context week 2 Flashcards
what does arthropathy mean
disease of a joint
what does arthritis mean
inflammation of joint
what is arthralgia
pain of the joint
there are two categories of conditions affecting joint. Name them and the common conditions .
non-inflammaroty - OA
inflammatroy: - seropositive = RA/CTD
- seronegative= many types
what are the four types of inflammatory arthritis
seropositive,(RA, SLE, vasculitis, scleroderma, Sjogren’s.)
seronegative (AS, psoriatic arthritis, reactive arthritits, enteropathic arthritis),
crystal (gout and pseudogout)
infectious
what does seropositive condition mean?
autimunity, many autoantibodies for many associated conditions.
antibody for SLE
anti-DNA
antibody for RA
anti-CPP
antibody for Strogen’s
anti-La
antibody for APS
anti-cardiolipin
antibody for small vessel vasculitis
ANCA
symptoms of inflammatory arthritis
morning stiffness (>30mins), helped by excursus, high CRP and PV, joint pain and associated swelling. synovitis
what is RA
immune response against synovium causing inflammatory pannus and attacking cartilage in tendon/joints.
where is first involved in RA
hands and feet
where must be closely monitored in RA and why?
C1/C2 as may compress spinal cord
what investigations done for RA?
check RF and ACPA (anti-CPP)
high ESR, CRP, PV noted.
US can detect synovial inflammation.
what are the extra-articular manifestations of RA
rheumatoid nodules, lung involvment, inc CVS risk, occular problems
treatment for RA
DMARD’s ASAP, short-term NSAID’s and steroids initially + analgesia. biological agent can be used once a few DMARD’s tried.
what is used to determine RA activity?
DAS28, >5.1 = severe and <2.6 = remission.
what are patients with seronegative pathologies often positive for in blood tests?
HLA-B27 positive
also inc ESR and CRP
what is Ankylosing spondylitis?
spine and Sacroiliac joint disease causing FUSION
investigations for AS
MRI detects early features
Schobers test determines flexibility of back
treatment for AS
physio, excersise, NSAID’s anti-TNF inhibitors (NO DMARDs’)
what are the common features of psoriatic arthritis?
psoriasis, nail pitting and onchyolitis, dactylitis
treatment for psoriatic arthritis
DMARD, then anti-TNF
what is enteropathic arthritis and how is it best to treat?
IBD related.
control IBD
what is reactive arthritis
arthritis in response to infection (GI or GU), infection triggers autoimmune arthropathies.
symptoms of reactive arthritis/Reiter’s syndrome
uveitis, urethritis, arthritis (all post-infection)
treatment of reactive arthritis/Reiter’s syndrome
self-limiting usually so NSAID’s or steroids.
treat underlying infection if necessary.
if chronic then DMARD
what is SLE?
chronic, highly variable AID. (CTD)
SLE commonly affects which systems?
skin, joint problems.
blood cells affected
nervous system commonly affected
(almost any system)
blood tests in SLE
anti-DAN, ANA, anti-Sm, antiphospolipid antibody, low complement.
pathogenesis of lupus
defective apoptosis clearnce in cell death, poor clearance allows persistence and deputisation of antigen + immune complex.
what is the prognosis for SLE
80% normal life-expectancy,
90% 10 year survival.
deaths usually due to inc CVS risk, fatal infections/side effects of medications rather than SLE itself
SLE symptoms and signs (20+ answers)
fever, weight loss, fatigue, myalgia, arthralgia, inflammatory arthritis, inc prevelence of AVN due to steroids in femoral head. discoid lupus, malaria rash, photosensitivity, raynaud’s, alopecia, lupus nephritis, leukopenia (low WCC), pneumonitis (pleuisy, pleural effusion). pericarditis, sterile endocarditis, inc ischemic heart disease, anaemia, thrombocytopenia, neurological (cerebral vassculitis, seizures, headache).
diagnosing SLE
FBC, ANA, anti-DNA, Anti-SM, low C3/C4 level (in active and renal affecting disease).
urinalyisis for glomerularnephritis
imaging of other organs (e.g: ECHO, MRI brain, CT lung)
why is anti-DNA good for SLE diagnosis
gives level of disease activity and high specificity.
