Connective, Vasculitis, Amyloid, Sarcoid

Question 1

SLE

Answer 1

Autoimm, Type III hyperseinsitivity. 
RF: Afrocab, women, drugs, classical complement def. 
HLA: DR3. 
Ab: a-dsDNA, ANA, a-Smith. (Drug-induced: a-Histone.)
Histology: 
  - LE bodies, 
  - kidney, 
  - CNS, 
  - Spleen: onion skin lesions 
  - Heart: Libman-Sack Endocarditis.

Question 2

SLE - S/Sx

Answer 2

4 of Soap brain MD:- 
Serositis 
Oral ulcers 
Arthritis 
Photosensitivity 
Blood disorders (AIHA, ITP, luekopaenia) 
Renal 
ANA +ve 
Immune phenomena: a-dsDNA, a-Smith, a-phospholipid; 
Neuro Sx 
Malar rash 
Discoid rash.

Question 3

Limited scleroderma

Answer 3

= CREST
HLA: DR5 and DRw8
Ab: a-Centromere
Histology: Increased collagen in skin and organs; onion skin thickening of arterioles.

Question 4

Limited scleroderma - S/Sx

Answer 4

Skin changes on face and distal to elbows/knees. 
Calcinosis, 
Raynaud's, 
Esophageal dysmotility, 
Sclerodactyly, 
Telangectasia. 

Assoc pulmonary hypertension.

Question 5

Diffuse scleroderma

Answer 5

HLA: DR5 and DRw8 (same)
Ab: a-Scl-70, a-Topo.
Histology: Inflam in / around muscle fibres.
Skin can change anywhere. Organ involvement (pulmonary fibrosis).

Question 6

Polymyositis and Dermatomyositis

Answer 6

assoc. underlying malignancy.
Ab: a-Jo-1 (=tRNA synthetase).
Histology: Endomysial (in muscle) inflam infiltrate.

Question 7

Polymyositis and Dermatomyositis - S/Sx

Answer 7

Proximal muscle weakness, ^CK, abnormal EMG.

DM has heliotrope rash and Gottron’s papules.

Question 8

Takayasu’s arteritis

Answer 8

Large vessel vasculitis.
Japanese women.
Pulseless, bruits, claudication.

Question 9

Temporal arteritis

Answer 9

Large vessel vasculitis.
Elderly, scalp tenderness, headache,jaw claudication, blurred vision.
^ESR.
Overlap with polymyalgia rheumatica.

Question 10

Polyarteritis nodosa (PAN)

Answer 10

Mainly renal.
Never lungs.
30% have Hep B.
Microaneurysms on angiography.

Question 11

Kawasaki’s disease

Answer 11

Children 5 days, rash, desquamation, strawberry tongue, cervical LN.
Coronary arteries may have aneurysms.

Question 12

Thrombangitis obliterans

Buerger’s disease

Answer 12

Heavy smokers.
Tibial and Radial artery inflam: pain, ulcers.
Corkscrew appearance on angiogram.

Question 13

Wegener’s granulomatosis

Granulomatosis with polyangitis.

Answer 13

Triad: 
- saddle nose, epistaxis, sinusitis;  
- pulmonary haemorrhage; 
- crescentic glomerulonephritis (subtype 3). 
cANCA

Question 14

Churg-Strauss

Answer 14

Asthma, hay fever, eosinophilia.

pANCA

Question 15

Microscopic polyangitis

Answer 15

Pulmonary renal syndrome:
- Pulmonary haemorrhage,
- Glomerulonephritis.
pANCA

Question 16

Henoch-Schonlein Purpura

Answer 16

IgA mediated vasculitis.

Children

Question 17

Amyloidosis - causes

Answer 17

Primary (AL) is most common; usually Bence Jones protein due to things like Multiple Myeloma.

Secondary:

• AA, acute phase protein, so any chronic inflam/infection;
• Haemodialysis, beta2-microglobulin deposition;
• Familial, several kinds, all rare, most seen is Familial Mediterranean Fever: IL-1 causes fever and inflam.

Question 18

Amyloidosis - Clinical features

Answer 18

1. Kidney: nephrotic syndrome;
2. Heart: conduction defects, heart failure;
3. Liver/spleen: both big;
4. Tongue: Macroglossia in 10%;
5. Neuropathies: carpal tunnel.

Question 19

Amyloidosis - Dx

Answer 19

Congo red stain shows apple green birefringence under polarised light

Question 20

Sarcoidosis

Answer 20

Non-caseating granulomas; Schaumann and asteroid bodies.
More common, and worse in Afrocabs; F>M.
Lungs.
Seen on CXR - bilateral hilar lymphadenopathy.
Pulmonary infiltrates: fine nodular shadowing in mid zones.
Presents with insidious SOB, cough, chest pain, night sweats.
Ix: ^Ca, ^ESR, ^ACE.

Question 21

Sarcoidosis -

extra-pulmonary manifestations

Answer 21

• Skin: erythema nodosum, lupus pernio;
• LNs: painless rubbery;
• Eyes: anterior uveitis (misting, pain), or posterior; (Uveoparotid fever = b/l uveitis, big parotids, facial N palsy)
• Hepatosplenomegaly;
• Low WCC, low Hb;
• ^Ca, calculi;
• Heart: arrhythmia, cardiomyopathy.