Systemic Autoimmune Conditions

Question 1

What are some connective tissue diseases?

Answer 1

SLE
Systemic sclerosis
Sjogren’s syndrome

Question 2

What is SLE?

Answer 2

Systemic Lupus Erythematous
Non specific symptoms
Relapsing-remitting course
Chronic inflammation

Question 3

What is the pathophysiology of SLE?

Answer 3

Anti nuclear antibodies (ANA)- antibodies against self
Immune complex formation
Complement activation
Tissue injury

Question 4

What is the presentation of SLE?

Answer 4

Malar rash
Discoid rash
Photosensitivity
Oral ulcers
Arthritis
Pleuritis
Renal- proteinuria, haematuria
Neurological- seizures, psychosis
Haematological- cytopenia
Immunological- autoantibodies
Myalgia, Arthralgia

Question 5

What are the investigations for SLE?

Answer 5

Autoantibodies
FBC
C3 and C4
CRP and ESR
Immunoglobulins
Urinalysis
Renal biopsy- lupus nephritis

Question 6

What autoantibodies are associated with SLE?

Answer 6

ANA
anti- dsDNA
anti-Smith
anti-phospholipid

Question 7

What are SLE complications?

Answer 7

CVD
Infection
Anaemia of chronic disease
Pleuritis/pericarditis
Interstitial lung disease
Lupus nephritis

Question 8

What is SLE treatment?

Answer 8

NSAIDs
Steroids
Hydroxychloroquine
Methotrexate
Biologics- rituximab

Question 9

What is systemic sclerosis?

Answer 9

Autoimmune inflammatory and fibrotic connective tissue disorder

Question 10

What are the 2 main patterns of disease in systemic sclerosis?

Answer 10

Limited cutaneous SSc
Diffuse cutaneous SSc

Question 11

What is limited cutaneous SSc?

Answer 11

More limited version
CREST syndrome

Question 12

What does CREST stand for in SSc?

Answer 12

Calcinosis
Raynaud’s
Esophageal dysmotility
Sclerodactyly
Telangiectasia

Question 13

What is diffuse cutaneous systemic sclerosis?

Answer 13

Features of CREST syndrome plus internal organ damage
Cardio- CAD, HTN
Lungs- pulmonary HTN, pulmonary fibrosis
Kidney problems- golmerulonephritis

Question 14

What is scleroderma?

Answer 14

Hardening of the skin

Question 15

What is sclerodactyly?

Answer 15

Skin changes in the hands- breaks and ulcerates as it tightens

Question 16

What is telangiectasia?

Answer 16

Dilated small blood vessels in the skin

Question 17

What is calcinosis?

Answer 17

Calcium deposits under the skin

Question 18

What is Raynaud’s?

Answer 18

Fingertips go white then blue due to vasoconstriction of finger vessels

Question 19

What autoantibody is associated with limited cutaneous SSc?

Answer 19

Anti-centromere antibodies

Question 20

What autoantibody is associated with diffuse cutaneous systemic sclerosis?

Answer 20

Anti Scl-70 antibodies

Question 21

What is the management of SSc?

Answer 21

Steroids
Immunosuppressants
Physio and OT
Avoid cold triggers for Raynaud’s
Medical management for organ damage

Question 22

What is Sjogren’s syndrome?

Answer 22

Autoimmune condition affecting the exocrine glands
Primary or Secondary to SLE or RA

Question 23

What are the features of Sjogren’s?

Answer 23

Dry mucous membranes- eyes, mouth, vagina

Question 24

What antibodies are associated with Sjogren’s?

Answer 24

anti-RO
anti-LO

Question 25

What is the test for Sjogren's?

Answer 25

Schirmer test- filter paper inserted under lower eyelid for 5 mins Tears should travel 15mm

Question 26

What is the management of Sjogren's?

Answer 26

Artificial tears Artificial salvia Vaginal lubricants Hydrochloroquine

Question 27

What types of vasculitis affect the large vessels?

Answer 27

Giant cell arteritis Takayasu’s arteritis

Question 28

What types of vasculitis affect the small vessels?

Answer 28

Eosinophilic Granulomatosis with Polyangiitis Microscopic polyangiitis Granulomatosis with polyangiitis

Question 29

What is the name for Eosinophilic Granulomatosis with Polyangiitis?

Answer 29

Churg-Strauss syndrome

Question 30

What is the name for granulomatosis with polyangiitis?

Answer 30

Wegener's

Question 31

What are generic features of vasculitis?

Answer 31

Purpura Joint and muscle pain Peripheral neuropathy Renal impairment GI disturbance Anterior uveitis HTN Systemic symptoms

Question 32

What blood tests are done for vasculitis?

Answer 32

CRP and ESR p-ANCA and c-ANCA

Question 33

What are the features of GCA?

Answer 33

Most common - Age>50 - New headache - Temporal artery tenderness/reduced pulsation - ESR>50 - Abnormal temporal biopsy - Jaw claudication Responds immediately to prednisolone (40-60mg per day)

Question 34

What are the features of Eosinophilic granulomatosis with polyangiitis?

Answer 34

Elevated eosinophils Late onset asthma Nasal polyps pANCA

Question 35

What are the features of microscopic polyangiitis?

Answer 35

Renal failure Pulmonary involvement No granulomatous inflammation p-ANCA

Question 36

What are the features of granulomatosis with polyangiitis?

Answer 36

Resp. tract- cough, wheeze, haemoptysis Kidneys- glomerulonephritis cANCA