Rheumatology Flashcards
What are the red flags for back pain?
age <20 or >55 sphincter disturbance current or recent infection history of malignancy morning stiffness constant or progressive pain neurological disturbance bilateral or alternating leg pain FLAWS thoracic back pain acute onset in elderly people nocturnal pain
what does a nerve root lesion at L2 cause?
weakness in hip flexion and adduction
what does a nerve root lesion at L3 cause?
weakness in knee extension and hip adduction
what does a nerve root lesion at L4 cause?
weakness in foot dorsiflexion + inversion + knee extension
what does a nerve root lesion at L5 cause?
weakness in: great toe dorsiflexion foot dorsiflexion + inversion knee flexion hip extension + abduction
what does a nerve root lesion at S1 cause?
weakness in foot plantar flexion + eversion
nerve roots of knee jerk reflex?
L3,L4
nerve roots of ankle jerk reflex?
S1,S2
Ix lower back pain?
MRI only if suspected malignancy, infection, fracture, CES or ank spond
DRE
Mx lower back pain?
c: physical activity and exercise
M: NSAIDS + PPI if >45
Other: group exercise programme
manual therapy (techniques such as massage)
radio frequency denervation
epidural injections of Land steroid for acute/severe sciatica
what is rheumatoid arthritis?
chronic systemic inflammatory condition characterised by a symmetrical defaming poly arthritis
epidemiology RA?
F>M (2:1), 50-60yo, 1% prevalence (higher in smokers)
HLA DR1, DR4
what are some poor prognostic indicators of rheumatoid arthritis?
\+ve RhF insidious onset early erosions HLA DR4 \+ve anti-CCP extra-articular features
S/S rheumatoid arthritis?
swollen, painful small joints in hands and feet
ulna deviation of MCP and radial deviation at wrist
stiffness better with exercise/worse in morn
swan neck, boutonniere, z-thumb
associated features rheumatoid arthritis?
eyes - episcleritis, keratoconjunctivitis sicca
neck - Atlanta-axial subluxation (may cause cord compression)
heart -pericarditis
lungs - fibrosis, rheumatoid nodules
hands - de quervains tenosynovitis, CTS, trigger finger
speen -splenomegaly, felty’s syndrome
kidneys - amyloid
all - rheumatoid nodules
what is Felty’s syndrome?
SANTA Splenomegaly anaemia neutropenia thrombocytopenia arthritis
Diagnostic criteria for rheumatoid arthritis?
American College of Rheumatology Criteria
NICE recommend clinical diagnosis
ix rheumatoid arthritis?
- DAS28 [disease activity score]
- squeeze test positive (pain across MCPJ or MTPJ)
Bloods: FBC (anaemia, raised ESR, CRP), RhF (+ve 70%), anti-CCP (90-95% specific, 80% sensitive), ANA(+ve in 30%)
TJC/SJC
Imaging: XR (baseline), USS (synovitis), MRI, CXR
what are TJC and SJC
tender joint count and swollen joint count (part of DAS28)
What should be monitored in rheumatoid arthritis?
DAS28, CRP
if DAS28 >5.1 consider stepping up Mx
Mx rheumatoid arthritis?
1st line: conventional DMARD
2nd line: combination cDMARD therapy (2x)
3rd line: biological DMARD + cDAMRD
Surgery: ulna stylectomy, joint prosthesis
Flare ups: corticosteroids (PO, IM methylprednisolone)+/-NSAIDs
Give some examples of cDMARDs?
methotrexate sulfasalazine hydroxychloroquine MMF cyclophosphamide azathioprine
Give some examples of bDMARDs?
(TNF-a inhibitors, B cell/T depletion) EtanercepT (SE demyelination) Infliximab Adalimumab Rituximab
How are methotrexate, sulfasalazine and hydroxychloroquine monitored?
M- regular FBC and LFT (risk of myelosuppression and cirrhosis)
S - salicylate so not given if aspirin sensitive
H - annual visual acuity testing. OK IN PREGNANCY.
what joints does OA affect?
weight bearing: hip and knee
hands: CMCJ, DIPJ, PIPJ
What joints does RA affect?
