Rheumatology Flashcards
What are the common associations with Ankylosing Spondylosis
A’s
Ant Uveitis
Aortic Regurgitation
Apical Fibrosis
AV nodal block
Achilles Tendonitis
Amyloids
Quada Equina
What HLA is associated with Ankylosing Spondylosis
HLA-B27
What are the Ix for diagnosing Ankylosing Spondylosis
Shobers Test
Xray;
Bamboo spine
Syndesmophytes
Sacroilities
Squaring of vertebral
CXR: Apical Fibrosis
Spirometry - restrictive pattern
Mx:
Exercise, swimming
NSAID
Physio
Anti-TNF ( Etanercept, Adalimumab)
Note:
Antirheumatoid frugs such as Sulfazalsine only used if peripheral joint inv. present
What is the Ix and Mx of Antiphospholipid Syndrome
Ix;
Prolonged APTT
Thrombocytopenia
Test for;
Anti-Cardiolipin
Anti-Beta2 glycoprotein
Lupus anticoagulant
Mx:
Primary VTE prophylaxis - Asprin
LMWH + Aspirin if high risk
( pregnant )
Secondary ;
Life long Warfarin ; INR 2-3
If clots , when on warfarin;
Add Aspirin + Warfarin ( INR 3-4)
If arterial thrombus ;
Warfarin ( INR 2-3)
INR pregnancy
LMWH + Aspirin
What are the risk factors for Avascular necrosis of Hip
Long term steroids
Chemo
Alcohol
Trauma
MX:
Hip replacement
What skin cancer is Azathioprine usually associated with
Non Melanoma Skin cancers
What HLA is associated with Behcets
HLA - B51
What is the triad of behcets
Oral ulcers
Genital ulcers
Anterior Uveitis
Note:
Erythema nodosum is also seen
High risk of clots and thrombophelibitis
Neuro inv.
GI upset
What is pathergy test and where is it used
Puncture site with needle becomes inflamed and pustule formed
Behcets +ve
What are the common disorders and its lab values
Proximal muscle weakness
Rash in shoulders and back ( Shawl sign)
rash around eyes ( Heliotropic rash)
Thickened knuckles ( Guttorns)
Whats Is your Dx
Dermatomyositis
What are the common association complication of dermatomysotis
Cancer
( Breast, Lung, Ovaries)
What blood Ix for Dermatomyositis
(around 80%) are ANA positive
around 30% of patients have antibodies to
-aminoacyl-tRNA synthetases (anti-synthetase antibodies),
including:
-antibodies against histidine-tRNA ligase (also called Jo-1)
-antibodies to signal recognition particle (SRP)
-anti-Mi-2 antibodies ( Most important)
What is the Mx of Dermatomyositis
Steroids
What is ;
Diffuse idiopathic skeletal hyperostosis
relatively common finding of ossification at sites of tendinous and ligamentous insertion of the spine. It tends to be seen in elderly patients.
What is the Ix for Discitis
MRI spine
CT guided biopsy
Blood cultures
Most common; Staph Aureus
Pt. should also be Ix for IE ( TOE/TTE)
MX:
Abx for 8 week
What is the Mx of Discoid Lupus
Topical Steroids
Oral Hydroxychloroquine
Avoid Sun exposure
Where do you angiod retinal streaks
Ehler Danlos
What are the cardiac associations of Ehler Danlos ( autosomal dominant)
Aortic Regurg
MVP
Aortic dissection
Note:
Easy bruising
Fragile skin
Hyper-elastic - recurrent dislocations
SAH
What group is commonly afftected by FMF
It is more common in people of Turkish, Armenian and Arabic descent.
