rheumatology Flashcards
what is raised in blood test of polymyositis and dermatomyositis
creatinine kinase
what is polymyositis and dermatomyositis
AI disorders
Polymyositis is a condition of chronic inflammation of muscles.
Dermatomyositis is a connective tissue disorder where there is chronic inflammation of the SKIN AND muscles.
most common cause of septic arthritis
staph aureus
(gram +ve diplococci in clusters)
Gout synovial fluid analysis
needle shaped
negatively birefringent monosodium urate crystals under polarised light
main SE of colchicine
diarrhoea
Gout mx
NSAIDS 1st line
(w PPI for GI protection)
or
COLCHICINE
If CI prednisolone
gout prevention
allopurinol - Inhibits Xanthine oxidase
Wait 3 wks after acute ep
once initiated, ctu during an acute attack
colchicine cover should be used when starting allopurinol
NSAIDs can be used if colchicine cannot be tolerated
2nd line = febuxostat
when do you get anti-dsDNA antibodies
SLE
when do you get anti-CCP antibodies
RA
(more specific than RF)
when do you get anti-GBM antibodies
(glomerular basement membrane)
Goodpasture’s syndrome
when do you get c-ANCA antibodies
small vessel vasculitis e.g. granulomatosis w polyangiitis (AKA Wegener’s granulomatosis)
when do you get p-ANCA antibodies
broad range of conditions inc Primary sclerosing cholangitis (PSC), autoimmune hepatitis and ulcerative colitis.
eosinophilicgranulomatosis w polyangiitis (churg stauss)
presentation of adult onset still’s disease (systemic onset JIA)
Pyrexia (often very high and of uncertain origin at first)
Arthralgia
A fine nonpruritic salmon pink rash
lab results in adult onset still’s disease
v high ferratin
elevated liver enzymes
ANA + RA are -ve
what is GCA/temporal arteritis
systemic vasculitis of the medium and large arteries. It typically presents with symptoms affecting the temporal arteries
what condition does GCA have a link w
polymyalgia rheumatica
who is at highest risk of GCA
white females over 50.
sx of GCA
HEADACHE
- unilateral
- severe
- temple + forehead
scalp tenderness
jaw claudication
blurred/double vision
-> Irreversible painless complete sight loss can occur rapidly
assoc systemic sx:
fever
fatigue
muscle aches
weight loss
peripheral oedema
GCA dx
clinical pres
raised ESR (usually 50+)
temporal artery biopsy = multinucleated giant cells
(skip lesions may be present so ctu steroids even if -ve)
other test results in GCA
Full blood count = normocytic anaemia and thrombocytosis (raised platelets)
Liver function tests =raised ALP
CRP = raised
Duplex ultrasound of the temporal artery = hypoechoic halo sign
mx of GCA and refs
STEROIDS - start b4 dx confirmed
40-60mg pred OD
review response within 48hrs
ctu high dose until sx resolved then slowly wean off
other
- aspirin
- PPI
ref to vascular surgeons for temporal artery biopsy in all suspected GCA
Rheumatology for specialist diagnosis and management
Ophthalmology review as an emergency same day appointment if they develop visual symptoms
pseudogout synovial fluid analysis
brick shaped calcium pyrophosphate crystals that are positively birefringent under polarised light
features of marfan’s syndrome
- tall stature with arm span to height ratio > 1.05
- high-arched palate
- arachnodactyly (long fingers)
- pectus excavatum
- pes planus (flat feet)
- scoliosis of > 20 degrees
- heart: dilation of the aortic sinuses (seen in 90%) which may lead to aortic aneurysm, aortic dissection, aortic regurgitation, mitral valve prolapse (75%),
- lungs: repeated pneumothoraces
- eyes: upwards lens dislocation (superotemporal ectopia lentis), blue sclera, myopia
- dural ectasia (ballooning of the dural sac at the lumbosacral level)
what is marfan’s syndrome + what is its inheritance
autosomal dominant connective tissue disorder
caused by a defect in the FBN1 gene on chromosome 15 that codes for the protein fibrillin-1
who typically gets ank spon
males 20-30 yrs
what is systemic sclerosis
autoimmune inflammatory and fibrotic connective tissue disease.