MCPJ, PIPJ
What is swan neck deformity?
stretching of the volar plate
PIPJ hyperextension
DIPJ flexion
what is seen on XR in OA?
LOSS loss of joint space osteophytes subchondral cysts subchondral sclerosis
what is seen on XR in rheumatoid?
LESS loss of joint space erosions (periarticular) soft tissue swelling subluxation and deformity
aetiology of gout?
monosodium urate crystals deposition in and around joints -> erosive arthritis
epidemiology of gout?
M>F 5:1
gout associations?
HTN
IHD
Metabolic syndrome
Causes of gout?
hereditary
drugs (loop diuretics, thiazide diuretics,aspirin,)
decreased excretion (primary gout, renal impairment)
increased cell turnover (lymphoma, leukaemia, haemolysis
ETOH XS
Purine rich foods
S/s gout?
mono arthritis with severe joint inflammation (ankle, foot, hand, wrist elbow) 60% get podagra trophy radiolucent kidney stones interstitial nephritis
Ix gout?
polarised light microscopy (-ve birefringent needle shaped crystals) XR (late; para-articular punched out erosions, reduced joint space) serum urate (increased OR normal)
Acute Mx gout?
Acute:
1st line = colchicine, NSAIDs (not aspirin) +/- PPI
renal impairment = PO steroids (prednisolone 15mg /d)
intraarticular steroid injection
follow up in 4-6w and check BP, HbA1c, serum urate, U&E and lipids -> consider urate lowering therapy
- do not stop allopurinol if already established
- do not stop aspirin 75 if for cardioprotective
Chronic mx for gout?
conservative: WL, avoid ETOH XS, avoid prolonged fasting
urate lowering therapy: started after inflammation stopped
- XO inhibitors: 1. allopurinol, 2. febuxostat
- uricosuric drugs: probenecid, losartan
- recombinant urate oxidase: rasbicurase
what are the S/S of pseudo gout?
mono arthritis (knee, wrist, hip - BIGGER JOINTS)
RFs for pseudo gout?
increased age OA DM hypothyroidism hyperparathyroidism hereditary haemochromatosis Wilsons
Ix pseudo gout?
polarised light microscopy (+ve birefringent, rhomboid shaped crystals)
XR chondrocalcinosis
Mx pseudo gout?
analgesia
NSAIDs
PO/IM/intra-articular steroids
what are seronegative spondyloarthropathies?
a group of inflammatory arthritidies affecting the spine and peripheral joints with no production of rheumatoid factors and associated with HLA-B27 allele
Give some examples of seronegative spondyloarthropathies?
PEAR HEADS Psoriatic arthritis Enteropathic arthritis Ankylosing spondylitis Reactive arthritis HLA B27 allele Enthesitis Axial, asymmetrical, oligoarthritis Dactylitis Seronegative
what is the key difference between ankylosing spondylitis and psoriatic arthritis?
lack of hand S/S in AS
What are the S/S of ankylosing spondylitis?
back pain relieved by exercise anterior chest pain (costochondritis) SOB (pulmonary fibrosis) morning stiffness eye pain osteoporosis (60%)
epidemiology of ankylosing spondylitis?
M>F (6:1); 18-25
Ank Spond associated features?
AAAAAA anterior uveitis apical lung fibrosis aortic regurgitation AV node block achilles tenoditis amyloidosis
Ankylosing spondylitis investigations?
Graded by New York Criteria Schobers test (<5cm increase = positive) 1st: XR (late changes) 2nd: MRI (if XR normal as more sensitive) HLA-B27 testing ESR +/- CRP
what is seen on XR/MRI of ankylosing spondylitis?
sacroilitis > vertebrae (corner erosions, syndesmophytes)
scerlosis
ankylosis (fusion)
bamboo sign (squaring of lumbar vertebrae)
dagger sign (supraspinatus ossification)
what is the management of ankylosing spondylitis?
conservative: exercise/physiotherapy
medical: NSAID, anti-TNFa (etanercept), secukinumab
surgical: hip replacement to decrease pain and increase mobility
what % of those with psoriatic arthritis are affectedly psoriasis?
10-40%
40-60yo
S/S of psoriatic arthritis?
psoriasis nail (posh = pitting, onycholysis, sublingual hyperkeratosis) enthesitis dactylitis arthritis affects DIP
what is oligoarthritis ?