Note:
Features - attacks typically last 1-3 days
pyrexia
abdominal pain (due to peritonitis)
pleurisy
pericarditis
arthritis
erysipeloid rash on lower limbs
Management
colchicine may help
if uric acid level < 360 umol/L during a flare and gout is strongly suspected, What do you do
repeat the uric acid level measurement at least 2 weeks after the flare has settled
Note;
If Uric Acid >/= 360 umol/L - It confirms gout
What is the synovial fluid analysis in Gout
needle shaped negatively birefringent monosodium urate crystals under polarised light
What is the Mx of gout
In acute flare; (uric acid > 0.45 mmol/l)
> NSAID ( use PPI cover)
Colchicine
( used with caution in renal impairment: the BNF advises to reduce the dose if eGFR is 10-50 ml/min and to avoid if eGFR < 10 ml/min )
oral steroids may be considered if NSAIDs and colchicine are contraindicated.
a dose of prednisolone 15mg/day is usually used
another option is intra-articular steroid injection
2 weeks after;
Start Urate lower therapy;
Alopurinol
What is the 2nd line Urate lowering therapy if Allopurinol Contrainicated/ Not tolerated
Febuxostat
Where do you see Juvenile Idiopathic Arthritis
arthritis occurring in someone who is less than 16 years old that lasts for more than three months.
Note;
associated with +ve ANA
and anterior uveitis
Langerhans cell histiocytosis
Features
>bone pain, typically in the skull or proximal femur
>cutaneous nodules
>pituitary involvement: leads to diabetes insipidus due to pituitary stalk involvement
>pulmonary involvement: More >common in adults, presenting with dyspnoea, cough, and chest pain
>recurrent otitis media/mastoiditis
tennis racket-shaped Birbeck granules on electromicroscopy
Pain and tenderness localised to the lateral epicondyle
Pain worse on wrist extension against resistance with the elbow extended or supination of the forearm with the elbow extended.
What is your Dx
Lateral Epicondylitis
Unilateral or bilateral leg pain (with or without back pain), numbness, and weakness which is worse on walking.
Resolves when sits down. Pain may be described as ‘aching’, ‘crawling’.
What is your Dx
Spinal Stenosis
positional element to the pain. Sitting is better than standing and patients may find it easier to walk uphill rather than downhill.
Lumbar Spinal Stenosis
Ix: MRI
Mx: Laminectomy
What gene is affected in Marfans
FBN1
muscle pain and stiffness following exercise
muscle cramps
second wind phenomenon
McArdle Syndrome
( autosomal recessive)
What are the Ix finding of McArdle Syndrome
rhabdomyolysis & myoglobinuria
low lactate levels during exercise
How long should women avoid pregnancy after stropping methotrexate
at least 6 months after treatment has stopped
What will you monitor when someone is on Methotrxate
FBC, U&E and LFTs
What Abx should you avoid when someone is on MTX
Trimethoprim and Co-Trimoxazole
What are the common causes of myopathies
inflammatory: polymyositis
inherited: Duchenne/Becker muscular dystrophy, myotonic dystrophy
endocrine: Cushing’s, thyrotoxicosis
alcohol
Note: Key Features ;
Features
symmetrical muscle weakness (proximal > distal)
common problems are rising from chair or getting out of bath
“sensation normal, reflexes normal, no fasciculation”
What are some examples of NSAID
Examples of NSAIDs include
ibuprofen
diclofenac
naproxen
aspirin
What is the mx of OA
> oral analgesia
intra-articular injections: provide short-term benefit
total hip replacement remains the definitive treatment
What are X-ray changes of osteoarthritis
(LOSS)
Loss of joint space