management of SLE (general/mild)
HCQ (everyon), topical steroid, NSAID (skin and arthralgia). DMARD’s for arthritis/organ involvement (ASA)
classification and management of SLE [severe]
causing sever organ disease (CNA, renal…)
give IV steroid and cyclophosphamide.
classification and management of SLE (unresponsive)
not responding to other treatments
give IV immunoglobulin and ritixumab
monitoring SLE
urinalyisis for blood/protein.
check anti-DNA for disease activity
investigate symptoms as they appear
CVS risk assess (BP + cholesterol.)
what is Sjogren’s
autoimmune lymphocytes infiltrates in exocrine glands.
may be primary or secondary (SLE, RA…)
Sjogren’s symptoms
causes dry mouth/eyes/vagina with arthralgia and fatigue.
parotid swelling, tooth decay, peripheral neuropathy, ILD,
what cancer does Sjogren’s increase risk of
lymphoma
treatment for Sjogren’s
saliva supplement, lubricate eyes, regular dental check-ups, HCQ for arthralgia and fatigue
investigations for Sjogren’s
Schirmers test (occular dryness).
anti-Ro and anti-La
atypical lip biopsy.
what is systemic sclerosis (SS)
CTD, vasomotor disturbance/Raynaud’s + fibrosis + subsequent atrophy of skin/cutaneous tissue.
pathogenesis of SS
excessive collagen deposition causes changes.
in lung/kidney → death
describe the skin involvement in SS
oedematous → indurative → atrophic.
“beaking of nose” and “tight mouth”
what are complications of SS
pulmonary fibrosis/hypertension.
GI malabsorption + small bowel bacterial overgrowth
inflammatory arthritis/myositis
describe the two types of SS
diffuse - rapid skin changes and to trunk, significant early organ involvement, anti-Scl-70 antibody
limited - confined to face, feet, hands, forearms,; organ involvement occurs later; Anti-Centromere antibody
diffuse SS antibody
anti-Scl-70 antibody
limited SS antibody
Anti-Centromere antibody
investigations for SS
ANCA, anti-Scl-70 + organ screening (echo, renal, pulmonary function)
management of SS
raynaud’s/digital ulcers → CCB’s, iloprost, bosentan
renal involvment = ACEi
GI = PPI for reflux
ILD = immunosuppression (cyclophosphamide)
what is mixed Connective tissue diseases (MCTD)
defined condition exibiting symptoms of other CTDs
what diseases does MCTD have symptoms of?
raynauds, arthraliga, myositis, sclerodactyly, ILD, pulmonary hypertension.
diagnosing MTCD
anti-RNP
managing MTCD
regular PFT’s and ECHO’s
manage symptoms accordingly: CCB in raynauds + significant disease = immunosupress
what is anti-phospholipid syndrome (APS)?
recurrent venous/arterial thrombosis + fetal loss
how does APS present
some can have no features, some continual PE/DVT or repeated pregnancy problems, some have associated RA/SLE
when is foetus normally lost in pregnancy in APS
spontaneous 2nd/3rd trimersters
what are complications of APS
inc CVS risk (MI/stroke), PE recurrent cauing pulmoary hypertension, migranes, fetal loss
what is a common cutaneous finding in APS
livedo reticularis.
investigations for APS
thrombocytopenia, prolongation of APTT,
lupus anticoagulant,
anti-cardiolipin antibodies,
anti-Beta2 glycoprotein
management of APS
anticoagulation = life-long warfarin (if pregnancy then LWMH as warfarin is teratogenic)
patients with positive antibodies and no thrombotic episode require no treatment.
what causes gout
urate crystals deposited in joint triggered by trauma, surgery or dehydration.
inc serum uric acid in blood (hyperuricaemia)
where is uric acid derived from
purines (A and G nucleotides).
what can hyperuricaemia occur?
due to renal underexcretion (exacerbated by diuretic or renal failure)
due to diet (alcohol, seafood, red meat)
[also genetic component]
what drug exacerbates gout?
diuretics
where does gout affect
any joint
commonly big toe (1st MTP), then ankle/knees
symptoms of Gout
red, hot swollen joint, acutely and severely painful. resolves in 7-10 days
(mimic of septic arthritis)
what can occur in chronic gout
gouty tophi (painless) occur because build up erupts through skin from soft tissue
erosion/destructive joint arthritis (rare)
investigations and results in Gout
aspirate synovial fluid with polarised microscopy.