<= 4 joints
what arthritic features are seen in psoriatic arthritis?
asymmetrical hligoarthritis (20-30%) distal arthritis of the DIPJ symmetrical poly arthritis (30-40%, RA but w DIPJ) arthritis mutilans spinal (50%)
what is the investigations for psoriatic arthritis?
XR: pencil in cup deformity
Mx psoriatic arthritis?
NSAIDs methotrexate ciclosporin sulfasalazine anti-TNF
what is reactive arthritis/reiters?
sterile arthritis 1-4w after urethritis or dystentery
what causes urethritis?
chlamydia, ureaplasma (not gonorrhoea)
what causes dysentery?
campylobacter, salmonella, shigella, yersinia
what are the S/S of reactive arthritis?
cant see, pee or climb a tree
- lower limb oligarthritis
- conjunctivitis/uveitis
- urethritis
keratoderma blenorrhagicum (plaques on soles/palms) circinate balanitis (penile ulceration) enthesitis, mouth ulcers
Ix reactive arthritis?
raised ESR
raised crp
stool culture if diarrhoea, urine chlamydia PCR
Mx reactive arthritis?
NSAIDs, PO steroids
relapsing -> sulfasalazine
what is enteropathic arthritis?
arthritis occurs in 15% of those with UC or Crohn’s
S/S of enteropathic arthritis?
asymmetrical lower limb oligoarthritis, sacroilitis
Mx enteropathic arthritis?
treat IBD
nsaids
local steroids
what are some autoimmune CTDs?
SLE Sjogrens Mixed CTD systemic sclerosis myositis relapsing polychondritis Behcets
Which conditions are anti rheumatoid factor +ve?
sjogrens (50%)
feltys (100%)
RhA (70%)
SLE (20%)
Which conditions are ANA +ve?
SLE (99%)
autoimmune hepatitis (75%)
sjogrens (70%)
RhA (30%)
Which conditions are anti dsDNA +ve?
SLE (70%)
Which conditions are anti CCP +ve?
RhA (80%)
Which conditions are anti histone +ve?
drug induced SLE (100%)
which conditions are anti centromere +ve?
CREST (limited sclerosis)
which conditions are ENA +ve?
SLE sjogrens MCTD polymyositis CREST diffuse systemic sclerosis
which conditions are anti-Ro?
SLE, sjogrens (70%), heart block
which conditions are anti-La?
SLE
sjogrens (30%)
which conditions are anti-smith?
SLE (30%)
which conditions are anti-RNP ?
SLE
MCTD
which conditions are anti-jO-1?
polymyositis>dematomyositis
which conditions are anti Mi-2?
dermatomyositis > polymyositis
which conditions are anti SCL70/topoisomerase?
diffuse systemic sclerosis
which conditions are anti RNA pol 1,2,3?
diffuse systemic sclerosis
what are the indications for urate lowering therapy?
the British Society of Rheumatology Guidelines now advocate offering urate-lowering therapy to all patients after their first attack of gout
ULT is particularly recommended if:
>= 2 attacks in 12 months
tophi
renal disease
uric acid renal stones
prophylaxis if on cytotoxics or diuretics
(allopurinol 1st line)
what is atlantoaxial subluxation and how is it screened for?
rare complication of rheumatoid arthritis
Anteroposterior and lateral cervical spine radiographs preoperatively screen for this complication
ensuring the patient goes to surgery in a C-spine collar and the neck is not hyperextended on intubation.
What is hyperparathyroidism a risk factor for?
development of calcium pyrophosphate dihydrate deposition (CPPD) or pseudogout
chonedrocalcinosis
What is Antisynthetase syndrome?
Antisynthetase syndrome is caused by autoantibodies against aminoacyl-tRNA synthetase e.g. anti-Jo1.
It is characterised by
- myositis
- interstitial lung disease
- thickened and cracked skin of the hands (mechanic’s hands)
- Raynaud’s phenomenon
what is temporal arteritis?
Temporal arteritis is large vessel vasculitis which overlaps with polymyalgia rheumatica (PMR) (50%)
what is seen on histology of temporal arteritis?
