss

Answer 17

Around 40’s. Rare for children or over 65 to be affected

Question 18

What is goodpastures syndrome

Answer 18

Rare condition associated with pulmonary haemorrhage and rapidly progressive glomerulonephritis

Question 19

What causes goodpastures syndrome

Answer 19

Anti-glomerular basement membrane antibodies against type IV collagen
Associated with HLA DR2

Question 20

Features of goodpastures

Answer 20

Male:female is 2:1. Bimodal age distribution - peaks age 20-30 and age 60-70

Question 21

What increases risk of pulmonary haemorrhage in goodpastures syndrome

Answer 21

Pulmonary oedema, smoking, LRTI, inhalation of hydrocarbons, young males

Question 22

Investigations in goodpastures syndrome

Answer 22

Renal biopsy linear IgG deposits along basement membrane

Raised transfer factor after pulmonary haemorrhages

Question 23

Management of goodpastures

Answer 23

Plasma exchange, steroids, cyclophosphamide

Question 24

What is Behcets

Answer 24

Complex multisystem disorder characteristed with presumed autoimmune inflammation of arteries and veins

Question 25

Main 3 symptoms of Behcets

Answer 25

Oral ulceration, genital ulceration, anterior uveitis

Question 26

epidemiology features of behcets

Answer 26

M>F - more common and worse. 
Associated with HLA B5
More common in eastern Mediterranean 
Young adults 
30% family history

Question 27

Other symptoms of behcets

Answer 27

Arthritis, thrombophlebitis, neuro (aseptic meningitis), Gi (Abdo pain, diarrhoea + colitis), erythema nodosum, DVT

Question 28

diagnosis of behcets

Answer 28

no definitive test, clinical diagnosis

+ve pathergy test is indicative (puncture site following needle prick becomes inflamed with small pustule forming)