needle-shaped + negative birefringence= uric acid crystals
treating gout
acute - NSAID, corticosteroid (once infection not possible), opiod analgesia, colchicine (if unable to tolerate NSAID).
chronic - allopurinol or febuxostat (xanthase oxidase inhibitors) prevent attacks. start on acute attack stops
pseudogout caused by…?
calcium phosphonate crystals
calcium phosphonate crystals on biopsy
rhomboid and positive birefringence
what is chonedrocalcinosis?
deposit in cartilage + other soft tissues in absence of acute inflammation.
pseudogout occurs where?
wrist, knee, ankle commonly
pseudogout treatment
acute = same as gout
chronic/prophylaxis = no treatment
what are some common muscle diseases?
polymyalgia rheumatica, GCA, polymyositis/ dermatomyosistis, fibromyalgia
what is PMR
chronic, common, unknown atieology occurring in elderly.
investigating PMR
inc ESR and CRP (no specific test)
PMR PC
proximal myalgia of hip and shoulder + accompanying morning stiffness. GCA may be present
what condition is PMR closely linked to?
GCA
treatment of PMR
15mg prednisolone very quickly resolves (can be used as diagnostic test). reduce steroid over 18months → resolves.
what is GCA?
giant cell arteritis; commonest systemic vasculitis in adults, elderly, unknown aetiology.
transmural inflammation of intimate media and adventica of arteries (patchy/skip lesions of lymphocytes, macrophages and multi-neucleated cells.)
what occurs due to GCA in vessel wall
lymphocytes, macrophages and multi-neucleated cells cause inflammation and vessel wall thickens causing narrow lumen and ischemia.
PC of GCA (signs and symptoms)
headache (usually occipital/temporal areas)
Visual disturbances, (blurring, vision loss, double vision, blindness)
jaw claudication (eating/talking)
scalp tenderness
focal tenderness on palpation present
thickened and non-pulsing femoral artery.
(fatigue, malaise, fever).
what causes jaw claudication in GCA
what causes visual impairment in GCA
ischema of maxillary artery.
ischema of ophthalmic artery. [prolonged = permenant]
diagnosis of GCA
temporal artery biopsy ASAP. not sensitive (as skip lesions) but 100% specificity.
start treatment ASAP/before biopsy
treating GCA
don’t wait until biopsy - start 40mg/60mg prednisolone (later if visual disturbances) as waiting can cause problems and takes weeks for steroid to remove biopsy-able material completely.
what is polymyositis
idiopathic inflammatory myopathy causing symmetrical, proximal muscle weakness (occasionally myalgia)
who does polymyositis commonly affect
(F>M, 45-60years)
pathogenesis of polymyositis
T-cell mediated cytotoxic process directed against muscle antigens,
CD8+ cells + macrophages surround health tissue and invade/destroy
what antibodies are present in polymyositis
anti-Jo1 + anti-SRP antibiodies
also anti-RNP, ANA
complications of polymyositis
dysphagia in 1/3rd of cases (poor prognostic sign)
ILD can occur (esp. in anti-Jo1 positive people)
investigations for polymyositis
inc inflmaaory markers, inc CK MRI for extent of muscle involvement (inflammation, oedema or scarring) EMG abnormal muscle biopsy crucial = gold standard
management of polymyositis
prednisolone 40mg + MTX/AZA (responds well but slowly to treatment)
dermatopolymyositis (dermatomyositis) features
similar to polymyositis clinically but with skin changes
investigation and treatment same as polymyositis
skin involvement in dermatopolymyositis
V-shaped rash over chest.
Gottron’s papules.
heliotrope rash
dermatopolymyositis has increase risk of what?
malignancy - commonly breast, ovary, colon, lung, bladder, oesophagus.
screen at time of diagnosis and 5 years afterwards (peak risk time)
[[[up to 30% of dermatomyositis arise from cancer, probably as an autoimmune response.]]]
CREST acronym for limited systemic sclerosis
calcinosis, raynaud’s, esophageal dysmotility, sclerodactyly, telangiectasia.
Antiphospholipid syndrome treatment
normally: Warfarin and/or aspirin
if trying to conceive - LMWH and aspirin