what types of vasculitis affect the small vessels
Henoch-Schonlein purpura
EOSINIPHILIC Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
Microscopic polyangiitis
Granulomatosis with polyangiitis
what types of vasculitis affect the medium sized vessels
Polyarteritis nodosa
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
Kawasaki Disease
what types of vasculitis affect the large vessels
Giant cell arteritis
Takayasu’s arteritis
which antibodies are raised in Microscopic polyangiitis and Churg-Strauss syndrome (eosinophilic granulomatosis w polyangiitis)
p-ANCA
which antibodies are raised in Granulomatosis with polyangiitis
c-ANCA
what is Henoch-Schonlein Purpura (HSP) and how does it usually present
an IgA vasculitis most common in children < 10
- purpuric rash affecting the lower limbs or buttocks
- joint pain
- abdo pain
- renal involvement - IgA nephritis
often triggered by an URTI (like tonsilitis) or gastroenteritis
what is Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
a small and medium vessel vasculitis.
how does Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome) present
- lung and skin probs
can affect other organs eg kidneys - severe asthma in late teenage years or adulthood
FBC in Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
raised eosinophil levels
main features of Microscopic polyangiitis
renal failure
can also affect the lungs causing shortness of breath and haemoptysis
what is Granulomatosis with polyangiitis
(Wegner’s granulomatosis)
small vessel vasculitis
presentation of Granulomatosis with polyangiitis (Wegener’s granulomatosis)
- nose bleeds
- crusty nasal secretions
- hearing loss
- sinusitis
- saddle shaped nose
- cough, wheeze, haemoptysis (CXR may show consolidation)
- glomerulonephritis
what is polyarteritis nodosa + what is it assoc w
medium vessel vasculitis
hep B
what rash do you get in Polyarteritis Nodosa
livedo reticularis - a mottled, purplish, lace like rash.
what does Polyarteritis Nodosa affect + cause
medium sized vessels in locations such as the skin, gastrointestinal tract, kidneys and heart.
This can cause renal impairment, strokes and myocardial infarction.
features of Kawasaki Disease
usually <5 yrs old
Persistent high fever > 5 days
Erythematous rash
Bilateral conjunctivitis
Erythema and desquamation (skin peeling) of palms and soles
“Strawberry tongue” (red tongue with prominent papillae)
comp of Kawasaki Disease
coronary artery aneurysm
tx of Kawasaki Disease
aspirin
IV immunoglobulins
what is Takayasu’s arteritis + what does it affect
large vessel vasculitis
It mainly affects the aorta and it’s branches. It also affect the pulmonary arteries.
presentation of Takayasu’s arteritis
< 40 yrs w non specific sx
- fever, malaise, muscle aches
- arm claudication !
- syncope
dx of Takayasu’s arteritis
CT or MRI angiography
Doppler ultrasound of the carotids can be useful in detecting carotid disease.
initial therapy of RA
DMARD monotherapy (methotrexate) +/- a short-course of bridging prednisolone.
what is paget’s disease of bone
a disease of increased but uncontrolled bone turnover
Excessive osteoclastic resorption followed by increased osteoblastic activity -> leads to patchy areas of high density (sclerosis) and low density (lysis) = enlarged and mishapen bones w structural probs
presentation of paget’s disease of bone
Typically affects older adults.
More common in males.
