Connective Tissue Diseases & Vasculitides

Question 1

Describe systemic lupus erythematosus (SLE)

Answer 1

Multi-system autoimmune disorder causing a type III hypersensitivity reaction and an increase in classical complement deficiencies. Can be drug-induced. More common in females and those of Afro-Caribbean descent

Question 2

State the HLAs associated with SLE

Answer 2

HLA-DR3, HLA-DR2

Question 3

State the HLAs associated with scleroderma

Answer 3

HLA-DR5, HLA-DRw8

Question 4

State the autoantibodies involved in SLE

Answer 4

Anti-nuclear antibodies (ANA), anti-double-stranded DNA, anti-smooth muscle, anti-histone

Question 5

State the autoantibodies involved in limited scleroderma (CREST syndrome)

Answer 5

Anti-centromere

Question 6

State the autoantibodies involved in diffuse scleroderma

Answer 6

Anti-Scl70, fibrillarin, RNA polymerase I, II, and III, PM-Scl

Question 7

State the autoantibodies involved in polymyositis and dermatomyositis

Answer 7

Anti Jo-1 (tRNA synthetase)

Question 8

Describe the histological features of SLE

Answer 8

LE bodies. Small vessel angiopathy in CNS, onion skin lesions in spleen, Libman-Sacks endocarditis in heart, crescentic glomerulonephritis in kidney

Question 9

Describe the histological features of limited scleroderma

Answer 9

Increased collagen in skin and organs, onion skin thickening of arterioles

Question 10

Describe the histological features of diffuse scleroderma

Answer 10

Inflammation within or around muscle fibres

Question 11

Describe the histological features of polymyositis

Answer 11

Endomysial inflammatory infiltrate

Question 12

Describe the histological features of dermatomyositis

Answer 12

‘Drop out’ of capillaries, myofibre damage

Question 13

Describe the clinical features of SLE

Answer 13

At least 4 of 11 of SOAP BRAIN MD: Serositis, Oral ulcers, Arthritis, Photosensitivity, Blood disorders (AIHA, ITP), Renal involvement, ANA positive, Immune phenomena (anti-dsDNA, anti-Sm), Neurological symptoms, Malar rash, Discoid rash

Question 14

Describe the clinical features of limited scleroderma

Answer 14

CREST: Calcinosis, Raynaud’s, Esophageal Dysmotility, Sclerodactyly, Telangiectasia. Plus skin changes (on face and distal to elbows and knees) and pulmonary hypertension

Question 15

Describe the clinical features of diffuse scleroderma

Answer 15

Fibrotic skin changes anywhere on body, widespread organ involvement, pulmonary fibrosis

Question 16

Describe the clinical features of polymyositis

Answer 16

Proximal muscle weakness, raised creatine kinase, abnormal EMG. Associated with pulmonary fibrosis

Question 17

Describe the clinical features of dermatomyositis

Answer 17

Proximal muscle weakness, raised creatine kinase, abnormal EMG, heliotrope rash, Gottron papules. Associated with pulmonary fibrosis

Question 18

Name 2 large vessel vasculitides

Answer 18

Takayasu’s arteritis (pulseless disease), temporal arteritis

Question 19

Name 3 medium vessel vasculitides

Answer 19

Polyarteritis nodose (PAN), Kawasaki disease, Buerger’s disease (thrombangitis obliterans)

Question 20

Name at least 3 small vessel vasculitides

Answer 20

Granulomatosis with polyangiitis, Churg-Strauss syndrome (eosinophilic granulomatosis with polyangiitis), microscopic polyangiitis, Henoch-Schoenlein purpura

Question 21

Describe the key features of temporal arteritis

Answer 21

Scalp tenderness, temporal headache, jaw claudication, blurred vision. Usually affects the elderly and may occur alongside polymyalgia rheumatica

Question 22

Describe the histological features of temporal arteritis

Answer 22

Granulomatous transmural inflammation, giant cells, skip lesions

Question 23

Describe the key features of polyarteritis nodosa

Answer 23

Predominantly renal involvement, may involve other organs but spares lungs. Microaneurysms on angiography. 30% have hepatitis B

Question 24

Describe the key features of Kawasaki disease

Answer 24

Young children, fever 5+ days, polymorphous rash, red and oedematous palms and soles with later desquamation, conjunctivitis, inflammation of lips, mouth, and tongue (strawberry tongue), cervical lymphadenopathy, coronary artery aneurysm

Question 25

Describe the key features of Buerger's disease/ thrombangitis obliterans

Answer 25

Inflammation of arteries of the extremities (tibial, radial) causing pain and ulceration. Usually affects male heavy smokers <35. Corkscrew appearance of segmental occlusive lesions on angiography

Question 26

Describe the key features of granulomatosis with polyangiitis

Answer 26

Triad of upper respiratory tract symptoms (sinusitis, epistaxis, saddle nose), lower respiratory tract symptoms (cavitation, pulmonary haemorrhage), and kidney symptoms (crescentic glomerulonephritis). cANCA (anti-PR3) positive

Question 27

Describe the key features of Churg-Strauss syndrome (eosinophilic granulomatosis with polyangiitis)

Answer 27

Asthma, allergic rhinitis, eosinophilia, progressive systemic involvement. pANCA (anti-MPO) positive

Question 28

Describe the key features of microscopic polyangiitis

Answer 28

Pulmonary renal syndrome (pulmonary haemorrhage, glomerulonephritis). pANCA (anti-MPO) positive

Question 29

Describe the key features of Henoch-Schoenlein purpura

Answer 29

IgA mediated vasculitis in children <10 years. URTI triggers palpable purpuric rash on lower limbs, extensors, and buttocks, with colicky abdominal pain, glomerulonephritis, arthritis, orchitis