CTD Flashcards by S B

pathogenesis of SLE

loss of immune regulation
increased and defective apoptosis
nuclear material act as autoantigens
B and T cells stimulated 
autoantibodies produced

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what kidney problems can occur in SLE

Glomerulonephritis

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how might glomerulonephritis due to SLE present

Proteinuria
Urine sediments
Urine RBC and casts
Hypertension
Acute renal failure
Chronic renal failure

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investigations for SLE

FBC
U&amp;Es
CRP, ESR
Complement levels
Renal function tests

Anti-nuclear antibody (ANA)
Anti-double stranded DNA antibodies (dsDNA)
Extractable nuclear antigens (ENA) - Anti-Sm, Anti-RNP, Anti-RO

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why does a positive ANA not mean it is SLE

it is found in other conditions

e.g. RA, HIV, Hep C

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when should a positive ANA be taken seriously

when other anti-nuclear antibodies are positive 
Anti-dsDNA
Anti-Sm
Anti-Ro
Anti-RNP

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what antibody is highly specific for SLE

Anti-double stranded DNA antibody (Anti-dsDNA)

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what is Anti-Ro associated with

anti-la
Cutaneous manifestations
secondary sjogre’s features
congenital heart block

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which antibody is high specific for SLE and associated with neurological involvement

Anti-Sm

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which antibody in SLE correlates with activity

Anti-dsDNA

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SLE general management

lifestyle changes

dietary advice, smoking cessation, sun protection, and exercise

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SLE drug treatment

NSAIDs - normally Naproxen
Analgesia
When NSAID ineffective;
Anti-malarial - hydroxychloroquine

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what is used in SLE when NSAIDs and hydroxychloroquine ineffective

steroids

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what is the last line of treatment for SLE

immunosuppressives

e.g. Azathioprine, Methotrextae

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cons of immunosuppressies

All can cause bone marrow suppression
All can cause increased susceptibility to infection
Potentially teratogenic

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what do patients with SLE die of

Infection

CV disease

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what are key features of anti-phospholipid syndrome

Hx of 1 or more episodes of venous, arterial, or microvascular thrombosis
Hx of pregnancy loss
Hx of pregnancy-associated morbidity e.g. pre-eclampsia

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what is the antibody associated with anti-phospholipid syndrome

Positive anti-cardiolipin

what other CTD is anti-phospholipid syndrome associated with

SLE

what investigations can be done to confirm anti-phospholipid syndrome

lupus anticoagulant activity - positive on 2 occasions
anticardiolipin antibodies - positive on 2 occasions
anti-beta2-glycoprotein I antibodies - positive on 2 occasions

what are the features/symtoms of anti-phospholipid syndrome

CLOTS
Coagulation defect
Livedo reticularis 
Obstetric (recurrent miscarriages)
Thrombocytopenia (low platelets)

what is the treatment of anti-phospholipid syndrome

Thrombosis – LMWH
Pregnancy loss – aspirin + LMWH during pregnancy

Management of risk factors

what is Sjogren’s syndrome

Lymphocyte infiltration of exocrine glands causing xerostomia (dry mouth) and keratoconjunctivitis sicca (dry eyes)

what test can be done in Sjogren’s Syndrome to prove dry mouth

Schirmer test

what are other symptoms of Sjogren's syndrome

``` Fatigue Joint pain Raynauds Lymphadenopathy Skin/Vaginal dryness ```

antibodies associated with Sjogren's syndrome

Anti-Ro and La

treatment of sjogren's syndrome

eye drops/punctal plugs e.g. hypromellose salvia replacement pilocarpine - treats Dry mouth Hydroxychloroquine - for joint pain steroids immunosuppression - in severe cases

what is systemic sclerosis

autoimmune | excess deposition of collagen in skin and internal organs causing fibrosis

what was limited systemic sclerosis previously known as

``` CREST C - calconosis R - Raynauds E - esophageal dysmotility S - sclerodactyly (localized thickening and tightness of the skin of the fingers or toes) T - telangiectasia ```

what common symptoms is not mentioned in CREST in LIMITED systemic sclerosis

Pulmonary hypertension

what antibodies is LIMITED systemic sclerosis associated with

anti-centromere antibodies

what are the two types of scleroderma

localised | systemic

what are the 3 subtypes of systemic scleroderma

Limited Diffuse Systemic

what are the features of DIFFUSE systemic sclerosis

skin changes within 1 year of Raynaud's Truncal and acral skin involvement Early significant organ involvement (kidneys, lungs, gut, muscle, joints, heart)

what is the antibody associated with diffuse systemic sclerosis

Anti-Scl-70

what are cutaneous features of Systemic sclerosis

``` Raynaud's phenomenon Ulcers Swelling of hands and feet Skin thickening Sclerodactyly ```

what are organ manifestations of systemic sclerosis

GI - Oesophageal hypomobility - Small bowel hypo mobility - Pancreatic insufficiency - Rectal hypomobility Resp - interstitial lung disease - pulmonary hypertension chest wall restriction Renal - hypertensive renal crisis - ischaemic CV - Raynaud’s with digital ulceration - Atherosclerotic disease - Hypertensive cardiomyopathy

treatment for systemic sclerosis

``` ACE inhibitors CCB Prednisolone Immunosuppression Biological agents ```

what antibody is associated with mixed connective tissue disease

Anti-RNP

what antibody is associated with polymyositis

Anti-Jo-1

what are the major symptoms of mixed connective tissue disease

``` Severe myositis. Pulmonary involvement. Raynaud's phenomenon. Swollen hands observed. Sclerodactyly. ```

what are minor features of MCTD

``` Alopecia Leukopenia Anaemia Pleuritis Pericarditis Arthritis Trigeminal neuralgia Malar rash Thrombocytopenia Mild myositis History of swollen hands ```

all antibodies associated with SLE

ANA, Anti-DNA binding, Anti-Sm, Anti-Ro and La

Criteria for diagnosing SLE | need more than 4 out of a 11 to be diagnosed with SLE

1 - Malar/Butterfly Rash 2 - Discoid rash 3- Photosensitivity 4 - Oral Ulcers 5 - Non-erosive arthritis 6 - Serositis (either pleuritis or pericarditis) 7 - Renal disorder (Glomerulonephritis, persistent proteinuria) 8 - CNS disorder (seizures, psychosis) 9 - Haematological disorder (haemolytic anaemia, thrombocytopenia) 10 - Immunological disorder (presence of antibody) 11 - ANA positive