Bone pain
Bone deformity
Fractures
Hearing loss can occur if it affects the bones of the ear
classical, untreated features: bowing of tibia, bossing of skull
bloods in paget’s disease of bone
isolated raised ALP
normal calcium
normal phosphate
XR in paget’s disease of bone
Bone enlargement and deformity
“Osteoporosis circumscripta” = well defined OSTEOLYTIClesions that appear less dense compared with normal bone
“Cotton wool appearance” of the skull = poorly defined patchy areas of increased density (sclerosis) and decreased density (lysis)
“V-shaped defects” in the long bones = V shaped osteolytic bone lesions within the healthy bone
paget’s disease of bone mx
bisphosphonates (either oral risedronate or IV zoledronate) - interfere with osteoclast activity and seem to restore normal bone metabolism
NSAIDs for bone pain
Calcium and vitamin D supplementation, particularly whilst on bisphosphonates
Surgery is rarely required for fractures, severe deformity or arthritis
what are px w paget’s disease of the bone more at risk of
osteosarcoma
Spinal stenosis and spinal cord compression
features of Goodpasture’s syndrome (ant-GBM disease)
pulmonary haemorrhage
rapidly progressive glomerulonephritis
this typically results in a rapid onset AKI
nephritis → proteinuria + haematuria
ix for Goodpasture’s syndrome (ant-GBM disease)
renal biopsy: linear IgG deposits along the basement membrane
raised transfer factor secondary to pulmonary haemorrhages
mx of Goodpasture’s syndrome (ant-GBM disease)
plasma exchange (plasmapheresis)
steroids
cyclophosphamide
what is Buerger disease (also known as thromboangiitis obliterans)
inflammatory condition that causes thrombus formation in the small and medium-sized blood vessels in the distal arterial system (affecting the hands and feet)
buerger disease presentation
typically affects men aged 25 – 35 and has a very strong association with smoking
(consider in px w no other RFs for atherosclerosis)
- painful, blue discolouration to the fingertips or tips of the toes
- pain is often worse at night.
- may progress to ulcers, gangrene and amputation
finding on angiograms in buerger disease
Corkscrew collaterals - where new collateral vessels form to bypass the affected arteries
buerger disease tx
stop smoking -> signif improvement
IV iloprost?
mx septic arthritis
IV flucloxacillin
Penicillin Allergy = Clindamycin
what is psoriatic arthropathy
inflammatory arthritis associated with psoriasis and is classed as one of the seronegative spondyloarthropathies
occurs in 10-20% px w psoriasis
Asymmetrical oligoarthritis
affects 1-4 joints at any given time, often on only one side of the body
Symmetrical polyarthritis
More than four joints are affected, such as the hands, wrists and ankles
Spondylitis
back stiffness and pain. It involves the axial skeleton (spine and sacroiliac joints)
Arthritis mutilans
severe form of psoriatic arthritis. It affects the phalanges (the bones of the fingers and toes). There is osteolysis (destruction) of the bones around the joints, leading to progressive shortening of the digits. The skin folds as the digit shortens, giving an appearance described as a telescoping digit
“pencil-in-cup” appearance on XR
diss between psoriatic arthritis + RA
Psoriatic arthritis tends to affect the distal interphalangeal (DIP) joints and axial skeleton, whereas rheumatoid arthritis tends not to affect these joints
Also has dactylitis + onycholysis
Psoriasis
presentation psoriatic arthritis
Plaques of psoriasis on the skin
Nail pitting (tiny indents in the fingernails and toenails)
Onycholysis (separation of the nail from the nail bed)
Dactylitis (inflammation of the entire finger)
Enthesitis (inflammation of the entheses, which are the points of insertion of tendons into bone)
XR changes in psoriatic arthritis
Periostitis (inflammation of the periosteum, causing a thickened and irregular outline of the bone)
Ankylosis (fixation or fusion of the bones at the joint)
Osteolysis (destruction of bone)
Dactylitis (inflammation of the whole digit, seen as soft tissue swelling)
MX psoriatic arthritis
Non-steroidal anti-inflammatory drug (NSAIDs)
Steroids
DMARDs (e.g., methotrexate, leflunomide or sulfasalazine)
Anti-TNF medications (etanercept, infliximab or adalimumab)
Ustekinumab is a monoclonal antibody that targets interleukin 12 and 23
what is Ankylosing spondylitis (AS)
inflammatory condition affecting the axial skeleton (mainly the spine and sacroiliac joints), causing progressive stiffness and pain. It is also known as axial spondyloarthritis. It is part of the seronegative spondyloarthropathy group
main affected joints are the sacroiliac joints and the vertebral column joints
can progress to spine and sacroiliac joint fusion
gene link in Ankylosing spondylitis (AS)
HLA-B27 gene
Ankylosing spondylitis presentation
young adult male in their 20s.
