Connective Tissue Disease Flashcards
What are connective tissue diseases characterised by?
presence of spontaneous over-activity of the immune systerm
Who does SLE affect?
M:F 1:9; higher in asians, afro-americans, afro-caribbeans, hispanic americans
How do hormones affect SLE incidence?
increased in those with higher oestrogen exposure- contraceptives and HRT
What environmental factors can increase SLE?
virsues; UV light; silica dust
What is the pathogenesis of SLE?
loss of immune regulation; defective apoptosis; necrotic cells release nuclear material which act as potential auto-antigens and autoimmunity results from extended exposure to nuclear and intra-cellular antigens
What causes renal disease in SLE?
deposition of immune complexes in mesngium; activate complement which attracts leucocytes which release cytokines; perpetuates inflam which causes necrosis and scarring over time
What are the general symptoms seen with SLE?
fever; malaise; poor appetite; wt loss; fatiuge
What are the mucocuntanoues features?
photosensitivty; malar rash (spares the naso-labial folds); discoid lupus erythematosus (may scar); subacute cutaneous lupus; painless mouth ulcers; alopecia
What are the MSK features of SLE?
non-deforming polyarthritis/polyathralgia; myopathy- weakness, myalgia and myosistis
What are the serositis features of SLE?
pericarditis; pleurisy; pleural effusion; pericardial effusion
What are the features of renal disease clinically?
proteinuria of >500mg in 24 hours; red cell casts
What test should always be done in pts with SLE?
urinalysis
What are the neurological features seen in SLE?
depression/psychosis; migraneous headache; seizures; cranial or peripheral neuropathy; mononeuritis mulitplex
What are the haemotological features of SLE?
lymphadenopathy; leucopenia; lymphopenia; haemolytic anaemia; thrombocytopenia
What is anti-phospholipid syndrome?
venous and arterial thrombosis
What are the features of anti-phospholiid syndrome?
recurrent miscarriage; DVT; young stroke; livido reticularis; assoc. with other autoimmune condiitons esp. SLE; thrombocytopenia; prolnged platelet time
What are the intrinsic factors that increase an SLE pts susceptibilty to inection?
low complement; defective phagocytosis; impaired cell mediated immunity
What are the extrinsic factors that contribute to an SLE pts susceptibilty to infection?
steroids and other immunosuppressive drugs
What are the benefits and bad of ANA?
positive in 95% of pts, found in lots of other conditions
What other antinuclear antibodies can be positive in SLE?
anti-dsDNA
anti-Sm
anti-Ro
anti-RNP
What are the benefits of anti-dsDNA?
occurs in about 60% of SLE and is highly specific for SLE
What is Anti-Ro usually associated with?
anti-La
What is anti-Ro often associated with?
congential heart block, neonatal LE, cutaenous manifestation
What is the most specific autoantibody for SLE?
anti-Sm
What are the antibodies present in antiphospholipid syndrom?
anti-cardiolipin
lupus anticoagulant
anti-beta 2 glycoprotein
What antibody level postivitely correlates with activity?
anti-dsDNA
What negatively correlate with activity?
C3/C4
What are the drug treatmnents for SLE?
NSAIDs and simple analgesia anti-malarials- hydroxychlorquine steroids immunosuppressives eg azathioprine biologics eg rituximab
What drugs are used in mild SLE?
HCQ; topical steroids; NSAIDS
What drugs are used in moderate disease?
oral steroids; azathioprine; methotrexate
What drugs are used in severe SLE?
IV steroids; cyclophosphamide; tituximab
What is gender distribution in anti-phospholipid syndrome?
M:F 1:3.5
What neurological and cardiac symptoms are seen with anti-phospholpi syndrome?
Libman-Sacks endocarditis; migrane, transverse
What happens in Sjogren’s syndrome?
lymphocyte infiltration of exocrine glands causing xerostomia and keratoconjunctivitis
What immunology might be seen with Sjogrens?
anti-Ro; anti-La
What test can be done to determine mouth and eye dryness?
schirmer test- paper on eye and in mouth
What are the more serious manifestations of Sjogrens?
ILD; neuropathy; lymphoma x40 risk; renal tubular acidosis; neonatal complete heart block (anti-Ro)
Who gets Sjogrens?
40-60yo; M:F 1:9
What are the treatment options for Sjogrens?
eye drops; saliva replacement; HCQ; steroid and immunosuppression; CVS risk factors
What is systemic sclerosis caused by?
vasculopathy of small vessels; inflammation and then fibrosis- excess deposition of collagen in skin and internal organs
What are the symptoms of systemic sclerosis?
Calcinosis Raynauds Esophageal dysmotility Sclerodactlyl Telangiectasia AND pulmonary HT
What antibodies are systemic sclerosis associated with?
anti-centromere
anti-Scl-70
What is diffuse systemic sclerosis?
skin changes within 1 year of Raynauds; truncal and acral skin involvemtn; early signif organ involvemtne
What are the GI organ manifestations of systemic sclerosis?
small bowel dysmotility bacterial overgrowth rectal dysmotility pancreatic insufficiency dysphagia
What are the respiratory manifestations of systemic sclerosis?
ILD; pulmonary HT; chest wall restriction
What are the CVS manifestations of systemic sclerosis?
Raynauds; atherosclerosis; hypertensive cardiomyopathy
Who is systemic sclerosis seen in?
25-55yo
M:F 1:4
What are the treatments for systemic sclerosis?
CCBs; prostacyclin analogue- Raynauds; ACEI; steroids; immunosupprestion
What is undifferentiated connective tissue disorder?
connecitve tissueish but dont fit a partiuclar syndrome (yet)
What antibody is seen with mixed connective tissue disease?
anti-RNP