Osteophytes forming at joint margins
Subchondral sclerosis
Subchondral cysts
presents in childhood
fractures following minor trauma
blue sclera
deafness secondary to otosclerosis
dental imperfections are common
Osteogenesis Imperfecta
( Autosomal Dominant)
abnormality in type 1 collagen
Ix;
Investigations
adjusted calcium, phosphate, parathyroid hormone and ALP results are usually normal in osteogenesis imperfecta
What are the key features of osteomalacia
Low Ca due to Low Vit D
Features
bone pain
bone/muscle tenderness
fractures: especially femoral neck
proximal myopathy: may lead to a waddling gait
Investigation
bloods
low vitamin D levels
low calcium, phosphate (in around 30%)
raised alkaline phosphatase (in 95-100% of patients)
Treatment
vitamin D supplmentation
a loading dose is often needed initially
calcium supplementation if dietary calcium is inadequate
x-ray
translucent bands (Looser’s zones or pseudofractures)
What is your Dx
Osteomalacia
What are the common causes of osteomyelitis
Microbiology
Staph. aureus is the most common cause
except in patients with sickle-cell anaemia where “Salmonella species” predominate
Note:
Non diabetic + Puncture wound - Pseudomonas
Diabetic + Puncture wound - Staph Aureus
Implant related infection - Coag -ve Staph
Mx:
Management
flucloxacillin for 6 weeks
What is the mx of Osteomyelitis if Pt. is Pen allergic
clindamycin if penicillin-allergic
Pt. with sickle cell has osteomyelitis
What is the common organism
sickle-cell anaemia where “Salmonella species” predominate
Bone pain;
But Ca; Po, APT, PTH all normal
Osteopetrosis (marble bone disease)
Mx:
stem cell transplant
interferon-gamma
How do you assess ;
Osteoporosis: Assessing patients following a fragility fracture
> /=75 and has a fragility fracture
( Directly start Tx ; no need for DEXA)
if <75;
DEXA
When do you offer DEXA scan even prior to assessing fragility scores
> 50 years of age with a history of fragility fracture
< 40 years of age who have a major risk factor for fragility fracture - these patients should be referred to a specialist depending on the T-score
before starting treatments that may have a rapid adverse effect on bone density (for example, sex hormone deprivation for treatment for breast or prostate cancer)
What are common fragility fractures sites
Vertebrae (most common)
Hip (proximal femur)
Distal radius (Colles’ fracture)
Proximal humerus
What is the threshold to start TX based on DEXA scan
T-score of - 2.5 SD or below
Bone pain
Isolated ALP rise
Paget disease
Features;
bowing of tibia, bossing of skull
Bone scintigraphy
increased uptake is seen focally at the sites of active bone lesions
Mx: Bisophosphonates
Calcitonin if above Is not tolerated
Raised
procollagen type I N-terminal propeptide (PINP)
serum C-telopeptide (CTx)
urinary N-telopeptide (NTx)
urinary hydroxyproline
Where do you see it
Pagets disease
What are the key features of PAN
vasculitis affecting medium-sized arteries with necrotizing inflammation leading to aneurysm formation.
Features
fever, malaise, arthralgia
weight loss
hypertension
mononeuritis multiplex, sensorimotor polyneuropathy
testicular pain
livedo reticularis
haematuria, renal failure
p-ANCA +ve
hepatitis B +ve
Ix: Angiogram showing microanuerysms
What are the key features of PMR
Features
typically patient > 60 years old
rapid onset (e.g. < 1 month)
aching, morning stiffness in proximal limb muscles
NO weakness
also mild polyarthralgia, lethargy, depression, low-grade fever, anorexia, night sweats
Investigations
raised inflammatory markers e.g.