sx develop gradually over at least three months.
Pain and stiffness in the lower back
Sacroiliac pain (in the buttock region)
- worse with rest + improves with movement, worsens at night + in the morning, may wake them. 30mins to improve in the morning.
other sx:
Chest pain related to the costovertebral and sternocostal joints
Enthesitis (inflammation of the entheses, where tendons or ligaments insert into bone)
Dactylitis (inflammation of the entire finger)
Vertebral fractures (presenting with sudden-onset new neck or back pain)
Shortness of breath relating to restricted chest wall movement)
conditions assoc w ankylosing spondylitis
A – Anterior uveitis
A – Aortic regurgitation
A – Atrioventricular block (heart block)
A – Apical lung fibrosis (fibrosis of the upper lobes of the lungs)
A – Anaemia of chronic disease
A - Achilles tendonitis
what is schobers test
assesses spinal mobility
Find L5, mark 10cm above 5cm below + bnd forward
A length of less than 20cm indicates a restriction in lumbar movement
ix ankylosing spondylitis
Inflammatory markers (e.g., CRP and ESR) may rise with disease activity
HLA B27 genetic testing
X-ray of the spine and sacrum
MRI of the spine can show bone marrow oedema early in the disease before there are any xray changes
XR ankylosing spondylitis
A “bamboo spine” is the typical x-ray finding in the later stages of ankylosing spondylitis, where there is fusion of the sacroiliac and spinal joints.
Squaring of the vertebral bodies
Subchondral sclerosis and erosions
Syndesmophytes (areas of bone growth where the ligaments insert into the bone)
Ossification of the ligaments, discs and joints (these structures start turning into bone)
Fusion of the facet, sacroiliac and costovertebral joints
SACROILIITIS
mx ankylosing spondylitis
rheumatology MDT
Non-steroidal anti-inflammatory drugs (NSAIDs) are first-line
Anti-TNF medications are second-line (e.g., adalimumab, etanercept or infliximab)
Secukinumab or ixekizumab are third-line (monoclonal antibodies against interleukin-17)
Upadacitinib is another third-line option (JAK inhibitor)
Intra-articular steroid injections may be considered for specific joints.
presentation reactive arthritis
‘Can’t see, can’t pee, can’t climb a tree’
within 4 wks of initial infection - sx generally last around 4-6 months
around 25% of patients have recurrent episodes whilst 10% of patients develop chronic disease
asymmetrical oligoarthritis of lower limbs
dactylitis
symptoms of urethritis
conjunctivitis (seen in 10-30%)
anterior uveitis
circinate balanitis (painless vesicles on the coronal margin of the prepuce)
keratoderma blenorrhagica (waxy yellow/brown papules on palms and soles)
what is reactive arthritis
Reactive arthritis is defined as an arthritis that develops following an infection where the organism cannot be recovered from the joint.
