Connective Tissue Disease

Question 1

What is systemic lupus erythematosus?

Answer 1

chronic autoimmune connective tissue disease

highly variable presentation and disease course

Question 2

What is the pathophysiology of SLE?

Answer 2

circulating small immune complexes (form in small vessels)
activate complement - inflammation
antibody-antigen complexes deposited on basement membrane (skin, kidneys)

Question 3

How is SLE present?

Answer 3

constitutional symptoms (fever, fatigue, weight loss)

lupus nephritis

myalgia, arthralgia, inflammatory arthritis

skin: malar rash, discoid lupus, photosensitivity, oral ulceration

thrombocytopenia, anaemia, leukopenia

cardiac and resp manifestations

Question 4

How is SLE diagnosed?

Answer 4

FBC: anaemia, leukopenia, thrombocytopenia

urinalysis: evidence of glomerulonephritis

CT chest: other organ involvement

Immunology: ANA (not specific), anti-dsDNA (varies with disease activity), anti-Sm

Question 5

How is SLE managed?

Answer 5

skin disease and joint disease: NSAIDs, topical steroids, hydroxychloroquine

Other organ involvement: moderate dose steroids, immunosuppression e.g. azathioprine, mycophenolate

Lupus nephritis: IV steroids + hydroxychloroquine + cyclophosphamide/mycophenolate/tacrolimus/ciclosporin

Unresponsive: IV immunoglobulin, rituximab

Question 6

What causes Sjogren’s?

Answer 6

lymphocytic infiltrates in exocrine organs

Question 7

How does Sjogren’s present?

Answer 7

DRY stuff 
keratoconjunctivitis sicca
xerostomia 
vaginal dryness
arthralgia, fatigue

Question 8

How is Sjogren’s diagnosed?

Answer 8

Schirmer’s test (ocular dryness)
Anti Ro, Anti La
Lip gland biopsy

Question 9

How is Sjogren’s managed?

Answer 9

lubricating eye drops, saliva replacement products

pilocarpine (may stimuate saliva production)

hydroxychloroquine (arthralgia, fatigue)

Question 10

What is a side effect of pilocarpine?

Answer 10

flushing

Question 11

What are the classifications of systemic sclerosis?

Answer 11

limited - skin involvement confined to face, hands and feet, internal organ involvement later on

diffuse - early organ involvement, skin changes rapid and involve the trunk

Question 12

What causes systemic sclerosis?

Answer 12

excessive collagen deposition

Question 13

What are cutaneous manifestations of systemic sclerosis?

Answer 13

‘beaking’ of nose
Raynauds
Sclerodactyly
Pinched mouth

Question 14

What are internal organ manifestations of systemic sclerosis?

Answer 14

pulmonary fibrosis
pulmonary hypertension

renal crisis (due to htn)

Question 15

What are the antibodies for diffuse systemic sclerosis?

Answer 15

anti-Scl 70

Question 16

What are the antibodies for limited systemic sclerosis?

Answer 16

anti-centromere

Question 17

How is Raynauds managed?

Answer 17

calcium channel blocker e.g. nifedipine

Question 18

What is CREST syndrome?

Answer 18

subtype of limited systemic sclerosis

calcinosis 
raynauds 
oesophageal dysmotility 
sclerodactyly 
telangectasia

Question 19

What features do you see in mixed connective tissue disease?

Answer 19

Raynauds 
arthralgia 
myositis 
sclerodacyltyl 
ILD 
pulmonary htn

Question 20

What are the antibodies for MCTD?

Answer 20

anti RNP

Question 21

How does antiphosphlipid syndrome manifest?

Answer 21

recurrent venous or arterial thrombosis +/- fetal loss

Question 22

What are the antibodies for antiphospholipid syndrome?

Answer 22

anti-cardiolipin

Question 23

What is the APTT in antiphospholipid?

Answer 23

prolonged

Question 24

How is antiphospholipid managed?

Answer 24

lifelong anticoagulation

Question 25

What antibodies are present in drug induced lupus/

Answer 25

anti-histone

Question 26

What drugs can cause drug induced lupus?

Answer 26

hydralazine
isoniazid
procainamide
phenytoin