Histology shows changes that characteristically ‘skips’ certain sections of the affected artery whilst damaging others.
what are the features of temporal arteritis?
typically patient > 60 years old
usually rapid onset (e.g. < 1 month)
headache (found in 85%)
jaw claudication (65%)
visual disturbances:
- amaurosis fugax
- blurring
- double vision
- vision testing is a key investigation in patients with suspected temporal arteritis
- secondary to anterior ischemic optic neuropathy
tender, palpable temporal artery
around 50% have features of PMR: aching, morning stiffness in proximal limb muscles (not weakness)
also lethargy, depression, low-grade fever, anorexia, night sweats
ix temporal arteritis?
raised inflammatory markers
ESR > 50 mm/hr (note ESR < 30 in 10% of patients)
CRP may also be elevated, alp raised, plts raised
USS temporal artery (halo sign, if -ve do biopsy)
temporal artery biopsy
skip lesions may be present
note creatine kinase and EMG normal
Mx temporal arteritis?
- urgent high-dose glucocorticoids should be given as soon as the diagnosis is suspected and before the temporal artery biopsy (40-60mg prednisolone) -> PPI AND alendronate for 2y
- no visual loss: high-dose prednisolone is used
- evolving visual loss: IV methylprednisolone is usually given prior to starting high-dose prednisolone
- urgent ophthalmology review same day, visual damage is often irreversible
other treatments:
bone protection with bisphosphonates is required as long, tapering course of steroids is required
low-dose aspirin is sometimes given to patients as well, although the evidence base supporting this is weak
What are the 3 patterns of disease for systemic sclerosis?
- limited cutaneous systemic sclerosis
- diffuse cutaneous systemic sclerosis
- scleroderma
Which antibodies are present in systemic sclerosis?
ANA positive in 90%
RF positive in 30%
anti-scl-70 antibodies associated with diffuse cutaneous systemic sclerosis
anti-centromere antibodies associated with limited cutaneous systemic sclerosis
Where does limited cutaneous systemic sclerosis affect?
face and distal limbs predominately
where does diffuse cutaneous systemic sclerosis affect?
trunk and proximal limbs predominately
What are the adverse effects of methotrexate?
mucositis myelosuppression pneumonitis pulmonary fibrosis liver fibrosis
what should be monitored whilst on methotrexate?
FBC, U&E and LFTs need to be regularly monitored.
‘FBC and renal and LFTs before starting treatment and repeated weekly until therapy stabilised, thereafter patients should be monitored every 2-3 months’
What should be co-prescribed with methotrexate
folic acid 5mg once weekly should be co-prescribed, taken more than 24 hours after methotrexate dose
What is antiphospholipid syndrome?
Antiphospholipid syndrome is an acquired disorder characterised by a predisposition to both venous and arterial thromboses, recurrent fetal loss and thrombocytopenia
What does antiphospholipid commonly occur secondary to
SLE
also:
other autoimmune disorders
lymphoproliferative disorders
phenothiazines (rare)
features of antiphospholipid?
venous/arterial thrombosis recurrent fetal loss livedo reticularis thrombocytopenia prolonged APTT other features: pre-eclampsia, pulmonary hypertension
Mx antiphospholipid?
primary thromboprophylaxis - low dose aspirin
secondary -
- initial venous thromboembolic events: lifelong warfarin with a target INR of 2-3
- recurrent venous thromboembolic events: lifelong warfarin; if occurred whilst taking warfarin then consider adding low-dose aspirin, increase target INR to 3-4
- arterial thrombosis should be treated with lifelong warfarin with target INR 2-3
which autoantibody is most linked to sjogrens
anti-Ro
What is behcets?
rare and poorly understood inflammatory condition that mostly occurs in Turkish, Mediterranean and Japanese
what are the S/S of behcets?
recurrent oral and or genital ulceration
uveitis
erythema nodosum
VTE
Ix behcets?
skin pathergy test (pinprick - papule formation)
Mx behcets?
immunosuppression
Epidemiology sjogrens?
