CTD Flashcards
pathogenesis of SLE
loss of immune regulation increased and defective apoptosis nuclear material act as autoantigens B and T cells stimulated autoantibodies produced
what kidney problems can occur in SLE
Glomerulonephritis
how might glomerulonephritis due to SLE present
Proteinuria Urine sediments Urine RBC and casts Hypertension Acute renal failure Chronic renal failure
investigations for SLE
FBC U&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
why does a positive ANA not mean it is SLE
it is found in other conditions
e.g. RA, HIV, Hep C
when should a positive ANA be taken seriously
when other anti-nuclear antibodies are positive Anti-dsDNA Anti-Sm Anti-Ro Anti-RNP
what antibody is highly specific for SLE
Anti-double stranded DNA antibody (Anti-dsDNA)
what is Anti-Ro associated with
anti-la
Cutaneous manifestations
secondary sjogre’s features
congenital heart block
which antibody is high specific for SLE and associated with neurological involvement
Anti-Sm
which antibody in SLE correlates with activity
Anti-dsDNA
SLE general management
lifestyle changes
dietary advice, smoking cessation, sun protection, and exercise
SLE drug treatment
NSAIDs - normally Naproxen
Analgesia
When NSAID ineffective;
Anti-malarial - hydroxychloroquine
what is used in SLE when NSAIDs and hydroxychloroquine ineffective
steroids
what is the last line of treatment for SLE
immunosuppressives
e.g. Azathioprine, Methotrextae
cons of immunosuppressies
All can cause bone marrow suppression
All can cause increased susceptibility to infection
Potentially teratogenic
what do patients with SLE die of
Infection
CV disease
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
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
