Rheumatology Flashcards
what is rheumatoid arthritis?
it is a autoimmune disease (associated with antibodies against rheumatoid factor and anti-CCP) resulting in synoviocytes and damaged joint spaces
describe the pathophysiology behind rheumatoid arthritis?
citrullination of self antigens which are recognised by T and B cells which produce antibodies -> RF and anti-CCP
Stimulated macrophages and fibroblasts then release TNFalpha and inflammatory cascade leads to proliferation of synoviocytes (RA joint swelling) which grow over the cartilage and leads to restriction of nutrients
activated macrophages also stimulate osteoclast differentiation contributing to bone damage
what is the typical history of a patient with RA?
usually female 30-50y/o progressive, peripheral and symmetrical polyarthritis affects MCPs, PIPs, MTPs (sparing DIPS) history >6wks morning stiffness > 30 mins \+ fatigue, malaise
how would you examine clinically for diagnpsis pf RA?
soft tissue swelling and tenderness
ulnar deviation + palmar sublaxation of MCPs
swan neck and boutonniere deformities
rheumatoid nodules (espec at elbows)
check median nerve - carpal tunnel association
what investigations are done to diagnose RA?
- RF and anti-CCP
- FBC - normocytic anaemia (chronic disease), WCC is concerns re septic arthritis
- inflammatory markers - elevated
- xray changes in established disease, US/MRI in early disease
- may need PFTs and HRCT chest is lung involvment (pulmonary ifbrosis)
what is the treatment for RA?
- initially DMARD monotherapy - usually methotrexate or combo (leflunomide, hydroxychloroquine, sulfasalazine)
- steroids
- symptom control with NSAIDS if no contraindication
- biologics (anti-TNFs life etanercept) - as last resort
(non drug - OT/PT, podiatry, psychological)
name some extra articular manifestations of RA - using the 3Cs, 3As, 3Ps, 3Ss
3Cs - carpal tunnel syndrome, elevated cardiac risk, cord compression
3As - anemia, amyloidosis, arteritis
3Ps - pericarditis, pleural disease, pulmonary disease
3Ss - sjogrens, scleritis/episcleritis, splenic enlagrment
what are the xray features of RA?
- Loss of joint space
- Erosions
- Soft tissue swelling
- Sublaxation
LESS
what is osteoartheritis?
the most common type of arthritis - degenerative disorder with progressive loss of articular cartilage + new bone formation +capsular fibrosis
what are the causes of OA (physiology)?
- failure of normal cartilage subject to abnormal or incongruous loading for long periods
- damaged or defective cartilage failing under normal conditions of loading
- break up of cartilage due to defective stiffened subchondral bone passing more load to it
what are teh key features of cartilage in OA?
- loss of elasticity with a reduced tensile strenght
- cellularity and proteoglycan content are reduced
what are teh risk factors fo developing OA?
- increases with AGE
- gender - WOMEN
- OBESITY
- TRAUMA
- FHx - for knee and hand OA
A Woman Of Traumatic History
what are the signs and symptoms of OA?
- hip, knee and spine most commonly affects
- pain provoked by movement and weight bearing
- intermittent at first , then constant
- feeling of joint giving way
- Heberdens and Bouchards nodes
what rae the key features of OA of xray?
- loss of joint space
- subchondral sclerosis
- subchondral cysts
- osteophytes
what is teh treatment for OA?
non drug:
- strengthening and range of movement exercises
- weight loss
- laterally wedged insoles or walking stick
pharm:
- paracetamol
- NSAIDs
- topical rubefacients, capsaicin
- intraarticular corticosteroids
surgery:
- replacement of joint
what is fibromyalgia?
a common disorder of central pain processing characterised by chronic widespread pain in all 4 quadrants of body + allodynia present (heightened and painful response to innocuous stimuli)
what is the pathogenesis of fibromyalgia
can be induced by deliberate sleep deprivation (reduced REM sleep and delta wave sleep) = hyperactivation in response to noxious stimualtion and neural activation in brain regions associated with pain perception in response to non painful stimuli
what are teh signs and symptoms of fibromyalgia?
- joint/muscle stiffness
- profond fatigue
- unrefreshed sleep
- numbness
- headaches
- IBD (bowel and bladder)
- depression and anxiety
- poor concentration and memory fibrofog
only physical abnormalities = tender points of muscles
what are the risk factors for developing fibromyalgia?