F>M 9:1 (40-50yo)
Types of sjogrens?
primary: associated with AI (thyroid, AIH, PBC), MALT lymphoma
secondary: secondary to RhA, SLE, systemic sclerosis
S/S sjogrens?
decreased tear production and dry eyes bilateral parotid swelling decreased salivation (xerostomia) vaginal dryness (dyspareunia) systemic: polyarthritis, raynaud's, bibasal pulmonary fibrosis, vasculitis, myositis sensory polyneuropathy
Ix sjogrens?
bloods: abs (anti-ro [70%], anti La [30%], RhF [50%], hypergammaglobulinaemia
special: schirmers test, parotid biopsy
Mx Sjogrens?
artificial tears, saliva replacement
NSAIDS,hydroxychloroquine, immunosuppression
N.B. Abs may cause heart block in baby ->need O&G input
Epidemiology SLE?
0.2% 9:1 F, child bearing age, afro-carribean, asian
S/S SLE?
SOAP BRAIN MD serositis oral ulcers, hair loss arthritis photosensitivity
blood (all counts low) renal (proteinuria, haematuria) ANA (>95%) Immunological (anti-dsDNA, AIHA) neurological (psych, seizures)
malar rash
discoid rash
pulmonary involvement is very common with pleuritic being the most frequent manifestation
What are the causes of drug induced lupus?
Hydralazine PIMP Hydralazine Procainamide Isoniazid Minocycline Phenytoin
S/S of drug induced lupus?
arthralgia, myalgia, recent serositis
Ix drug induced lupus?
anti histone Abs (100%)
Classification of APLS?
1st (70%)
2nd to SLE (30%)
Which antibodies are present in APLS?
anti-cardioliptin
lupus anticoagulant
S/S of APLS?
CLOTS Coagulation (venous and arterial thromboembolism; INCREASED APTT) Livedo reticularis (web like rash) Obstetric complications Thrombocytopenia
Management of APLS?
No prev VTE -> OD low dose aspirin
prev VTE -> OD warfarin
Investigations SLE/APLS?
Antibodies: ANA (99% sensitive, poorly specific) Anti-dsDNA (99% specific, 70% sensitive) Anti-smith (99% specific, 70% sensitive) RF (20% sensitive)
Disease activity monitoring:
Anti-dsDNA titres
C4 reduction (Mod active lupus), C3 reduction (very active lupus)
ESR levels
Management of SLE?
SEVERE FLARE - prednisolone + IV cyclophosphamide
proteinuria -> ACEi
aggressive lupus nephritis -> immunosuppression
Maintenance:
hydroxychloroquine +/- DMARDs (MMF, azathioprine) +/- low dose steroids
severe disease: biologicals (belimumb, rituximab)
sun protection: sun cream, low dose steroids
what is mixed connective tissue disease?
SLE, scleroderma, polymyositis, RhA
Ix: anti-RNP
what is relapsing polychondritis?
an inflammatory disease of the cartilage (tenderness, inflammation and destruction of cartilage)
what are the associations with relapsing polychondritis?
aortic valve disease
polyarthritis
vasculitis
S/S relapsing polychondritis?
floppy ears
saddle nose
larynx (stridor)
Mx relapsing polychondritis?
immunosuppression
Aetiology raynaud’s phenomenon?
peripheral digit ischaemia precipitated by cold or emotion
Classification of raynauds?
primary: idiopathic, Raynaud’s disease
secondary: Raynaud’s syndrome
- rheumatological (systemic sclerosis, SLE, RhA, sjogrens)
- haematological (thrombocytosis, pv)
- drugs (COCP, non cardio selective beta blockers)
- congenital (cervical rib -> thoracic outflow syndrome)
S/S raynauds?
digit pain, triphasic colour change, digital ulceration and gangrene
Mx raynauds?
gloves/avoid cold
CCB (nifedipine)
PDE V inhibitor (sildenafil)
IV prostacyclin
what are the types of systemic sclerosis
- Limited systemic sclerosis (crest = 70%)
2. diffuse systemic sclerosis (30%)
What is CREST?
