Connective, Vasculitis, Amyloid, Sarcoid Flashcards Preview

Path (AJC) > Connective, Vasculitis, Amyloid, Sarcoid > Flashcards

Flashcards in Connective, Vasculitis, Amyloid, Sarcoid Deck (21):
1

SLE

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.

2

SLE - S/Sx

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.

3

Limited scleroderma

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

4

Limited scleroderma - S/Sx

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

Assoc pulmonary hypertension.

5

Diffuse scleroderma

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).

6

Polymyositis and Dermatomyositis

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

7

Polymyositis and Dermatomyositis - S/Sx

Proximal muscle weakness, ^CK, abnormal EMG.
DM has heliotrope rash and Gottron's papules.

8

Takayasu's arteritis

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

9

Temporal arteritis

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

10

Polyarteritis nodosa (PAN)

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

11

Kawasaki's disease

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

12

Thrombangitis obliterans
(Buerger's disease)

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

13

Wegener's granulomatosis
Granulomatosis with polyangitis.

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

14

Churg-Strauss

Asthma, hay fever, eosinophilia.
pANCA

15

Microscopic polyangitis

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

16

Henoch-Schonlein Purpura

IgA mediated vasculitis.
Children

17

Amyloidosis - causes

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.

18

Amyloidosis - Clinical features

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

19

Amyloidosis - Dx

Congo red stain shows apple green birefringence under polarised light

20

Sarcoidosis

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.

21

Sarcoidosis -
extra-pulmonary manifestations

- 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.