Connective Tissue Disease Flashcards
what are the connective tissue diseases
SLE, sjogren’s syndrome, systemic sclerosis, dermatomyositis, polymyositis, mixed connective tissue disease, anti-phospholipid syndrome
what are connective tissue diseases
spontaneous over activity of the immune system
specific auto-antibodies involves
diseases evolve over years sometimes leading to organ failure and death
what is systemic lupus erthematosus
systemic autoimmune disease where the immune system attacks the bodys cells and tissue, resulting in inflammation and tissue (can affect any part of the body)
what is the role of antibody-immune complexes in systemic lupus erythematous
precipitate and cause a further immune response
who gets SLE
females 9:1 males
prevalence is higher in asians, afro- Americans and afro-Caribbeans
what is the aetiology of SLE
genetic factors, environmental factors, immunological factors, hormonal factors (higher oestrogen exposure)
what is the pathogenesis of SLE
loss of immune regulation
increased and defective apoptosis
necrotic cells release nuclear material which act as autoantigens
B and T cells stimulated
autoantibodies are produced
how can SLE cause renal disease
deposition of immune complexes (nuclear antigens and anti-nuclear antibodies) in mesangium which activate complement which attracts leucocytes which release cytokines- perpetuates inflammation which causes necrosis and scarring
what are immunologic diagnostic markers for SLE
positive ANA
anti DNA
what system when affected can be used to diagnose SLE
mucocutaneous musculoskeletal Serositis (inflammation of serous membranes) renal neurological haematological
what are the constititional symptoms of SLE
fever, malaise, poor appetite, weight loss, fatigue
what are the mucocutaneous features of SLE
photosensitivity malar rash discoid lupus erythematosus subacute cutaneous lupus mouth ulcers alopecia
what is discoid lupus erythematosus
form of cutaneous lupus- inflammation, sore and scarring of skin especially over face, ears, scalp
what is subacute lupus erythematosus
form of cutaneous lupus- lots of small papule or plaque with slight scaling that may merge
what are the MSK features of SLE
non deforming polyarthritis/ polyarthraglia
deforming arthropathy (jaccoud’s arthritis)
myopathy (weakness, myalgia and myositis)
what are forms of serositis
pericarditis, pleurisy, pleural effusion, pericardial effusion
what are the renal features of SLE
proteinuria of >500mg in 24 hours
red cell casts (microscopic bleeding from kidney)
what are the neurological features of SLE
depression/ psychosis migrainous headache seizures cranial or peripheral neuropathy mononeuritis multiplex
what are the haematological features
lymphadenopathy leucopenia lymphopenia haemolytic anaemia thrombocytopenia
what is anti-phospholipid syndrome
venous and arterial thrombosis
recurrent miscarriage
livido retiularis
association with other autoimmune conditions especially SLE
thrombocytopenia
prolonged APTT
describe SLE patients’ susceptibility to infection- intrinsic factors
low complements
impaired cell mediated immunity
defective phagocytosis
poor antibody response to certain antigens
describe SLE patients’ susceptibility to infection- extrinsic factors
steroids
other immunosuppressants
nephrotic syndrome
what are investigations into SLE used for
to confirm/establish diagnosis
to determine degree of organ involvement
what is ANA
anti-nuclear antibody
when should a positive ANA test be taken seriously
if other antinuclear antibodies are present
- anti-dsDNA
- anti-Sm
- anti-Ro
- anti-RNP
when the patient presents with CTD features
what is anti-dsDNA and what is it associated with
anti double stranded DNA antibody
lupus nephritis
what is anti-Ro associated with
cutaneous manifestations, secondary Sjogren’s features, congential heart block and neonatal LE
what are the anti- ENA antibodies
anti-Ro, anti-Sm, anti-RNP
what are the anti-phospholipid antibodies
anti-cardiolipin
lupus anticoagulant
anti-beta 2 glycoprotein
how do you monitor SLE activity
clinical assessment
Anti-dsDNA level positively correlates with activity
C3/C4 levels negatively correlate with activity
urine analysis for protein and casts
FBC
blood biochem
what is the general management of SLE
counselling
regular monitoring
avoid excessive sun exposure
pregnancy issues
what is the pharmacological treatment of SLE
NSAIDs and simple analgesia
anti malarials- hydroxychloroquine
(useful for arthritis, cutaneous manifestations and constitutional symptoms, may reduce systemic complications)
steroids: small doses- skin rashes, arthritis, serositis moderate doses- resistant serositis, haematologic abnormalities high doses- severe/ resisitant heamatological changes, renal disease, major organ involvement
immunosuppressants
biologics (rituximab, belimumab)
what is the drug treatment for mild disease
HCQ, topical steroids, NSAIDs
what is the drug treatment for moderate disease
oral steroids, azathioprine, methotrexate
what is the drug treatment for severe disease
IV steroids, cyclophosphamide, rituximab, belimumab