Calcinosis Raynaud's Oesophageal dysmotility Sclerodactyly Telangiectasia
Skin involvement (beak nose, microstomia, pul HTN) limited to face, hands, feet
what is diffuse systemic sclerosis?
diffuse skin involvement extends past the wrists and up the arms
What are the features of diffuse systemic sclerosis?
organ fibrosis
GI: GOR, aspiration, dysphagia, anal incontinence
lung 80%: fibrosis, pul HTN
cardiac: arrhythmias, conduction defects
renal: acute HTN crisis (most common cause of death)
Ix Systemic sclerosis?
bedside: urine dip, PCR
bloods: FBC, U&Es, abs
limited CREST -> anti-centromere
diffuse -> anti-SCL70, anti RNA polymerase 1,2,3
imaging:
CXR - cardiomegaly, bibasal fibrosis
Hands - calcinosis
Ba swallow - impaired oesophageal motility
HRCT - fibrosis
Echo - fibrosis
ECG - pulmonary HTN, conduction blocks, arrhythmias
Mx systemic sclerosis?
Conservative: exercise, skin lubricants, hand warmers
M: immunosuppression
Renal crisis - intensive BP control (ACEi)
oesophageal - PPI, pro kinetics
PHT: sildenafil, bosentan
what is polymyositis?
striated muscle inflammation
S/S myositis?
progressive symmetrical proximal muscle weakness (associated myalgia and arthralgia)
wasting of shoulder and pelvic girlde
dysphagia, dysphonia, respiratory weakness
malignancy- paraneoplastic phenomenon (lung, pancreas, ovarian, bowel)
what are the features of dermatomyositis?
myositis + skin signs
- periorbital heliotrope rash on eyelids +/- oedema
- Gottron’s papules on knuckles, elbows, knees
- mechanics hands
- macular rash (shawl sign +ve: over back and shoulders)
- nail fold erythema
- retinopathy (haemorrhages and cotton wool spots)
- subcutaneous calcifications
What are the extra articular features of dermatomyositis?
fever arthritis basal pulmonary fibrosis raynaud's phenomenon myocardial involvement: myocarditis, arrhythmias
Ix myositis?
EMG
Muscle enzymes: Raised CK (in 1000s), raised AST, ALT and LDH
Antibodies: anti-Jo1, Anti-Mi2 [DM>PM], Anti-SRP [PM]
Malignancy screen (tumour markers, CXR, mammogram, USS, CT)
Mx myositis?
Screen for malignancy
immunosuppression (steroids, cytotoxic agents: azathioprine, methotrexate)
which criteria are used to classify vasculitides?
Chapel hill criteria
Give some large vessel vasculitis?
GCA/temporal arteritis
Takayasu arteritis
Give some medium vessel vasculitis?
Polyarteritis nodosa
Kawasaki disease
Give some small vessel vasculitis?
p-ANCA: Churg-Strauss (eGPA), microscopic polyangitis
cANCA: Wegener’s granulomatosia (GPA)
ANCA -ve: HSP, good pasture’s, cryoglobulinaemia
epidemiology of PMR?
> 50yo, association to GCA
Which arteries are affected in polymyalgia rheumatica?
temporal
maxillary
ophthalmic
central retinal
what are the S/S of pMR?
pain/stiffness in shoulder, neck and hips (no weakness)
polyarthritis, tenosynovitis, CTS
Ix PMR?
raised ESR, CRP, ALP, normal CK
Mx PMR?
15 mg PO prednisolone
taper down to 5 mg + PPI + alendronate
What is the epidemiology of takayasu arteritis?
Japanese/asian females
20-40yo (granulomatous vasculitis similar to GPA/eGPA)
S/S Takayasu arteritis?
weak/unequal upper limb pulses HTN Large vessel blockage - COMMONLY AORTA (HF, CRF) Intermittent limb claudication AR (20%)
mx takayasu?
corticosteroids
Epidemiology polyarteritis nodosa?
young male adults (not common in UK)
s/s polyarteritis nodosa?
systemic symptoms skin (rash) GIT (melaena, abdo pain) renal (HTN) liver dysfunction (HBV)
Ix polyarteritis nodosa?
HBV serology, rosary bead sign on renal angiogram
Mx polyarteritis nodosa?
prednisolone and cyclophosphamide
Epidemiology Kawasaki?
6m-5yo, 8/100000, Japanese and Black Caribbean ethnicity
S/S kawasaki?
