connective tissue diseases Flashcards
what are connective tissue diseases
autoimmune conditions associated with spontaneous overactivity of the immune system
-auto antibodies
what is systemic lupus erythematosus
systemic autoimmune condition that can affect almost anywhere in the body
immune system attacks cells and tissue resulting in inflammation and tissue damage
is SLE more common in males or females
females
pathogenesis of SLE 1
- loss of immune regulation
- increased and defective apoptosis
- necrotic cells release nuclear materials which act as auto-antigens
- auto-immunity results from exposure to nuclear and cellular auto-antigens
- B and T cells are stimulated
- autoantibodies are produced
pathogenesis of SLE 2
- complexes of antigens and auto-antibodies form and circulate
- deposition of immune complexes in basement membrane
- activation of complement which attracts leucocytes which release cytokines
- cytokine release perpetuates inflammation which causes necrosis and scarring
is ANA positive or negative in SLE
positive
can be negative but very very rare
what must EULAR/ACR score be to confirm SLE
> 10
cutaneous features of SLE
subacute cutaneous or discoid lupus
acute cutaneous lupus
non-scarring alopecia
oral ulceration
types of arthritis associated with SLE
synovitis of at least 2 joints with >30 mins early morning stiffness
Jaccouds arthropathy
neurological manifestations of SLE
- delirium
- psychosis
- seizure
- headache
- cranial nerve disorder
serositis manifestations of SLE
- pleural or pericardial effusion
- acute pericarditis
haemotological manifestations of SLE
- leukopenia
- thrombocytopenia
- haemolytic anaemia
- lymphadenopathy
renal involvement of lupus
- proteinuria >0.5g in 24 hrs
- biopsy proven nephritis
- red cell casts
what is a specific antibody for lupus
dsDNA
-present in 60% of lupus patients
what are some anti-phospholipid antibodies
- lupus anticoagulant
- anti-cardiolipin antibodies
- anti-beta2glycoprotein antibodies
what is APLS associated with
venous and arterial thrombosis and recurrent miscarriage
what happens to complement levels when lupus is treated
they will normalise again from being low
whats anti ro antibody associated with
neonatal lupus
congenital heart block
what is management for all patients with lupus
- sun protection measures
- hydroxychlororoquine
- steroids but in small doses and for short periods of time
- monitor disease activity using SLEDAI score
what are the symptoms of Sjrogen’s
- dry eyes - gritty feeling
- dry mouth
- dry throat
- vaginal dryness
- bilateral parotid gland enlargement
- joint pain
- fatigue
- unexplained increase in dental caries
what antibodies for Sjogrens
anti ro
anti la
also may have raised IgG
does Sjogrens patients have increased risk of lymphoma
YES
what is systemic sclerosis
multisystem autoimmune disease characterised by vasculopathy, autoimmunity and fibrosis
what are symptoms of systemic sclerosis
- raynaud’s
- skin thickening
- difficulty swallowing
- GORD
- telangiectasia
- calcinosis
- +/- SOB
red flags for systemic sclerosis
- onset of Raynaud’s in mid adulthood
- Raynaud’s and SOB
- Raynaud’s and Telangiectasia
- Digital ulcers/ischaemia
- skin tightness/loss of dexterity of hands
what defines diffuse cutaneous SSc
skin involvement on extremities above and below elbows and knees (plus face and trunk)
what defines limited cutaneous SSc
skin involvement on extemities and only below elbows and knees (plus face)
facial changes for systemic sclerosis
- small mouth with puckering of lips
- beaked nose
- tightened/tight skin
- telangiectasia
GI complications of systemic sclerosis
- dysphagia
- GORD
- gastric antral vascular ectasia
- small intestine bacterial overgrowth
- malabsorption
- fluctuating bowel habit (constipation/diarrhoea)
- faecal incontinence
cardio/respiratory manifestations of systemic sclerosis
- ILD
- pulmonary arterial hypertension
- myocardial disease
what are renal complications of systemic sclerosis
- scleroderma renal crisis
- non specific progressive renal dysfunction
msk changes in SSc
- sclerodactyly
- digital ischaemia
- myositis
what are the three phases of Raynaud’s
1) blanching
2) acrocyanosis
3) reactive hyperaemia
treatments of Raynaud’s/Vasculopathy
- calcium channel blockers (Nifedipine)
- others (fluoxetine, ARBs, Nitrates)
- PDE-5 inhibitor (sildenafil)
- Prostacyclin infusion (Iloprost)
- endothelin receptor antagonist (Bosentan)
what can increase risk of pulmonary hypertension
- telangiectasia
- anticentromere antibody
- increased duration of disease
treatment for systemic sclerosis pulmonary hypertension
- PDE 5 inhibitor (Sildenafil/Tadalafil/Vardenafil)
- ERA (Bosentan/Ambrisentan/Macitentan)
- Eproprostenol infusions
- oxygen
treatment for ILD with systemic sclerosis
- mycophenolate mofetil
- rarely cyclophosphamide
- Rituximab as second line
- Nintedanib (antifibrotic)
- lung transplant
which antibody suggests renal crisis
Anti RNA polymerase III antibody
what can put people at risk of renal crisis
high dose steroids
features of renal crisis
uncontrolled hypertension with proteinuria and rapidly worsening renal function
treatment for renal crisis
ACE inhibitors
treatment for skin fibrosis with systemic sclerosis
Methotrexate
Mycophenolate
