ask disease profiles pt 2 Flashcards
what are inflammatory myopathies characterised by
weakness
what is polymayalgia rheumatica characterised by
pain and stiffness
what is fibromyalgia characterised by
pain and fatigue
presentation of muscle diseases
muscle pain, weakness, tiredness, stiffness, abnormal blood test, other organ features
what is a myopathy
a disease of the muscle where the fibres do not function properly
what does a myopathy result in
muscle weakness
acquired inflammatory myopathies
polymyositis, dematositis and immune mediated necrotising myopathy
prevalence of polymyositis and dermatomyositis
1/100,000
peak incidence of polymyositis and dermatomyositis
40-50 years
female to ale ratio of polymyositis and dermatomyositis
40-50
what does polymyositis and dermatomyositis put people at risk of
malignancy
clinical features of polymyositis and dermatomyositis
insidious onset, worsening over months, usually symmetrical, proximal muscles, often specific problems like brushing hair and climbing stairs and mild myalgia in 25-50%
what is seen in dermatomyositis
gottrons sign, heliotrope rash and shawl sign
what is gottrons sign
red rash over the back of fingers
what is helioscope rash
rash round the eyes
greatest risk of malignancy in people with dermatomyositis and polymyositis
men over 45
investigations of polymyopathy and dermatomyopathy
muscle enzymes (CK), inflammatory markers, electrolytes, calcium, PTH, TSH, ANA, anti-Jo-1, myositis, specific antibodies, EMG
EMG findings in myopathies
increased fibrillations, abnormal motor potentials, complex repetitive discharges
diagnosis of muscle myopathies
muscle biopsy, MRI
muscle biopsy findings in muscle myopathies
perivascular inflammation and muscle necrosis
MRI findings of muscle myopathies
muscle inflammation, oedema, fibrosis and calcification
treatment of muscle myopathies
cotricosteroids and immunosuppression
who gets polymyalgia rheumatica
over 50s
what is the prevelance of polymyalgia rheumatica
1%
what is polymyalgia rheumatica associated with
temporal arteritis and giant cell arteritis
clinical presentation of polymyalgia rheumatica
ache in shoulder and hip girdle, morning stiffness, usually symmetrical fatigue, anorexia, weight loss and fever, reduced movement of shoulders, neck and hips muscle strength is normal
what is temporal arteritis/giant cell arteritis
granulomatous arteritis of large vessels
features of granulatous arteritis
headache, scalp tenderness, jaw claudication, visual loss, tender enlarged non pulsatile temporal arteries
diagnosis of granulatous arteritis
raised ESR,plasma viscosity, CRP, temporal artery biopsy, temporal artery USS
treatment of granulates arteritis
low dose steroids gradual reduction of steroids over 18 months to 2 years
what does of prednisolone for PMR
15mg
what does of prednisolone for GCA
40-60 mg
what is a common cause of chronic musculoskeletal pain
fibromyalgia
is fibromyalgia associated with inflammation
no
female to male ratio of fibromyalgia
6:1
what might trigger fibromyalgia
emotional or physical trauma
what is the diagnostic criteria for fibromyalgia
ACR
treatment of fibromyalgia
patient education, multidisciplinary response, graded exercise programme, CBT, acupuncture, anti depressants, analgesia, gabapentin and pregabalin
what is vasculitis
inflammation of blood vessels
what does primary vasculitis occur from
inflammatory response that targets the vessel walls and has no known cause
what does secondary vasculitis occur from
triggered by infection a drug, or a toxin or another type of inflammatory disorder or cancer
clinical features of vasculitis
depends on which vessel it affects, systemic symptoms - fever, malaise, weightless and fatigue
classifications of vasculitis
immune complex mediated vasculitis, medium vessel vasculitis, large vessel vasculitis, anti GBM disease and ANCA associated small cell vasculitis
what examples of immune complex mediated vasculitis
cryoglobulinemic vasculitis, IgA vasculitis, hyppelmentamic urticarial Vasculitis
large vessel vasculitis
