connective tissue diseases Flashcards by zoe coyne

what are connective tissue diseases

autoimmune conditions associated with spontaneous overactivity of the immune system
-auto antibodies

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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

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is SLE more common in males or females

females

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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

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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

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is ANA positive or negative in SLE

positive

can be negative but very very rare

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what must EULAR/ACR score be to confirm SLE

> 10

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cutaneous features of SLE

subacute cutaneous or discoid lupus
acute cutaneous lupus
non-scarring alopecia
oral ulceration

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types of arthritis associated with SLE

synovitis of at least 2 joints with >30 mins early morning stiffness
Jaccouds arthropathy

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neurological manifestations of SLE

delirium
psychosis
seizure
headache
cranial nerve disorder

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serositis manifestations of SLE

pleural or pericardial effusion

- acute pericarditis

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haemotological manifestations of SLE

leukopenia
thrombocytopenia
haemolytic anaemia
lymphadenopathy

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renal involvement of lupus

proteinuria >0.5g in 24 hrs
biopsy proven nephritis
red cell casts

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what is a specific antibody for lupus

dsDNA

-present in 60% of lupus patients

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what are some anti-phospholipid antibodies

lupus anticoagulant
anti-cardiolipin antibodies
anti-beta2glycoprotein antibodies

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what is APLS associated with

venous and arterial thrombosis and recurrent miscarriage

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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