- affecrs 5% of population
- female:male = 9:1
- peak onset 40-50
- onset may have obvious trigger e.g. emotional or physical
what is the treatment for fibromyalgia?
- specifically based on symptoms e.g. depression, fatigue, sleep disturbance
- drug treatment: low dose amitrypytline (or pregabalin)
- CBT also effective
what is osteoporosis?
skeletal condition characterised by low bone mass, deterioration of bone tissue and architecture = compromised bone strength bone strenght and increased fracture risk
what are the risk factors for osteroporosis?
non modifiable:
- advanced age
- female
- caucasian
- FHx
- Hx of low trauma fracture
modifiable:
- Low BMI
- premature menopaus e
- calcium/vit D deficiency
- inadequate physical activity
- smoking
- XS alcohol
- iatrogenic e.g. corticosteroids, aromatase inhibitors
how is the diagnosis of osteroporosis made?
- dual energy xray absorptiometry (DEXA) of lumbar spine and hip
- T score (bone density for age and gender compared to same gender at age of peak density - 25y/o) < 2.5 = osteoporosis
what is the treatment of osteopenia and osteroporosis?
risk modification in osteopenia : weight bearing exercises, Vit D supplementation, reduced alcohol, smoking cessation, dietary advice about calcium intake + supplements
osteoporosis: vit D and calcium supplementation + oral bisphosphonates (+ denosumab or teriparatide)
name some secondary cuases of osteoporosis?
coeliac disease eating disorders hyperparathyroidism hyperthyroidism mutliple myeloma can cause low BMD
what is gout?
gout is an inflammatory arthritis related to hyperuricemia (occurs in ‘flares’ not constantly)
what is the common joint affected by gout?
1st metatarsophalangeal joint is teh most common joint involved
how does gout occur?
hyperuricaemia and deposition of monosodium urate (MSU) that accumulate in joints and soft tissues result in acute and chronic arthritis, soft tissue masses called tophi, urate nephropathy and uric acid nephrolithiasis
what are the risk factors for gout?
non modifiable factors:
- age >40yrs
- male
modifiable risk factors:
- increased purine uptake (seafood adn meats)
- alchohol intake
- high fructose intake
- obesity
- CHF
- CAD (coronary artery disease)
- dyslipidaemia
- renal disease
- organ transplant
- HTN
- smoking
- diabetes
- urate elevating medicating (diuretics)
how is gout treated?
treat acute attacks and prevent recurrent disease
general prevention:
- maintain optimal weight
- exercise
- diet modification
- reduce alchohol
- smoking cessation
- maintain high fluid intake
medications:
- NSAIDS, oral/IM steroids, colchicine (FIRST LINE)
- in chronic cases: urate lowering therapy (ULTs) needed: allopurinol and febuxostat (xanthine oxidase inhibitors reducing urate formation)
what is lupus?
an autoimmune disease (with antibodies to certain cell nucleus components) leading to inadequate T cell suppressor activity with increased B cell activity
= complex multisystem disease characterised by remissions and flares
what are teh common symptoms and signs of lupus?
Use SOAPBRAIN
Serositis - pleurisy, pericarditis
Oral ulcers
Arthritis
Photosensitivity - or malar/discoid rash
Blood disorders - Low WCC, lymphopenia, thrombocytopenia, hemolytic anaemia
Renal involvement - glomerulonephritis
Autoantibodies -(ANA positive in >90%)
Immunologic tests - low complements
Neurologic disorders - seizures or psychosis
what investigations need to be done to diagnose lupus?
- FBC: raised ESR, raised plasma viscosity , anaemia and leukopenia common
- 95% ANA +ve
- ANti-Ro and Anti-La common
- anti-dsDNA
- antiphospholipid antibodies increase the risk of pregnancy loss and thrombosis
- urinalysis for renal disease!
- skin biopsy and renal biopsy can be diagnostic
what is the treatment for lupus?
