Rheumatology

Question 1

Behcets disease sx

Answer 1

oral ulcers- MOST COMMON and recurrent
genital ulcers
polyarthralgia
uveitis
erythema nodosum or acneform lesions
migratory thrombophlebitis
vasculitis
GI sx: abdominal pain and diarrhea

Question 2

Enteropathic arthralgia

Answer 2

Commonly seen with IBD (UC/ and more commonly with CD)
acute oligoarthritis
mouth ulcers
erythema nodosum
iritis

Question 3

Reactive Arthritis

Answer 3

Preceding URTI
arthritis
conjunctivitis
uveitis
urethritis

Question 4

Behcets Disease: RF and patho

Answer 4

epidemiology: mediterranean + east asia: TURKEY, ISRAELI, JAPAN
HLA B51
patho: AI/anti-inflammatory dx due to neutrophil hyperactivity

Question 5

Behcets diagnosis

Answer 5

Pathery testing: SC prick forms induration in skin within 48 hrs
RF, ANA and ANCA NEGATIVE
HLAB51 POSTIVE
CSF: lymphocytosis or polymorphonuclear cels
MRI: white matter changes

Question 6

Tx of Behcets dx

Answer 6

oral:
1. topical corticosteroids like triamcinolone
2. colchicine, prednisolone, azt
3. anti-TNF alpha like infliximab

eye: pred + azt or pred + infliximab

gi: pred + infliximab

CNS: pred+ infliximab

vascular: pred + cyclosporine

Question 7

Retroperitoneal Fibrosis

Answer 7

multifocal fibrosclerotic syndrome including mediastinal fibrosis, sclerosing cholangitis and riedel thydoriditis
males > females
50-60 years old
promontary of sacrum upto L2
sx: back pain, weight loss, fever
dx: intravenous pyelography showing displacement towards ureters
complications: htn, vte
RF: methysergide, BB, methyldopa, hydralazine

Question 8

Neonatal lupus

Answer 8

Anti-RO antibodies and anti-LA antibodies
usually presents with RASH which lasts 6-8 months
associated with congenital heart block - measure pulse son 18 week scan + give dexamethasone

Question 9

SLE Pathophysiology

Answer 9

multi-systemic inflammatory connective tissue disorder
auto-immune: type 2 HS
antibodies react with antigen, form complex, attacked by neutrophils and complement system which causes inflammation leading to damage of endothelium causing small vessel vasculitis
women > men
west indian population

Question 10

Drug induced lupus

Answer 10

men> women
resolves when drugs stopped
drugs causing it: procrainamde, isoniazid, hydralazine - active amino grou p
CNS and renal disease are rare
ANA +
antibodies to dsDNA -

Question 11

Hughes syndrome

Answer 11

anti-phospholipid antibody syndrome
sx:
- recurrent VTE and Arterial throbosis
- recurrent fetal loss
- thrombocytopenia
- liban sacks endocarditis
- focal neurology
- TIA

Question 12

Symptoms of SLE

Answer 12

arthralgia or non erosive arthritis
rash: dicoid lupus erythemtousos- scaly red plaques with follicular plugging on sun-exposed sites which heal with scarring, malar rash,
fever

pericarditis, pleurisy,
renal: proteinuria, nephrotic syndrome, glomeruloneprhritis
neuro/psych: psychosis, seizures
heam: leukopenia, thrombocytopenia, haemloysis
alopecia
raynauds
resp: pneumonitiis
cardiac: libman sacks

Question 13

Dx of SLE

Answer 13

Hx and physical exam showing clinical features
FBC: anemia of chronic dx with normal mcv, low platelets, low neutrophils, low wcc
serology: ANA +, anti-dsDNA antiboodies, low complment levels, anti-Sm antibodies, anti-ro/la and anti RNP antibodies, anti-phosphoslipid antibodies
CRP
low C3 and Low C4- lupus nephritis

Question 14

Mx of SLE

Answer 14

symptom relief: sunblock, analgesia
supress immune system: DMARDS like hydroxychloroquine, analgesia, mtx, azt, plasma exchange, rituximab

Question 15

PAN

Answer 15

systemic necrotising vasculities affecting medium artering and cases aneyrysm formation

Question 16

PAN epidemiology

Answer 16

Middle aged men, associated with HEP B and

Question 17

PAN aetiology

Answer 17

Idiopathic
Triggered by viral infections like HEP B and HEP C

Question 18

PAN pathophysiology

Answer 18

Not fully understood
Immune complexes deposit on arteries, cause inflammation, leading to aneurysm formation and thrombosis which leads to ischemia to organs

Question 19

PAN symx

Answer 19

General: malaise, fever, weight loss
Skin: livedo reticularis, nodules, purpura, necrotic ulcers,
Cardio: MI, pericarditis, HTN
GI: post-prandial ischemia causing abdo pain, bowel perforation
renal: CKD, hematuria, proteinuria- MOST COMMON ORGAN
neuro: stroke, mononeuritis multiplex

Question 20

DDx of PAN

Answer 20

ESR, CRP, ANCA negative, anemia, leukocytosis, HbsAG +
Biopsy: necrotising inflmmation and fibrinoid necrosis
imaging: angio- anuenyrusms –> ROSARY sign

Question 21

Tx of PAN

Answer 21

steroids, azt, cyclophosphamide, anti-viral for Hep B, Ace-I or ARB

Question 22

Granulmatosis with polyangitis

Answer 22

Upper and Lower respiratory tract involcement- sinuses and lungs

Question 23

Thalassemia

Answer 23

genetic disease causing low hemogobin syntehsis due to decrease or no synthesis of alpha/beta globin chains leading to hemolytic anemia

Question 24

Epidemiology of thalassemia

Answer 24

mediterranea, middle east or fast east
ALPHA- indian, south east asia, africa
BETA- china, south east asia, middle east, mediterranea

Question 25

Aetiology of thalessemia

Answer 25

AR- gene deletion/mutation leading to ineffetive hemoglobin sntehsis- poorly made RBCS which hemolyse causing anemia
Alpha: gene deletionon chromosome 16, 4 subtupes
A+ haplotype- low risk, low mcv- africa and india
A0 haplotype- high rish, low mcv and mch- south east asian and middle eastern
HbH- intermedia
Hb Barts- still birth

Beta- point mutation on beta globin chains on chomosome 11
B-thalessmia trait- microcytic anemia, only one Beta globin mutated
B thallesemia intermedia- occosional tranfusion needed during stress or pregnancy
B-thalessemia major- severe microcytic hypochromic anemia–> splenomegaly

Question 26

Symx of thalassemia

Answer 26

Depends on the sub type
usually associated with syms of anemia: pallor, jaundice
bone: frontal bossing
Cardia: arrhythmia, cardiac failure,
Endocrine: DM, hypocalcemia

Question 27

Ddx of thalessemia

Answer 27

Blood films will show polychromasia and macrocytosis, reticulocytes, mexican hat ,
FBC: low Hb, low MCV, raised WCC, low plats
Iron: normal or elevated
HB electrophoresis

Imaging: X-ray, ECG,

Question 28

Tx of Thalassemia

Answer 28

Diet: low in iron
Iron chelation- deferiprone and ascorbic acid
hydroxyurea
ascorbic acid for increase excretion of iron in the urine
gene therapy
splenectomy

Question 29

What are the symptoms of giant cell arteriis

Answer 29

Headache- UNILATERAL temporal
Fever
Malaise
Vision
Scalp sensitivity
Jaw/tongue claudication

Question 30

What is the treatment for giant cell arteritis

Answer 30

Prednisone- 40-60mg PO
Aspirin prophylaxis