XR for pseudogout
chondrocalcinosis
other changes similar to osteoarthritis (LOSS)
RFs osteoarthritis
obesity
age
occupation
trauma
F
FHx
joints commonly affected by osteoarthrtitis
Hips
Knees
Distal interphalangeal (DIP) joints in the hands (furthest out)
Carpometacarpal (CMC) joint at the base of the thumb
Lumbar spine
Cervical spine (cervical spondylosis)
XR changes in osteoarthritis
L – Loss of joint space
O – Osteophytes (bone spurs)
S – Subarticular sclerosis (increased density of the bone along the joint line)
S – Subchondral cysts (fluid-filled holes in the bone)
degenerative changes
sx osteoarthritis
joint pain + stiffness
- worsen w activity + at end of day
general signs osteoarthritis
Bulky, bony enlargement of the joint
Restricted range of motion
Crepitus on movement
Effusions (fluid) around the joint
signs of osteoarthritis in the hand
Heberden’s nodes (in the DIP joints)
Bouchard’s nodes (in the PIP joints)
Squaring at the base of the thumb (CMC joint)
Weak grip
Reduced range of motion
how to make dx osteoarthritis
> 45, typical pain assoc w activity + no morning stiffness (/<30 mins)
mx osteoarthritis
non-pharm:
Therapeutic exercise
Weight loss if overweight to reduce load
Occupational therapy
pharm:
Topical NSAIDs first-line for knee osteoarthritis
Oral NSAIDs (WITH A PPI for gastroprotection) - best used intermittently for flares
Weak opiates and paracetamol are only recommended for short-term, infrequent use
Intra-articular steroid injections may temporarily improve symptoms - up to 10 weeks
Joint replacement in severe cases
what is RA
inflam arthritis
AI condition -> chronic inflam in the synovial lining of the joints, tendon sheaths and bursa
symmetrical polyarteritis
usually dev in middle age
RFs RA
F
smoking
obesity
FHx - HLA DR4 gene assoc
presentation RA
pain, stiffness + swelling in joints
symmetrical distal polyarthritis affecting the small joints of the hands and feet
worse w rest + improve w activity
worst in morning
what are some extra-articular manifestations of RA
Eyes: dry eyes, scleritis, UVEITIS
Skin: leg ulcers, rashes
Heart: PERICARDITIS
Lungs: PULMONARY NODULES, pulmonary fibrosis
Kidneys: amyloidosis
RHEUMATOID NODULES
Anaemia
Hepatomegaly
Muscle wasting
Tendon rupture
DEPRESSION
most common affected joints in RA
Metacarpophalangeal (MCP) joints (closest to you)
Proximal interphalangeal (PIP) joints (middle)
Wrist
Metatarsophalangeal (MTP) joints (in the foot)
Also larger joints: ankle, knee, hips + shoulders, + cervical spine
palpation of joints in RA
tenderness and synovial thickening, giving them a “boggy” feeling
what is palindromic rheumatism
self-limiting episodes of inflammatory arthritis affecting only a few joints.
sx last days, then completely resolve. Joints normal in-between
late hand signs in RA
Z-shaped deformity to the thumb
Swan neck deformity (hyperextended PIP and flexed DIP)
Boutonniere deformity (hyperextended DIP and flexed PIP)
Ulnar deviation of the fingers at the MCP joints
what is felty’s syndrome
triad of rheumatoid arthritis, neutropenia and splenomegaly
dx RA
urgent rheumatology referral for patients with persistent synovitis (to be seen within three weeks)
bloods + NSAIDs when waiting
ix:
Rheumatoid factor
Anti-CCP antibodies
Inflammatory markers, such as C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR)
X-rays of the hands and feet for bone changes
Ultrasound or MRI can be used to detect synovitis (useful when clinical findings are unclear)
XR changes in RA
Periarticular osteopenia
Loss of joint space
Erosions
Soft tissue swelling
Subluxation
how to monitor success of RA tx
CRP + Disease Activity Score 28 Joints (DAS28)
tx RA
STEROIDS for acute exacerbations / initial pres
(IM methylprednisolone)
DMARDs monotherapy