- sun protection
- advice on healthy lifestyle in view of cardiovascular risk
- hydroxychloroquine is helpful for rash and arthralgia
- mycophenolate mofetil, azathioprine and rituximab
- short course predinisolone for flares
what is raynauds phenomenon?
a condition due to vasospasm of the digits - painful anf characterised by tupical sequence of colour chnages in response to cold stimulus - also precipitated by stress
what triggers raynauds?
cold and stress
what is the difference between raynauds phenomenon and syndrome
syndrome = idiopathic raynauds phenomenon
raynauds syndrome is common in young females and may improve as they get older (may be familial)
treatment - avoid smoking + keep warm
what diseases are associated with raynauds phenomenon?
raynauds symptoms developing over age 30 = underlying disease
abnormal nail fold capillaries or puffy fingers or photosensitive rash = rhuematic cause
associated with:
- scleroderma
- SLE
- dermatomyositis and polymyositis
- sjogrens syndromes
physical cuaseS:
- use of heavy vibrating tools
- cervical rib
- sticky blood e.g. cryoglbulinaemia
drug induced: beta blockers
what si teh treatment for raynauds?
- keep wamr
- avoid smoking
- CCB - 1st line
- phosphodiesterase-5 inhibitors
- prostacyclins usually effective
what are complications of Raynauds phenomenon?
- digital ulcers
- severe digital ischameia
- infection
how long do raynauds phenomenon episodes last?
last minutes but id there is an underlyign disease then can last hours (and pattern often asymmetrical)
What is vasculitis?
an inflammatory blood vessel disorder causing damage of blood vessel walls with subsequent thrombosis, ischaemia, bleeding and/or aneurysm formation
how is a diagnosis through a Hx made of vasculitis?
symptoms: fever, weight loss, malaise, fatigue, diminished appetite, sweats
‘glove and sweater approach’
- raynauds?
- MSK: arthralgia, myalgia, proximal muscle weakness
- CNS/PNS: headache, visual loss, tinnitus, stroke, seizure, encephalopathy
- nose bleeds, crusts, ulcers
- heart/lungs: pericarditis, cough, chest pain, heamopytsis, dyspnoea
- GI: abdo pain
- renal: heamaturia
- limbs: neuropathy, digital ulcers/ischaemia
DHx
FHx of vasculitis
age/gender/ethnicity
what signs in a physical exam are present in a diagnosis of vasculitis?
vital signs: BP raised, pulse
ski: palpalble purpura, livedo reticularis, nodules, digital ulcers, gangrene, nail bed capillary changes
neurologic: cranial nerve exam, sensorimotor exam
ocular exam: visual fields, scelritis, uveitis, epislceritis
cardiopulmonary exam: crackles, pleural rub murmurs, arrythmias
abdo exma: tenderness, organomegaly
name the primary vasculitides?
- small vessel vasculitis: microscopic polyangitis (MPA), granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangitis (EGPA), IgA vasculitis
- medium vessel vasculitis: polyarteritis nodosa (PAN), kawasaki disease (KD)
- large vessel vasculitis: takayasu arteritis (TAK), giant cell arteritis (GCA)
what can vasculitis occur secondary to?
- infections
- drugs
- malignancy
- connective tissue disease
what diagnostic tests are done to diagnose vasculitis?
- urine dipstick +/- microscopy are needed to identify underlying glomerulonephritis
- FBC, U&Es, LFTs, CRP, PV, ESR
- ANA, ANCA, RF
- Complements C3 &C4
- hep screening for B and C, HIV
- cryoglobulins
- serum and urine protein electrophoresis
- CK, blood cultures, ECG
- CXR, CT, MRI, ateriography, and CT-PET to determine organ involvement
what is the treatment for vasculitis?
- rule out infection, stop offending drugs in secondary causes
1st line: corticosteroids
2nd line: cytotoxic medications, immunomodulatory or biologic agents (e.g. methotrexate, azathiprine, mycophenolate mofetil, rituximab, cyclophosphomide)
what is hypermobolity spectrum disorder?
a pain syndrome in people with joints that move beyond normal limits - is due to laxity of ligaments, capsules an d tendons (origin may be microtrauma)
who gets hypermobility spectrum disorder?
