Systemic Autoimmune Conditions Flashcards

1
Q

What are some connective tissue diseases?

A

SLE
Systemic sclerosis
Sjogren’s syndrome

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2
Q

What is SLE?

A

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

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3
Q

What is the pathophysiology of SLE?

A

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

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4
Q

What is the presentation of SLE?

A

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

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5
Q

What are the investigations for SLE?

A

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

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6
Q

What autoantibodies are associated with SLE?

A

ANA
anti- dsDNA
anti-Smith
anti-phospholipid

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7
Q

What are SLE complications?

A

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

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8
Q

What is SLE treatment?

A

NSAIDs
Steroids
Hydroxychloroquine
Methotrexate
Biologics- rituximab

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9
Q

What is systemic sclerosis?

A

Autoimmune inflammatory and fibrotic connective tissue disorder

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10
Q

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

A

Limited cutaneous SSc
Diffuse cutaneous SSc

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11
Q

What is limited cutaneous SSc?

A

More limited version
CREST syndrome

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12
Q

What does CREST stand for in SSc?

A

Calcinosis
Raynaud’s
Esophageal dysmotility
Sclerodactyly
Telangiectasia

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13
Q

What is diffuse cutaneous systemic sclerosis?

A

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

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14
Q

What is scleroderma?

A

Hardening of the skin

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15
Q

What is sclerodactyly?

A

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

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16
Q

What is telangiectasia?

A

Dilated small blood vessels in the skin

17
Q

What is calcinosis?

A

Calcium deposits under the skin

18
Q

What is Raynaud’s?

A

Fingertips go white then blue due to vasoconstriction of finger vessels

19
Q

What autoantibody is associated with limited cutaneous SSc?

A

Anti-centromere antibodies

20
Q

What autoantibody is associated with diffuse cutaneous systemic sclerosis?

A

Anti Scl-70 antibodies

21
Q

What is the management of SSc?

A

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

22
Q

What is Sjogren’s syndrome?

A

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

23
Q

What are the features of Sjogren’s?

A

Dry mucous membranes- eyes, mouth, vagina

24
Q

What antibodies are associated with Sjogren’s?

A

anti-RO
anti-LO

25
Q

What is the test for Sjogren’s?

A

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

26
Q

What is the management of Sjogren’s?

A

Artificial tears
Artificial salvia
Vaginal lubricants
Hydrochloroquine

27
Q

What types of vasculitis affect the large vessels?

A

Giant cell arteritis
Takayasu’s arteritis

28
Q

What types of vasculitis affect the small vessels?

A

Eosinophilic Granulomatosis with Polyangiitis
Microscopic polyangiitis
Granulomatosis with polyangiitis

29
Q

What is the name for Eosinophilic Granulomatosis with Polyangiitis?

A

Churg-Strauss syndrome

30
Q

What is the name for granulomatosis with polyangiitis?

A

Wegener’s

31
Q

What are generic features of vasculitis?

A

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

32
Q

What blood tests are done for vasculitis?

A

CRP and ESR
p-ANCA and c-ANCA

33
Q

What are the features of GCA?

A

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)

34
Q

What are the features of Eosinophilic granulomatosis with polyangiitis?

A

Elevated eosinophils
Late onset asthma
Nasal polyps
pANCA

35
Q

What are the features of microscopic polyangiitis?

A

Renal failure
Pulmonary involvement
No granulomatous inflammation
p-ANCA

36
Q

What are the features of granulomatosis with polyangiitis?

A

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