Rheumatology Flashcards
pANCA associations
Vasculitis
UC and Crohns (more so UC)
PSC
Anti GBM disease
Features of AS
HLAB27 spondyloarthropathy (males, 20-30)
Lower back pain and stiffness of insidious onset
Stiffness worse in morning and improves with exercise
Pain at night- improves on getting up
Exam;
Reduced lateral flexion
Reduced foreword flexion (Schobers)
Reduced chest expansion
Associations of AS
The A’s
Apical fibrosis
Anterior uveitis
Aortic regurgitation
Achilles tendinitis
AV node block
Amyloidosis
Arthritis (peripheral)
And cauda equina
Investigations for AS
Spinal exam, observations, spirometry (restrictive disease)
Bloods- ESR, CRP
Imaging- spinal (pelvic) XRay and MRI
X ray changes for AS
Early- normal
Later
Sacroilitis (subchondral erosions), loss of joint space
Squaring of lumbar vertebrae
Bamboo spine
Syndesmophytes (from front and side, lumbar vertebrae are connected by bony spurs)
Dagger sign (midline opacification of vertebral column)
Chest X-ray- apical fibrosis
Management of AS
Exercise and physio
NSAID
Anti TNF (etanercept) if high disease activity/ peripheral arthritis
Azathioprine
Adverse effects
Bone marrow depression
Nausea and vomiting
Pancreatitis
Non melanoma skin cancer
Adverse reaction with allopurinol
Safe in pregnancy
Features of Bechets syndrome
20-40 year old men
Oral ulcers, genital ulcers, anterior uveitis
Thrombophlebitis and DVT
Arthritis
Neuro involvement
Erythema nodosum
GI involvement
Pathergy test- needle prick causes inflamed skin pustule
Features of chronic fatigue syndrome
4 months of disabling fatigue affecting mental and physical function more than 50% of the time in the absence of other disease
Fatigue
Sleep disturbance eg. Insomnia
Myalgia and arthralgia
Headaches
Cognitive impairment
General malaise
Investigations CFS
Bedside- obs and exam and BM urinalysis (bence jones- myeloma)
Bloods- FBC UE LFT glucose HBA1c TFT ESR CRP Ca CK ferritin coeliac haemanitics
Management of CFS
CBT
Graded exercise programme
Pain management clinic if pain is a problem
Low dose amitriptyline (if poor sleep)
Denosumab
RANKL inhibitor
60mg S/C every 6 months for osteoporosis
120mg given every 4 weeks for prevention of skeletal related events (pathological fractures) in adults with bony metastases
Causes of drug induced lupus
Procainamide (anti arrhythmic)
Hydralazine (vasodilator- HTN)
Isoniazid
Phenytoin
Features of drug induced lupus
Arthralgia
Myalgia
Skin (malar rash)
Pulmonary involvement eg. Pleurisy
ANA positive, dsDNA negative
Anti histone antibodies
Ehler Danlos syndrome
Autosomal dominant
Collagen (type III)
Elastic fragile skin
Joint hyper mobility (dislocation)
Easy bruising
Aortic regurgitation mitral valve prolapse aortic dissection
SAH
Retinal streaks
Fibromyalgia
Chronic pain all over (tender spots)
Lethargy
Cognitive impairment eg. Fog
Sleep disturbance (unrefreshed), headaches, dizziness
Management of fibromyalgia
Exercise- best evidence
CBT
Medication- pregabalin, amitriptyline
Features of gout
Episodes lasting several days where gout flares, symptom free between episodes; significant pain, swelling, erythema, tophi (painless hard nodules)
1st MTP, ankle, wrist, knee
Can get permanent joint destruction
Radiological features of gout
Joint effusion
Well defined punched out erosions with sclerotic margins in a juxta articular distribution (and overhanging edges)
Soft tissue tophi may be seen
NB- no loss of joint space
Investigations for gout
Bedside- MSK exam, observations
Bloods- uric acid (can be low in acute attack), ESR, CRP, FBC
I&S- X ray, joint aspirate (bifringent light etc.)- would also rule out SA
NB- measure urate 2 weeks after attack
Management of gout
Lifestyle- reduce alcohol, lose weight, avoid food high in purines eg. Liver, kidneys, seafood, oily fish, yeast products eg. Marmite
Acute- NSAID or colchicine (diarrhoea). Steroids if these are contraindicated. Keep taking allopurinol.