= METHOTREXATE weekly
- must give FOLATE supplements w
- need to monitor FBC + LFTs due to the risk of myelosuppression and liver cirrhosis
(others: SULFASALAZINE, leflunomide, hydroxychloroquine)
BIO THERAPY - if inadequate response to at least 2 DMARDs
TNF-alpha blockers e.g. infliximab
Anti-CD20 - rituximab
risks = reactivation of TB
what skin changes might you see in dermatomyositis
GOTTRON lesions (SCALY erythematous patches) on the knuckles, elbows and knees
HELIOTROPE RASH (a purple rash on the face and eyelids)
Periorbital oedema (swelling around the eyes)
Photosensitive erythematous rash on the back, shoulders and neck
ix dermatomyositis
muscle biopsy
presentation of polymyositis and dermatomyositis
gradual-onset, symmetrical, proximal muscle weakness, causing difficulties standing from a chair, climbing stairs or lifting overhead
may be muscle pain (myalgia)
dermatomyositis will also have skin features
causes of raised creatine kinase
Polymyositis and dermatomyositis
Rhabdomyolysis
Acute kidney injury
Myocardial infarction
Statins
Strenuous exercise
what are anti-Jo-1 antibodies assoc w
polymyositis
mx polymyositis and dermatomyositis
steroids
most common infection triggers for reactive arthritis
gastroenteritis or STIs.
Chlamydia may cause reactive arthritis. Gonorrhoea typically causes septic arthritis rather than reactive arthritis
ix reactive arthritis
exclude septic - abx till then
joint aspiration - microscopy, culture and sensitivity testing for infection, and crystal examination for gout and pseudogout
- there will be sterile synovial fluid with a high white blood cell count (it is an aseptic pathological process, the causative bacteria will not be there)
mx reactive arthritis
Treatment of the triggering infection
NSAIDs
Steroid injection into the affected joints
Systemic steroids may be required, particularly where multiple joints are affected
most resolve in 6 mths
what is SLE
inflammatory autoimmune connective tissue disorder. It is “systemic” because it affects multiple organs and systems. “Erythematosus” refers to the typical red malar rash across the face
who usually gets SLE
Women
Asian, African, Caribbean and Hispanic ethnicity
Young to middle-aged adults
course of SLE
relapsing-remitting
antibodies in SLE
ANA - 85% have this, also raised in other things e.g. AI hepatitis
Anti-double stranded DNA (anti-dsDNA) antibodies are highly specific to SLE - half have these, levels vary w disease acitivity so gd for monitoring
30% will have +ve antiphospholipid antibodies as is assoc w antiphospholipid syndrome
presentation SLE
many non-specific symptoms:
Fatigue
Weight loss
Arthralgia (joint pain)
Non-erosive arthritis
Myalgia (muscle pain)
Fever
Photosensitive malar rash
Lymphadenopathy
Splenomegaly
Shortness of breath
Pleuritic chest pain
Mouth ulcers
Hair loss
Raynaud’s phenomenon
Oedema (due to nephritis)
Pericarditis
ix SLE
ANA Autoantibodies
FBC - anaemia of chronic disease, low WCC, low platelets
CRP + ESR may be raised with active inflammation
C3 and C4 levels may be decreased in active disease
Urinalysis and urine protein:creatinine ratio shows proteinuria in lupus nephritis
Renal biopsy may be used to ix this^
Anti-Ro and anti-La antibodies
most associated with Sjögren’s syndrome
Anti-Scl-70 antibodies
most associated with diffuse cutaneous systemic sclerosis
Anti-centromere antibodies
most associated with limited cutaneous systemic sclerosis
comps SLE
CVD - chronic inflam BVs
pericarditis
infection
anaemia
pleuritis
interstitial lung dis
lupus nephritis
neuropsychiatric SLE - optic neuritis, transverse myelitis or psychosis
recurrent miscarriage
VTE - assoc w antiphospholipid syndrome 2ndary to it
mx SLE
Suncream and sun avoidance for photosensitive malar rash.