ESR > 40 mm/hr
note CK and EMG normal
Treatment
prednisolone e.g. 15mg/od
patients typically respond dramatically to steroids,
failure to do so should prompt consideration of an alternative diagnosis
Note: PMR can be associated with GCA
What are the key features of polymyositis
Features
proximal muscle weakness +/- tenderness
Raynaud’s
respiratory muscle weakness
interstitial lung disease
e.g. fibrosing alveolitis or organising pneumonia
seen in around 20% of patients and indicates a poor prognosis
dysphagia, dysphonia
Investigations
elevated creatine kinase
other muscle enzymes (lactate dehydrogenase (LDH), aldolase, AST and ALT) are also elevated in 85-95% of patients
EMG
muscle biopsy
anti-synthetase antibodies
anti-Jo-1 antibodies are seen in pattern of disease associated with lung involvement, Raynaud’s and fever
Management
high-dose corticosteroids tapered as symptoms improve
azathioprine may be used as a steroid-sparing agent
What are the key differences between PMR and Polymyositis
PMR:
Proximal Muscle stiffness/Pain
Normal CK
Raised ESR
Rapid onset <1month
Can be associated with GCA
Mx: Low dose steroids 15mg/kg
Polymyositis;
Proximal Muscle Weakness +/- Pain
Raised CK +++
Anti- Jo Abs
Ix: Muscle Biopsy, EMG
Mx: High Dose steroids
What are the common associations of Psuedogout
haemochromatosis
hyperparathyroidism
low magnesium, low phosphate
acromegaly, Wilson’s disease
What is the synovial fluid analysis of Pseudo gout
weakly-positively birefringent rhomboid-shaped crystals
Xray;
Chondrocalcinosis
Mx;
aspiration of joint fluid, to exclude septic arthritis
NSAIDs or intra-articular, intra-muscular or oral steroids as for gout
What are the key features of psoriatic arthritis
Symmetrical
DIP
Skin Rash
Nail ; Pitting and onycholysis
Arthritis Mutilans
What is the X-ray features of psoriatic arthritis
pencil-in-cup’ appearance
What is the Mx of Renaud’s Phenomenon
first-line: calcium channel blockers e.g. nifedipine
IV prostacyclin (epoprostenol) infusions: effects may last several weeks/months
What type of cryoglobinemia is associated with Reynauds
Type I
What is the key triad of Reactive arthritis
Ant. Uveitis
Joint pain ( assymetircal, lower limbs)
Urethritis
Post infection;
Salmonella, Chlamydia
Dry Tap - -ve culture of synovial fluid
What skin rash is associated with Reactive Arthritis
keratoderma blenorrhagica
(waxy yellow/brown papules on palms and soles)
What is the Mx of Relapsing Polychondritis
Induce remission: steroids
Maintenance: azathioprine, methotrexate, cyclosporin, cyclophosphamide
Ears: auricular chondritis, hearing loss, vertigo
Nasal: nasal chondritis → saddle-nose deformity
Destruction of cartilage
What is your Dx
Relapsing Polychondritis
What are the extra-articular manifestations of Sarcoid
Respiratory: pulmonary fibrosis, pleural effusion, pulmonary nodules, bronchiolitis obliterans, methotrexate pneumonitis, pleurisy
ocular: keratoconjunctivitis sicca (most common), episcleritis, scleritis, corneal ulceration, keratitis, steroid-induced cataracts, chloroquine retinopathy
osteoporosis
ischaemic heart disease: RA carries a similar risk to type 2 diabetes mellitus
increased risk of infections
depression
Less common
Felty’s syndrome (RA + splenomegaly + low white cell count)
amyloidosis
What is the Dx marker of RA
Anti-CCP
What is the Mx of RA
For acute flare;
Steroids
DMARDS; +/- bridging steroids
1st line - Methotextrate;
If Fails;
Try another DMARD’s
sulfasalazine
leflunomide
hydroxychloroquine
If 2 DMARD’s Fail;
Then trial Anti-TNF
etanercept
infliximab
adalimumab
Rituximab
anti-CD20 monoclonal antibody,
What are the common causes of Septic Arthritis
Staph Aureus - most common
In young adults who are sexually active- Nesirrhoea Gonorrhea
Mx:
Fluclox/ Clindamycin ( 4-6 weeks)
Can switch to oral Abx after 2 weeks of IV
What are the Ix of septic arthritis
Synovial fluid sampling
( Before Abx Tx)
> shows a leucocytosis with neutrophil predominance
gram staining is negative in around 30-50% of cases
fluid culture is positive in patients with non-gonococcal septic arthritis
blood cultures: the most common cause of septic arthritis is hematogenous spread
joint imaging
Painful arc of abduction between 60 and 120 degrees
Tenderness over anterior acromion
Supraspinatus tendonitis
Where do you see
+ve Anti Ro and Anti-La
Sjogrens
Note:
RF can be +ve as well
Ix:
Schimers test
Parotid gland biopsy (focal lymphocytic infiltration)
hypergammaglobulinaemia, low C4
Mx;
Management
artificial saliva and tears
pilocarpine may be helpful to stimulate saliva production
Arthralgia
Salmon Pink Rash
Fever ( which spikes in afternoon to evenings) which accompanies joint pain
What is your Dx
Adult onset Stills Disease
Mx;
> NSAIDs
should be used first-line to manage fever, joint pain and serositis
they should be trialled for at least a week before steroids are added.