Urate lowering therapy (ULT)- 2-4 weeks after first attack of gout (allopurinol or febuxostat). May need colchicine of NSAID cover whilst starting (<300), as allopurinol can trigger gout initially
NB- stop thiazides, losartan is better if HTN, increase vitamin C
Causes of hyperuricaemia
Diuretics (thiazides)
CKD
Lead toxicity
Cytotoxic drugs/chemotherapy
Myeloproliferative disorder
Gout
HLA A3
Haemochromatosis
HLA B51
Bechets
HLA B27
AS
Reactive arthritis
Anterior uveitis
Psoriatic arthritis
Enteric arthritis
HLA DQ2/8
Coeliac
HLA DR3
Dermatitis herpetiformis
Sjögren’s syndrome
PBC
HLA DR4
T1DM
RA
Hydroxychloroquine
May cause bulls eyes retinopathy (can cause severe and permanent visual loss- baseline ophthalmological examination and annual screening reccomended)
Safe in pregnancy
Langerhans cell histiocytosis
Childhood bony lesions
Bone pain (skull and proximal femur)
Cutaneous nodules
Recurrent otitis media and mastoiditis
Tennis racket shaped Birbeck granules on electron microscopy
Lateral epicondylitis
AKA tennis elbow (caused by painting, tennis etc.)
Pain and tenderness localised to lateral epicondyle
Pain worse on wrist extension against resistance or supination, with elbow extended
Analgesia
Steroid injections
Physiotherapy
Features of Marfan’s syndrome
Defects in fibrillin 1
Tall stature- arm span to height ratio >1.05
Arachnodactylyl (wrap fingers around joints)
hyperextendable joints
High arched palate
Pectus excavatum
Pes planus (flat feet)
Scoliosis
Dilation of aortic sinuses
Dural ectasia (ballooning of dural sac at lumbosacral level)
Complications;
Aortic aneurysm, aortic dissection, regurgitation, mitral valve prolapse
Pneumothoraces
Upward lens dislocation
Management of Marfan’s syndrome
Beta blockers and ACE-I to control HTN and heart pathology
Physio for reducing hyper mobility issues
Genetic counselling- screen family members
Regular echocardiograms (1 year) and ophthalmology reviews, 5 yearly MR/CT aorta (complications)
Adverse effects of methotrexate
Hair loss
Mucositis
Myelosupression
Pnueomonitis
Pulmonary fibrosis
Liver fibrosis
Avoid pregnancy, and 6 months afterwards (men should use contraception for 6 months after finishing it too)
Methotrexate interactions
Avoid trimethoprim or co trimoxazole
High dose aspirin may increase risk of toxicity
Causes of myopathy
Proximal muscle weakness
Inflammatory eg. Polymyositis
Inherited- DMD
Endocrine- cushings, thyrotoxicosis
Alcohol
Management of OA
Supportive- weight loss, muscle strengthening exercises, physiotherapy, TENS device
Medical- paracetamol and topical NSAID eg. Ibuprofen gel, then oral NSAID (PPI) and opioids and intra articular steroids
Surgical- joint replacement when other measures fail
NB- topical NSAIDs only for hand/knee OA
Osteogenesis imperfecta
Autosomal dominant
Presents in childhood
Fractures following minor trauma
Blue sclera
Osteosclerosis causing deafness
Dental imperfections
Calcium, phosphate, PTH, ALP normal
Causes of osteomalacia
Vitamin D deficiency (malabsorption, lack of sunlight, diet)
CKD
Anticonvulsant
Features of osteomalacia
Bone pain and tenderness
Fractures
Proximal myopathy (waddling gait)
Investigations and management of osteomalacia
Bloods- low vitamin D, low calcium and phosphate, raised ALP
Imaging- translucent bands
Vitamin D supplementation, calcium supplementation if dietary intake is inadequate
Risks for Pagets disease of bone
Increased age
Male sex
FH
Features of Paget’s disease of bone
Skull, spine, pelvis, long bones of lower extremities are most commonly affected
Bone pain
Pathological fractures
Isolated raised ALP (calcium and phosphate normal)
Bony deformities eg. kyphosis, OA, enlarged maxilla, frontal bossing of skull, bowed femur and tibia
Investigation for Paget’s disease of bone
Bedside- obs and exam
Bloods- FBC UE CRP LFT bone profile
Imaging- X ray, isotope bone scan
Management of Paget’s disease of bone