CRASH and BURN Conjunctivitis Rash Adenopathy Strawberry tongue Hands/feet swollen Fever >5 days
Ix Kawasaki disease?
clinical (echocardiogram done in OPD)
Mx Kawasaki?
IVIG and high dose aspirin
What are some ANCA +ve conditions?
GPA/wegeners
eGPA (Churg-Strauss)
Microscopic polyangitis
what are some ANCA negative conditions?
HSP
Goodpastures
Cryoglobulinaemia
what are the S/S of wegeners/GPA?
URT - rhinitis, epistaxis, saddle nose
LRT - haemoptysis, cough
Renal - rapidly progressive glomerulonephritis (RPGN), nephritic syndrome
what are the Ix for GPA?
cANCA (pr3)
dipstick (p+, h+)
CXR (nodules)
what is eGPA/Churg Strauss?
rare disease occurs in atopic individuals
What are the S/S of eGPA?
eosinophilia
asthma (late onset)
vasculitis (incl. RPGN)
What are the Ix for eGPA?
pANCA (MPO n.b. also +ve in UC/TB)
What are the S/S of microscopic polyangitis?
RPGN
palpable purpura
haemoptysis
Ix for microscopic polyangitis?
pANCA (MPO)
What are the features of HSP?
Most common childhood vasculitis
often preceded by URTI (2-3 days)
S/S HSP?
purpuric rash (urticarial, maculopapular, spares trunk) arthralgia (and periarticular oedema, large joints) Abdominal pain (haematemesis, melena, intussusception) Glomerulonephritis (micro/macroscopic haematuria, nephrotic syndrome (rare))
Ix hsp?
Urinalysis (RBCs, protein, casts)
FBC, clotting screen, U&E,
Mx HSP?
follow up (weekly for 1m, 2 weekly for 2m, 3m, 6m, 12m) BP measurements Urine dip (haematuria) Most cases will resolve spontaneously within 4 weeks
what is good pastures?
antiGBM
What are the S/S of good pastures?
RPGN
haemoptysis
what are the investigations for good pastures?
Anti-gbm antibody
CXR (bilateral lower zone infiltrates)
Mx goodpastures?
immunosuppression + plasmapheresis
What is simple cryoglobulinaemia?
Monoclonal IgM -hyperviscosity: visual disturbances bleeding from mucous membranes thrombosis headaches and seizures
What is mixed cryoglobulinaemia?
Polyclonal IgM - immune complex mediated disease GN palpable purpura arthralgia peripheral neuropathy
aetiology of simple cryoglobulinaemia?
20%
secondary to: myeloma, CLL, Waldenstroms macroglobulinaemia
aetiology of mixed cryoglobulinaemia?
80% secondary to SLE sjogrens HCV mycoplasma
epidemiology of fibromyalgia?
10% of new rheumatology referrals
F>M (10:1)
RFs fibromyalgia?
neurosis (depression, anxiety, stress) work dissatisfaction overprotective family/lack of support middle age low income divorced low educational status
Associations fibromyalgia?
chronic fatigue syndrome
IBS
chronic headache syndrome
S/S fibromyalgia?
chronic widespread musculoskeletal pain and tenderness fatigue sleep disturbance morning stiffness poor concentration low mood
Ix fibromyalgia
all normal
Mx fibromyalgia?
educate
CBT
graded exercise programmes
amitriptyline, pregabalin, venlafaxine
Management of chronic pain?
all diabetics get peripheral neuropathy
Amitriptyline Duloxetine Gabapentin Pregabalin
1st line for neuropathic pain?
amitriptyline, pregabalin
1st line for diabetic neuropathy?
duloxetine
tramadol as rescue therapy
1st line for trigeminal neuralgia?
carbamazepine
Epidemiology still’s disease?
15-25yo, 35-45 yo
S/S stills disease?
Arthralgia
Salmon pink rash (maculopapular)
Pyrexia (rises in late evening in daily pattern alongside arthralgia)
Investigations Stills disease?
RhF, ANA -ve
Raised ferritin
Yamaguchi criteria (sensitivity of 93.5%)
Management of Still’s disease?
1st line: NSAIDs (for fever, joint pain, serositis)
2nd line: after 1 week of NSAIDs + steroids
3rd line: methotrexate, IL-1, anti-TNF
Epidemiology of pagets?