takayaski arteritis and giant cell arteritis
who does takayaski arteritis affect
females under 40
signs of takayaski arteritis
bruit in carotid artery and blood pressure differences
who gets giant cell arteritis
GCA over 50 years
symptoms of giant cell arteritis
temporal headache, temporary visual disturbances, blindness and/or jaw claudication
what is giant cell arteritis associated with
polymyalgia rheumatica
investigations of large vessel vasculitis
ESR,plasma viscosity and CRP is raised US and CT
management of large vessel vasculitis
40-60 mg prednisolone, steroid sparing agents (leflunamide, methotrexate, toclizumab
anca + small vessel vasculitis
churg strauss, wegeners, microscopic polyangitis
anca- small vessel vasculitis
cryoglobulnemia
what is wegeners also known as
granulonatosis with polangitis
what is churg strauss also known as
eosinophilic granulommatosis with polyangitis
GPA male to female ratio
1.5:1
age of onset of GPA
35-55 years
what is common in GPA
constitutional symptoms and arthralgia
ENT features of GPA
sinusitis, nasal crustin, epistaxis, mouth ulcers, sensorineural deafness, otitis media and deafness, saddle nose, sub glottic inflammation
ocular features of GPA
conjunctivitis, episcleritis, uveitis, optic nerve vasculitis, retinal artery occlusion, proptosis
resp symptoms of GPA
cough, heamoptysis , pulmonary infiltrates, diffuse alveolar haemorrhage, caveatting nodules on CXR
cutaneous features of GPA
palpale purpura, cutaneous ulcers
renal features of GPA
necrotising glomerulonephritis
nervous system manifestations of GPA
mononeuritis multiplex, sensorimotor polyneuropathy, cranial nerve palsies
difference between EGPA and GPA
late onset of asthma, high eosinophil count and ANCA specificity
what does microscopic polyangitis cause
renal impairment
what is Henoch - schonein purport
an acute immunoglobulin IgA mediated disorder
what does HSP involve
generalised vasculitis of small vessels of skin, gI tract, kidneys, joints and rarely lungs and CNS
what is the common infection leading to HSP
group A strep
preceding illness predates when in HSP
1-3 week s
what is the presentation of HSP
purpuric rash typically over buttocks and lower limbs, colicky abdominal pain, bloody diarrhoea joint pain, renal involvement
investigations of vasculitis
urine dipstick, FBc, liver and renal profile, inflammatory markers, ANCA and specific antibodies, connective tissue disease screen, compliment levels, Cxr, CT, nerve conduction tests, tissue biopsy
what is ANCA
auto antibodies against antigens in the cytoplasm if neutrophil granulocytes
what is used to detect ANCA
immuno fluorescenze
cANCA appearance
more round
pANCA appearance
more globular
varum
ditsal towards
valgum
distal part away
complications of knee replacememnt
deep infection, pain, stiffness, early failure/ loosening and medical complications and blood clots
what stress does MCL resist
valgus
what tsress does LCL resist
LCL
what stress does ACL RESIST
internal rotatio of the tibia
grade 1 ligament injury
sprain
grade 2 ligament injdury
partial tear
grade 3 ligamnet injury
complete tear
PCL injury leads to
recureent hyperextension
acute locked knee inidctaotes
displaced bucket handle meniscal tear
what isFAI
altered morphology of femoral neck or acetabular
PIncer typle iningement
acetabular
where is hip arthritis felt
in the groin
presentation of FAI
activity related pain in groin, difficulty sittig, C sign positive
management of FAI
observation in asymtomatic - symptomatic surgery
avascular necorosis
usually idiopathic faure of perfusion to the femoral head
risk factors of avascukar necrosis
STEROID AND TRAUMA
presentation of avascular necrosis
insiduois onset of groin pain, exacerbated by stairs or impact, examination normla
staging system for avascular necorsis
STeinberg
management of avascular necrosis
drilling,,
what stage does avascular necrosis become irreversible
stage 3
iTOH is
local hyperaremia and impaired venous return wuth marrow oedema and increased intramedullary pressure
diagnosis of ITOH
elevation ESR, radiographs, MRI and bone scan