First-line options include:
Hydroxychloroquine - reduce immune system activity
NSAIDs
Steroids (e.g., prednisolone)
more resistant/ severe
DMARDs (e.g., methotrexate)
Biologic therapies
- Rituximab (a monoclonal antibody that targets the CD20 protein on the surface of B cells)
- Belimumab (a monoclonal antibody that targets B-cell activating factor)
what will be raised in adult onset Still’s disease
serum ferratin
features of adult onset stills disease
arthralgia
rash = salmon pink, maculopapular
pyrexia - rises late afternoon + accompanies worsening of joint sx + rash
lymphadenopathy
RF + ANA -ve
mx adult onset Still’s disease
NSAIDs - trial for 1 wk b4 steroids
steroids - control sx
if sx persist consider methotrexate, IL-1 or anti-TNF therapy
features of limited systemic sclerosis (CREST syndrome)
Calcinosis
Raynaud’s phenomenon
oEsophageal dysmotility
Sclerodactyly
Telangiectasia
nail changes in iron deficiency anaemia
koilonychia - flaky, concave nails
what to do b4 surgery in px w RA
Anteroposterior and lateral cervical spine radiographs preoperatively to screen for atlantoaxial subluxation
- px wld need c spine collar
what is antiphospholipid syndrome
AI disorder caused by antiphospholipid antibodies
- they target the proteins that bind to the phospholipids on the cell surface -> inflammation + increasing the risk of thrombosis (blood clots).
Can occur in isolation or assoc with AI conditions, particularly SLE
antibodies in antiphospholipid syndrome
Lupus anticoagulant
Anticardiolipin antibodies
Anti-beta-2 glycoprotein I antibodies
comps antiphospholipid syndrome
VTE (e.g., DVT + PE)
Arterial thrombosis (e.g., stroke, MI and renal thrombosis)
Pregnancy-related complications (e.g., recurrent miscarriage, stillbirth and pre-eclampsia)
Catastrophic antiphospholipid syndrome is a rare complication with rapid thrombosis in multiple organs within a few days. This has a high mortality rate
rash assoc w antiphospholipid syndrome
livedo racemosa - purple lace-like (reticular) rash that gives a mottled appearance to the skin
mx antiphospholipid syndrome
Long-term warfarin with a target INR of 2-3 is used to prevent thrombosis.
LMWH (e.g., enoxaparin) + ASPIRIN are used in pregnancy to reduce the risks. Warfarin is CI in preg.
what is fibromyalgia
a syndrome characterised by widespread pain throughout the body with tender points at specific anatomical sites
fibromyalgia features
chronic pain: at multiple site, sometimes ‘pain all over’
lethargy
cognitive impairment: ‘fibro fog’
sleep disturbance, headaches, dizziness are common
gout RFs
Male
Family history
Obesity
High purine diet (e.g., meat and seafood)
Alcohol
Diuretics
Cardiovascular disease
Kidney disease
typical joints affected by gout
The base of the big toe – the metatarsophalangeal joint (MTP joint)
The base of the thumb – the carpometacarpal joint (CMC joint)
Wrist
XR of a joint affected by gout
Maintained joint space (no loss of joint space)
Lytic lesions in the bone
Punched out erosions
Erosions can have sclerotic borders with overhanding edges
SE colchicine
Abdominal symptoms and diarrhoea
typical presentation pseudogout
a patient over 65 years old with a rapid-onset hot, swollen, stiff and painful knee. Other commonly affected joints are the shoulders, hips and wrists.