Paget’s disease is common (UK prevalence 5%) but symptomatic in only 1 in 20 patients.
which bones are affected in Paget’s?
The skull, spine/pelvis, and long bones of the lower extremities
Paget’s RF?
increasing age
male sex
northern latitude
family history
Clinical features of Paget’s?
only 5% of patients are symptomatic the stereotypical presentation is an older male with bone pain and an isolated raised ALP bone pain (e.g. pelvis, lumbar spine, femur) classical, untreated features: bowing of tibia, bossing of skull
Ix pagets?
bloods:
raised alkaline phosphatase (ALP)
calcium and phosphate are typically normal. Hypercalcaemia may occasionally occur with prolonged immobilisation
other markers of bone turnover include: procollagen type I N-terminal propeptide (PINP) serum C-telopeptide (CTx) urinary N-telopeptide (NTx) urinary hydroxyproline
x-rays:
osteolysis in early disease → mixed lytic/sclerotic lesions later
skull x-ray: thickened vault, osteoporosis circumscripta
bone scintigraphy:
increased uptake is seen focally at the sites of active bone lesions
Management of paget’s?
indications for treatment include: bone pain skull or long bone deformity fracture periarticular Paget's
bisphosphonate (either oral risedronate or IV zoledronate)
calcitonin is less commonly used now
Complications of Paget’s?
deafness (cranial nerve entrapment) bone sarcoma (1% if affected for > 10 years) fractures skull thickening high-output cardiac failure
What is Marfan’s and what is it caused by?
Marfan’s syndrome is an autosomal dominant connective tissue disorder
defect in the FBN1 gene on chromosome 15 that codes for the protein fibrillin-1
What are the features of Marfan’s?
tall stature with arm span to height ratio > 1.05
high-arched palate
arachnodactyly
pectus excavatum
pes planus
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)
leading causes of death in marfan’s?
aortic dissection
Mx Marfan’s?
regular echocardiography monitoring and beta-blocker/ACE-inhibitor therapy
Blood results in osteogenesis imperfect?
Adjusted calcium, PTH, ALP and PO4 results are usually NORMAL in osteogenesis imperfecta
Features of osteogenesis imperfecta?
presents in childhood fractures following minor trauma blue sclera deafness secondary to otosclerosis dental imperfections are common
What is the management for secondary prevention for osteoporotic fractures in post menopausal women?
start a bisphosphonate before waiting for a DEXA
- vitamin D and calcium supplementation should be offered to all women unless the clinician is confident they have adequate calcium intake and are vitamin D replete
- alendronate is first-line
- around 25% of patients cannot tolerate alendronate, usually due to upper gastrointestinal problems. These patients should be offered risedronate or etidronate
- strontium ranelate and raloxifene are recommended if patients cannot tolerate bisphosphonates
Interpretation of DEXA?
T = no of SDs away from youthful average Z = no of SDs away from age matched average
T>-1 = normal
T-1 - -2.5 = osteopenia
T
what is ANA antibody seen in?
(SSSA)
systemic lupus erythematous (SLE)
systemic sclerosis
autoimmune hepatitis
Sjogren’s syndrome
What is AMA antibody raised in?
primary biliary cholangitis
autoimmune hepatitis
idiopathic cirrhosis
What is anti-GBM seen in?
Good pastures
What is pANCA seen in?
eosinophilic granulomatosis with polyangiitis
microscopic polyangiitis
Goodpasture’s disease.
Features of rheumatoid arthritis?
LESS Loss of joint space Erosions Subchondral Cysts Subchondral sclerosis
ie erosions differentiate from rheumatoid
What is HLA-B27 associated with?
Reactive arthritis
Ankylosing spondylitis
seronegative spondyloarthropathies
What HLA is behcets associated with?
HLA-B51
What is HLA-DQ2 associated with?
coeliac
what is HLA-DR3 associated with?
Addison's disease systemic lupus erythematosus type 1 diabetes mellitus Grave's disease myasthenia gravis dermatitis herpetiformis Sjogren's syndrome primary biliary cirrhosis
What is HLA-DR1 associated with?
T1DM
RA