Conservative- lifestyle measures, physiotherapy
Medical- Bisphosphonate (if pain, skull or long bone deformity, fracture, periarticular Paget’s), analgesia (NSAID), ensure adequate vitamin D and calcium
Surgery- correction of deformities
Polyarteritis nodosa association
Hepatitis B
Differentials for a polyarthritis
RA
OA
Psoriatic arthritis
SLE
AS
TB
Gout
Virus eg. HIV
Features of poly myalgia rheumatica
60+
Rapid onset (less than 1 month)
Aching, morning stiffness in proximal limb muscles (pelvic and shoulder girdle)
Worse with movement
Weakness not a symptom
Mild poly arthralgia, lethargy, depression
Investigations and management for PR
Raised ESR
CK and EMG normal
Activity modification, Prednisolone (usually respond dramatically), methotrexate if unsuitable
Risk factors for pseudo gout
Increasing age
Haemochromatosis
Hyperparathyroidism
Acromegaly
Wilson’s disease
Low magnesium and phosphate
Features of pseudo gout
Calcium pryophosphate dehydrate crystals
Mono arthritis, rarely oligoarthritis (gouty attack, red swollen, tender, painful)
Knee wrist and shoulders commonly affected
Joint aspirate- positively bifringent rhomboid crystals
Chondrocalcinosis on XRay
Management of pseudo gout
Aspirate joint fluid to exclude septic arthritis
NSAIDs or intra articular/oral steroids
(Colchicine during acute attack- not first line)
NB- no prophylaxis between attacks eg. Allopurinol
Features of psoriatic arthritis
Either a symmetric poly arthritis or an asymmetric oligoarthritis (few joints)
Sacroiltiis
DIP joint disease
Arthritis mutilans (telescope fingers)
Psoriatic skin lesions
Periarticular disease (tenosynovitis, enthetitis, dactylitis)
Nail changes eg, pitting, onchylosis
X ray changes psoriatic arthritis
Erosive changes and new bone formation
Periostitis
Pencil in cup appearance
Management of psoriatic arthritis
Similar to RA, but if just mild, can try NSAID without the DMARD
Raynaud’s disease
Young women with bilateral symptoms
Secondary causes of raynauds phonomenon
Connective tissue disorders- scleroderma, RA, SLE
Leukaemia
Cold agglutins
Vibrating tools
OCP
Cervical rib
Management of raynauds
If suspected secondary raynauds, refer to secondary care
Calcium channel blockers eg, nifedipine, or IV prostacyclin (epoprostenol)
Reactive arthritis
Preceding GI or STD illness 4 weeks before
Conjunctivitis, urethritis, arthritis
Also balantitis and sores on soles of feet (Keratoderma blennorrhagica
NB- young person with single, swollen joint, think gonococcal SA first rather than reactive arthritis, need to rule this out first
NSAIDS, intra articular steroids if one joint, oral steroids if several joints
Sulfalazine and methotrexate often used
Extra articular manifestations and complications of RA
Pulmonary fibrosis, pleural effusion, bronchiolitis, pleurisy
Keratoconjunctivitis sicca, epislceritis, scleritis, corneal ulcers
Osteoporosis
IHD
Infection risk
Depression
Felty syndrome (RA and Splenomegaly)
Carpal tunnel syndrome
Amyloidosis
Initial investigations for suspected RA
Rheumatoid factor (anti CCP if negative as more specific)
X-rays of hands and feet
USS suspected joints (synovitis)
Rheumatoid arthritis management
Refer to rheumatology
First presentation- steroids, NSAID (PPI), DMARD
Continued therapy- 2x DMARD if one insufficient, NSAIDs or paracetamol if continued pain
Flare ups- IM corticosteroids (or PO/articular)
Supportive- physio, occupational therapy, orthotics, surgery for joint deformities
CRP and DAS28 to monitor response to treatment
Features of rheumatoid arthritis
Swollen painful joints in hands and feet
Can come in rapidly (days), or over months to years
Stiffness worse in morning, improves with activity
Insidious development over months
Positive squeeze test- discomfort on squeezing across metacarpal or metatarsal joints)
Nodules
Extrarticular manifestations
Hand deformities are late signs, as is Atlantio axial subluxation
NB- no erythema (this is either gout, pseudogout, or SA)