10% of popn
often familial
mroe common in women and asian people
usually presents in childhood
what are the signs and symptoms of hypermobility spectrum disorder?
pain around joints, worsened with activity, with fatigue
- soft tissue rheumatism
- abnormals skin: hyperextensible, thin, striae
- marfanoid habitus
- arachnodactyly
- drooping eyelids, myopia
- hernias and uterine/rectal prolapse
- recurrent sublaxations or dislocations
what si teh treatment for hypermobility spectrum syndrome?
imrpove pain (paracetamol) and reduce disability strenghtening exercises to reduce joint sublaxation work on posture and balance splinting and possible surgical interventions
what is sjogrens syndrome?
chronic autoimmune inflammatory disorder characterised by diminished lacrimal and salivary gland secretion
(80% are women )
what is secondary from of sjogresn sydnrome associated with?
udnerlying rheumatic disease
what are teh common signs and symptoms of sjogrens syndrome? (MADFRED)
Myalgia Arthralgia Dry mouth Fatigue Raynauds phenomenon Enlarged parotids Dry eyes
what are the investgiations done to diagnose sjogrens syndrome?
anti ro and anti la antiboides (90% of patients)
RF and anti dsDNA antibodies
Schirmers test - measures tear volume
salivary gland biopsy may be needed
what is the treatment for sjogrens syndrome?
avoid dry or smoky atmospheres
dry eyes - artifical tears
dry mouth - artifical saliva, sugar free gum
skin emollients, vaginal lubricants
immunosuppressants/steroids (RARELY needed)
what is GCA?
giant cell arteritis is a chronic vasculitis of alrge and medium sized vessels (most commonly arteries form arch of aorta) that occurs in people >50y/o (often referred to as temporal arteritis)
symptoms of GCA?
- headache (70%)
- localised, unilateral, boring, lancinating in quality over the temple
- tongue or jaw claudication upon mastication
- visual findings include amaurosis fugax, blindness, diplopia and blurring (occlusive arteritis can result in anterior ischaemic optic neuropathy and acute visual loss)
- scalp tenderness over temporal artery
what are the risk factors for GCA?
- age
- increased prevalence in northern latitude
- women (x2-4 more common)
- common in white people
- strong association with polymyalgia rheumatica (50%)
- genetic predisposition: HLA-DR4
how is a diagnosis of GCA made?
presence of any 2 or more of the following in patients >50y/o with:
- raised ESR, CRP or PV
- new onset of localised headache
- tenderness and decreased pulsation of temporal artery
- new visual symptoms
- biopsy revealing necrotising arteritis
how is GCA treated?
- prednisolone 60-100mg PO per day for 2 wks then taper down
- fro acute visual symptoms, consider 1g methylprednisolone IV pulse therapy fro 1-3 days
- low dose aspirin therapy to reduce thrombotic risks
what is polymyalgia rhuematica?
clinical syndrome characterised by pain and stiffness of the shoulder, hip girdles and neck
impacts elderly, associated with mornign stiffness and elevated inflammatory markers
who is usually affected by polymyalgia rheumatica?
- increases with age
- average onest age >70y/o
- is associated with GCA
what are teh symptoms of polymyalgia rheumatica?
- new sudden onset or proximal limb pain and stiffness (neck, shoulders, hips)
- difficulty rising from chair or combing hair
- nigth time pain
- morning stiffness
- systemic symptoms around 25% (fatigeu, weight loss, low grade fever)
- decreased range of motion of shoulders, hip and neck
- muscle strength is usually normal - may be limited by pain or stiffness
- muscle tenderness
how is the diagnosis of polymyalgia rheumatica made?
Hx and exam supported by finding raised inflammtory amrkers
check ESR or PV as well as CRP
consider temporal arteyr biopsy if symptoms of GCA
what is the treatment for polymyalgia rheumatica?
prednisolone 15mg daily then tapered slowly
methotrexate can be steroid sparing in relapsing patietns
what is systemic sclerosis?
multisystem autoimmune disease
increased fibroblast activity resulting in abnormal growth of connective tissue which leads to vascular damage and fibrosis
2 types: limited SSc and diffuse SSc
what are the common signs and symptoms of limited SSc? (CREST)
usually many yrs of raynauds symptoms before scleroderma Calcinosis cutis Raynauds phenomenon Eosophageal dysmobility Sclerodactyly Telangioectasia
what are the common signs and symtpoms of diffuse SSc?
less common and higher risk of mortality
characterised by sudden onset of skin involvment, and is proximal to elbows and knees
what investigations are doen to diagnose SSc?
inflammatory markers usually normal !