mx pseudogout
Symptomatic management options include:
NSAIDs (e.g., naproxen) first-line (co-prescribed with a proton pump inhibitor for gastroprotection)
Colchicine
Intra-articular steroid injections (septic arthritis must be excluded first)
Oral steroids
Features of systemic onset JIA
Subtle salmon-pink rash
High swinging fevers
Enlarged lymph nodes
Weight loss
Joint inflammation and pain
Splenomegaly
Muscle pain
Pleuritis and pericarditis
how does macrophage activation syndrome (MAS) present
an acutely unwell child with:
disseminated intravascular coagulation (DIC)
anaemia
thrombocytopenia
bleeding
non-blanching rash
It is life threatening
key ix finding in macrophage activation syndrome (MAS)
low ESR
what is macrophage activation syndrome (MAS)
severe activation of the immune system with a massive inflammatory response
how does oligoarticular JIA present
<4 joints
usually single joint, the larger ones (knee/ankle)
girls < 6 yrs
what other thing do you usually get with oligoarticular JIA
anterior uveitis
what is enthesitis-related arthritis
the paediatric version of the seronegative spondyloarthropathy group of conditions
px have inflam arthritis as well as enthesitis (enthesis is the pt where the tendon of a muscle inserts into a bone)
most have HLA B27 gene
what are the seronegative spondyloarthropathy group of conditions that affect adults
ankylosing spondylitis, psoriatic arthritis, reactive arthritis and inflammatory bowel disease-related arthritis
what scan can demonstrate enthesitis
MRI
how do you get enthesitis
traumatic stress, such as through repetitive strain during sporting activities
or
an autoimmune inflammatory process
which areas to palpate to elicit tenderness of the entheses
Interphalangeal joints in the hand
Wrist
Over the greater trochanter on the lateral aspect of the hip
Quadriceps insertion at the anterior superior iliac spine
Quadriceps and patella tendon insertion around the patella
Base of achilles, at the calcaneus
Metatarsal heads on the base of the foot
what to assess px for when they have enthesitis-related arthritis
consider signs and symptoms of psoriasis (psoriatic plaques and nail pitting) + inflammatory bowel disease (intermitted diarrhoea and rectal bleeding).
Will be prone to anterior uveitis - ophthalmologist for screening, even if they are asymptomatic.
Juvenile Psoriatic Arthritis examination signs
Plaques of psoriasis on the skin
Pitting of the nails (nail pitting)
Onycholysis, separation of the nail from the nail bed
Dactylitis, inflammation of the full finger
Enthesitis, inflammation of the entheses, which are the points of insertion of tendons into bone
mx JIA
specialist in paediatric rheumatology, with a specialist MDT
NSAIDs, such as ibuprofen
Steroids, either oral, intramuscular or intra-artricular in oligoarthritis
Disease modifying anti-rheumatic drugs (DMARDs), such as methotrexate, sulfasalazine and leflunomide
Biologic therapy, such as the tumour necrosis factor inhibitors etanercept, infliximab and adalimumab
poor prognostic features in RA
rheumatoid factor positive
anti-CCP antibodies
poor functional status at presentation
X-ray: early erosions (e.g. after < 2 years)
extra articular features e.g. nodules
HLA DR4
insidious onset
alternative to allopurinol in gout prophylaxis
febuxostat
what can sometimes cause dermatomyositis/polymyositis
underlying cancer -paraneoplastic syndromes (derm more common)
a viral infection may be the trigger (e.g., Coxsackie virus or HIV)
do a CT CAP
causes drug induced lupus
procainamide
hydralazine
isoniazid
minocycline
phenytoin
features drug induced lupus
antibodies
arthralgia
myalgia
skin (e.g. malar rash) and pulmonary involvement (e.g. pleurisy) are common
ANA positive in 100%, dsDNA negative
anti-histone antibodies are found in 80-90%
score for hypermobility
Beighton score
tx for methotrexate OD
folinic acid
pathogenesis of visual impairment in GCA
secondary to ANTERIOR ISCHAEMIC OPTIC NEUROPATHY from inflammation of the posterior ciliary arteries that causes optic nerve infarction
what to give if can’t take bisphosphonates in osteoporosis mx
risedronate or etidronate
monitoring whilst on hydroxychloroquine
visual acuity testing at baseline, and then every 6-12 months as it can cause retinopathy