Poor prognostic factors in RA
RF or antiCCP positive
Poor functional status at presentation
Early erosions on XRay
Extra articular features eg. Nodules
Rheumatoid arthritis x ray findings
Loss of joint space
Periarticular osteopenia (early finding)
Soft tissue swelling
Marginal erosions
Subluxation (late finding)
Not seen in early disease- can take 6-24 months
Pathophysiology of septic arthritis
Staph aureus
Neisseria gonorrhoea in young sexually actively adults
Haematogenous spread eg. abscess
Knee
Features of SA
Acute red swollen joint
Restricted movements
Warm to touch, fluctuating
Fever
Investigations for SA
Bedside- obs, MSK exam
Bloods- 2 cultures from 2 sites, FBC UE ABG CRP ESR LFT
I&S- US guided joint aspirate- microscopy and culture and sensitivities (crystal microscopy too- gout and pseudo gout), imaging
NB- crystal arthritis (gout) and septic arthritis can co exists (may get crystals with bacterial growth)
NB- if a patient develops a staph aureus bacteraemia, they should undergo a TTE to exclude endocarditis
Management of SA
Sepsis 6
MicroGuide for local policy on which ABX to use- IV flucloxacillin and rifampicin (clindamycin if allergy) for 6-12 weeks
May need arthroscopic lavage
NSAIDs and physiotherapy input
Features of Sjögren’s syndrome
Can be primary or secondary to RA or SLE
Keratoconjunctivitis sicca
Dry mouth
Dry vagina
Arthralgia
Raynauds
Myalgia
Polyneuropathy
Parotitis
Increased risk of lymphoid malignancy
Investigations and management of Sjögren’s
ANA and RF (if secondary to RA and SLE)
Anti RO Anti La
Schrimers tear test (less than 10mm- bad)
Artificial saliva and tears (vaginal lubricants) and regular dental check ups
May use hydroxychloroquine
Features of stills disease
Arthralgia
Myalgia
Pharyngitis
Serum ferritin raised, leukocytosis
Salmon pink maculopapular rash
Pyrexia (worse in afternoon evening)
Lymphadenopathy
RF and ANA negative
Management of Stills disease
NSAIDs
Add Steroids
Add DMARD
Cautions with sulfalazine
G6PD deficiency
Allergy to aspirin or sulphonamides (eg. Glicazide, co trimazole)
Adverse effects of sulfalazine
Oligospermia
Steve’s Johnson syndrome
Pneumonitis and lung fibrosis
Myelosupression (anaemia)
Coloured tears (stained contact lenses)
Safe in pregnancy and breast feeding
NB- Sulfalazine (4 S’s- sperm, SJS, stains contacts, safe in pregnancy)
Features of SLE
Type 3 hypersensitivity reaction
More common in women and people of black/Asian origin
General- fatigue, fever, mouth ulcers, lymphadenopathy, arthralgia
Skin- malar rash (not nasolabial folds), discoid rash, photosensitive, raynauds, livedio reticularis, alopecia
Resp- pleurisy, fibrosing alveolitis
Renal- diffuse proliferative glomerulonephritis
Neuropsychiatric- anxiety, depression, psychosis, seizures
Investigations for suspected SLE
Bedside- observations, examination (check lungs), urinalysis
Bloods- FBC UE ABG antibodies (ANA, anti dsDNA) ESR (monitoring- CRP can be normal, if raised= underlying infection), complement levels (C4 low in active disease so good for monitoring)
Imaging- CXR (lung involvement), joint x rays and kidney biopsy (symptom dependent)
SLE management
Refer to rheumatology
NSAIDs, vaccines before immunosuppression
Sun block
Hydroxychloroquine
May need steroids if renal or neuro involvement, more severe disease may also require methotrexate (folic acid), azathioprine, ciclosporin etc.
Limited cutaneous systemic sclerosis
Raynauds
Scleroderma of face and distal limbs
Subtype is CREST- calcinosis, raynauds, esophageal dysmotility, sclerodactyly, telangiectasia
Diffuse cutaneous systemic sclerosis
Scleroderma of face and proximal limbs
Interstitial lung disease and pulmonary artery HTN
Renal involvement- HTN and AKI
Scleroderma
Without internal organ involvement
Tightening and fibrosis of skin
Features of temporal arteritis (GCA)
60+
Rapid onset (less than 1 month)
Headache (exacerbated by combing hair eg.)