XR hands - calcinosis
CXR, HRCT, PFT - pulmonary disease
ECG and ECHO - PA HTN, heart failure, myocarditis and arrythmias
antibodies:
- ANA +ve (90%)
- anti-centromere antibody (limited SSc)
- Scl-70 and anti RNA polymerase III antibodies (diffuse SSc)
what is the treatment for SSc?
- no cure
- psychological support may be needed
- calcium antagonist for raynauds
- methotreaxate and mycophenolate mofetil may reduce skin thickening
- ACEi prevent hypertensive crisis and reduce mortality from renal failure
- short courses of prednisolone for flares
- PPI for GI symptoms
what are the spondyloarthropathies?
these are a group of conditions that affect teh spine and peripheral joints and are associated with the presence of HLA-B27 they comprise: - ankylosing sponylitis - enteropathic arthritis - psoriatic arthritis - reactive arthritis
what are the common clinical features of spondyloarthropathies?
- sacroiliac disease (back/buttock pain)
- inflammatory arthropathy of peripheral joints
- enthesitis (inflammation at tendon insertions)
- extra-articular features (skin/eye/gut)
describe presentation of ankylosing spondylitis.
usually in young men (teens - mid thirties)
presentation with bilat buttock pain, chest wall and thoracic pain
what examination + investigatiosn is done to diagnose ankylosing spondylitis?
often normal at first
then later: loss of lumbar lordosis, exaggerated thoracic kyphosis, schobers test +ve, reduced chest expansion
CRP maybe raised, MRI spine and SI joints
what treatment is needed for ankylosing spondylitis?
NSAIDs and physio, TNF inhibitors, IL-17 inhibitors
what typical exam findings are found on patients with psoariativ arthritis?
- psoriasis (10%)
- oligo-arthritis with dactylitis or ‘sausage’ digit
- symmetrical or monoarthritis
what ivnestgiations are done to diagnose psoriatic arthritis
CRP often raised
central joint erosions seen early on MRI leading to ‘pencil in a cup’ XR sign
what is the treatment for psoriatic arthritis?
NSAIDd, DMARDs, TNF inhibitors, IL-17 inhibitors, IL 12/23 inhibitors
what is the presentation for reactive arthritis?
few days - 2 weeks post infection, acute asymmetrical lower limb arthritis develops
infection usually post dysentery (salmonella /shigella /campylobacter) or post urethritis/cervicitis (chlamydia trachomatis)
skin - circinate balanitis, keratoderma blennorrhagica
eye - conjunctivitis, uveitis
enthesitis
what investigations are done to diagnose reactive arthritis ?
serology/microbiology
inflammatory markers raised
joint aspirate to rule out septic/crystal arthritis
what is the treatment for reactive arthritis?
treat infection - ABX
NSAIDs and joint injections
those that do no resolve within 2 yrs -> need DMARDs
what are teh two different types of periheral disease in enteropathic arthritis?
type 1 - oligoarticualr, asymmetric and has a correlation with IBD flares
type 2 - polyarticular symmetrical and less correlation with IBD flares
(10-20% of those with IBD develop athropathy)
what % of patients with enteropathic arthritis develop peripheral disease and axial disease?
2/3 peripheral disease
1/3 axial disease
what si teh treatment for enteropathic arthropath?
NSAIDs may flare IBD so consider DMARDs
TNF inhibitors will treat both bowel disease and arthritis
what are the extra articualr manifestations of ankylosing spondylitis? (ALL the As)
Anterior uveitis Aortic incompetence AV block Apical lung fibrosis Amyloidosis
what are teh features of inflammatory back pain? (IPAIN)
Insidious onset Pain at night Age at onset <40 Improvement with exercise No improvement with rest
what are dermomyositis and polymyositis?
rare idiopathic muscle diseases that are characterised by inflammation of striated muscle
peak onset 40-50 y/o
what are the signs and symptoms of dermomyositis and polymyositis?
insidious onset of muscle proximal weakness, often painless
SOB, rash, raynauds
what are the ivnestgiations to diagnose dermomyositis and polymyositis?
some have raised inflammatory markers FBC usually normal kidney not affected rasied ALT (from muscle) 80% ANA +ve myositis seen on MRI
what is the treatment for dermomyosisit and polymyositis?
high dose corticosteroids
inflammatory markers and CK used to monitor disease
repeat EMG studies/MRI or biopsy may be needed
long term control - methotrexate or azathioprine (or rituximab or IV immunoglobulin)