> steroids
may control symptoms but won’t improve prognosis
> If symptoms persist, the use of methotrexate, IL-1 or anti-TNF therapy can be considered
What are the key Ix of SLE
Antibodies
99% are ANA positive
this high sensitivity makes it a useful rule out test, but it has low specificity
20% are rheumatoid factor positive
anti-dsDNA: highly specific (> 99%), but less sensitive (70%)
anti-Smith: highly specific (> 99%), sensitivity (30%)
also: anti-U1 RNP, SS-A (anti-Ro) and SS-B (anti-La)
Note:
anti-dsDNA titres can be used for disease monitoring
Note: Complement levels are low during active disease
What is the Mx of SLE
NSAIDs
sun-block
Hydroxychloroquine
the treatment of choice for SLE
anti-scl-70 antibodies associated with
diffuse cutaneous systemic sclerosis
associated with a higher risk of severe interstitial lung disease
anti-centromere antibodies associated with
limited cutaneous systemic sclerosis/CREST
( Pulm HTN)
GCA with vision problems
IV methyl pred x 3 days followed by oral pred (1mg/kg ) ~ 100-160mg/day
GCA without vision problems
High dose oral pred ( 60mg/day)
What are the key Ix of GCA
Temporal artery biopsy - Skip lesions seen
Raised inf markers and ESR
Normal CK and EMG
What is Finkelstein test used in
DeQuervains Tenosynovitis
( Class thumb in palm within fingers and ulnar deviate -> Causes pain)
What are the key features of Takayasu’s arteritis
Large/medium vessel arteritis
Affects Aorta and its branches
Fever
Malaise
Weight loss
TIA/ Stroke
MI/ Claudication
Ix;
Raised ESR, CRP , CT-A
Mx:
Steroids with addition of MTX/ Azathioprine
Pigmentation of urine, eye, connective tissue
Cartilage pigmentation ( Ochronosis)
Urine after standing becomes dark
Bone pains
What is your Dx
Alkaptonuria
What Abs are +ve in Drug Induced Lupus
Anti-Histone Abs
If DEXA shows <-4.5 or who have 2 or more fragility fractures
what is the 1st line mx
Teriparatide
What are the eye Ix features of Stills disease
Leucocytosis
Thrombocytosis
Increased Ferritin
Increased ESR
Increased CRP
All Auto-Abs will be NEGATIVE
Which Abs will likely be +ve in Scleroderma Rena Crisis with skin inv
Anti RNA Polymerase III Abs
What are the key features of
Pseudo-Hypoparathyroidism
Shortening of 5th Metacarpal
Hypocalcaemia
What is +ve Shimmers test
<5mm wetting In 5mins
What is the DEXA cutoff for starting Osteoporosis Mx in someone taking long term steroids
<-1.5
Purpuric rash , Renauds, Polyarthralgia
+ve RF
+ve ANA
Low complement ( c4)
Normal C3
What is your Dx
Cryoglobunemia
Where do you see +ve Anti RNP abs
Mixed Connective Disease
What is the Mx of Pulm HTN in SS
Iloprost infusion +/- Sildneafil