Jaw claudication
Visual disturbance- amaurosis fugax, blurring double vision
Tender palpable temporal artery
Features of PMR (ask about these)
Lethargy, depression, low grade fever, night sweats (vasculitis)
Investigations for temporal arteritis
Bedside- ophthalmology exam, observations
Bloods- FBC UE ESR (raised), CRP
I&S- temporal artery biopsy (skip lesions)
NB- GCA can cause Anterior ischemic optic neuropathy - fundoscopy typically shows a swollen pale disc and blurred margins
Treatment of temporal arteritis
Urgent high dose corticosteroids before biopsy- no visual loss, Prednisolone, visual loss (or ischaemic anterior neuropathy), IV methylprednisolone before Prednisolone
If visual loss- urgent ophthalmology review same day
NB- give bone protection (bisphosphanate) and PPI when giving these steroids (will be taking them for a while)
DAS 28 scoring
<2.6- remission
2.6-3.2 - low
3.2-5.1 - moderate
> 5.1 - severe
Leflunomide
HTN and peripheral neuropathy
Sulfalazine
Male infertility (oligospermia), SJS, stained contacts
Hydroxychloroquine
Nightmares and reduced visual acuity
Anti TNF a
Reactivation of TB or hepatitis B
Rituximab
Night sweats and thrombocytopenia
DMARDs in pregnancy
Sulfalazine and hydroxychloroquine
Causes of anaemia in RA
Anaemia of chronic disease
Iron deficiency anaemia secondary to use on non steroidals
Feltys syndrome- anaemia leukopaenia and enlarged Splenomegaly
Pernicious anaemia
Haemolytic anaemia
Platelet count will go up as part of the inflammatory process (also raised LFTs and ferritin)
Tophi
Seen in gout not pseudo gout
Light microscopy findings of gout synovial fluid
Negative bifringent needles
Gout vs pseduogout
On history alone, gout tends to affect big toe, pseudo gout affects the knee
Fibromyalgia associations
Functional somatic syndromes (IBS, CFS, migraines)
Psychiatric disorders eg. Depression, GAD
Sleep disorders- restless leg syndrome
Inflammatory rheumatoid conditions eg. SLE, RA
Investigations for fibromyalgia
Clinical- 3 months of widespread pain in muscles and joints, and 11/18 tender points (bloods still good to do to rule out an inflammatory process, and joint USS if swelling reported)
Steroids and DONT STOP
Don’t stop taking abruptly- have to be tapered down
S- sick day rules (double)
T- treatment card
O- osteoporosis prevention
P- PPI
Drug induced lupus
Doesn’t typically affect the oral mucosa, CNS, or kidneys
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
Investigations for systemic sclerosis
Bedside- observations, physical exam, ECG, urinalysis
Bloods- ANA and anti-scl 70 for diffuse, anti centromere for limited, antibodies, organ involvement eg. echocardiogram, UE
Imaging- hand x ray (calcinosis), CXR (fibrosis), echocardiogram (fibrosis)
Management of systemic sclerosis
No cure
Usual lifestyle modification measures
Symptomatic treatment- GI (PPI), Raynaud’s (nifedipine)
Preventative- lung fibrosis (immunosuppressant), renal crises (low dose ACE)
What test needs to be done prior to starting a biologic
chest X-ray to look for TB prior to starting biologics for rheumatoid arthritis as they can cause reactivation
Antibodies for systemic sclerosis
Limited cutaneous systemic sclerosis: anti-centromere
Diffuse cutaneous systemic sclerosis: anti-scl-70
Management of Bechet’s disease
Topical steroids to mouth ulcers (e.g. soluble betamethasone tablets), topical anaesthetics for genital ulcers (e.g. lidocaine ointment), colchicine can also be used acutely
Maintain remission- systemic steroids (i.e. oral prednisolone), immunosuppressants such as azathioprine, biologic therapy such as infliximab
X ray findings of Paget’s
Bone enlargement and deformity
“Osteoporosis circumscripta” describes well defined osteolytic lesions that appear less dense compared with normal bone
“Cotton wool appearance” of the skull describes poorly defined patchy areas of increased density (sclerosis) and decreased density (lysis)
“V-shaped defects” in the long bones are V shaped osteolytic bone lesions within the healthy bone
Complications of Paget’s
Osteogenic sarcoma (osteosarcoma)
Spinal stenosis and spinal cord compression
CRP and ESR in SLE
CRP normal (if raised, possible infection)
ESR raised (used for monitoring along with complement)
What score can investigate hypermobility
Beighton score
Beighton score is positive if at least 5/9 in adults, or at least 6/9 in children
Live vaccines
Live influenza vaccine
Measles, Mumps and Rubella (MMR) vaccine
Rotavirus vaccine
Shingles vaccine
BCG vaccine
Oral typhoid vaccine
Varicella vaccine
Yellow Fever vaccine
Who cant have a live vaccine
Haematological malignancy
HIV/AIDS
SCID
Stem cell transplant
Chemotherapy or radiotherapy
Immunosuppressive treatment eg. biologics, DMARDs (eg.methotrexate), steroids
What medication should you not prescribe alongside allopurinol
Azathioprine
Azathioprine and allopurinol have a severe interaction causing bone marrow suppression
What imaging do patients with RA require pre-operatively?
Anteroposterior and lateral cervical spine radiographs
The neck cannot be hyperextended during intubation
Atlanto-axial subluxation
Organisms causing arthritis
SA- staph aureus, young: gonorrhoea
Reactive A- E. coli, young: chlamydia
