Rheum I and II Flashcards
cause of RA
- autoimmune
- triggered by T cell activation
- causes deformities and disability
synovitis and RA
- swollen, warm, stiff joints
- ALWAYS multiple joints
- symmetrical
- low back NOT involved
- tethering of tissues -> decreased ROM and erosion of joints -> deformity and loss of function
rheumatoid nodules
- usually found on pressure points
- elbow, heel, MCP joint
- nodule has central area of fibroid necrosis
- less fluid filled than cysts
common joints involved in RA
- c spine
- shoulders
- elbows
- hands and wrists (DIP spared)
- hip (later in disease)
- knee
- foot and ankle
boutonniere deformity
- assoc with RA
- flexion of PIP
- hyperextension of DIP
swan neck deformity
- assoc with RA
- hyperextension of PIP
- flexion of DIP
other organs impacted by RA
- lungs
- eyes
- skin
- heart
- NS
- blood
heart complications of RA
- often see pericardial effusions
- MI
- stroke
- atherosclerosis
- pericarditis
- endocarditis
- LVHF
- vasculitis
what causes NS issues with RA
- c spine instability (usually at C1-2)
- causes peripheral nerve entrapment and vasculitis -> mononeuritis multiplex
blood sx associated with RA
- hypochromatic microcytic anemia
- also have low serum ferratin or normal Fe binding capacity
- seen in almost all RA pts
common PE findings in RA
- warm, erythematous joints
- commonly involves hands and wrists
- DIP excluded
- ulnar deviation of digits
- nodules
- baker cysts
- general decreased ROM
serology for RA
- ANA- not diagnostic and doesnt follow disease activity
- RF- often seroneg in first year of disease
- anti-CCP- highly specific, associated with shared epitope
diagnostic criteria for RA
- joint involvement (5 pt max)
- serology (3 pt max)
- duration (1 pt max)
- acute phase reactants (1 pt max)
- need 6 points for definitive RA dx
other considerations for RA dx
- family hx
- joint tap for synovial fluid
- x ray
- MRI or bone scan
clinical course of RA
- damage occurs early in most pts
- 50% show joint space narrowing in first 2 years
- 50% disabled after 10 years
- early death
what is the DOC for RA?
- methotrexate
- works by inhibiting DNA synthesis through inhibition of dihydrofolate reductase
- requires monitoring of blood, liver, lungs, kidneys
treatment of RA
- NSAIDS
- steroids
- mtx/ DMARDS
- tumor necrosis inhibitors
- if newly dx use steroids until mtx takes effect
- steroids for flares
- intra-articular triamcinolone
- hydroxychloroquine and sulfasalazine often in combo with mtx
felty’s syndrome
- RA + neutropenia + splenomegaly
polymyalgia rheumatica (PMR)
- idiopathic inflammatory disorder
- proximal muscle pain
- abrupt onset, self limited
- sx worse in AM
- “trouble getting dressed”
- highly associated with temporal arteritis
- dx of exclusion, get ESR
treatment of PMR
- PO steroids
- app pts should be biopsied for temporal arteritis or consult optho
- no imaging
reactive arthritis
- autoimmune reaction after infections (GI or GU)
- associated with HLA- B27 halotype
- triad of urethritis, arthritis, conjunctivitis
common infections associated with reactive arthritis
- chlamydia
- campylobacter
- salmonella
- shigella
- yersinia
diagnosis of reactive arthritis
- based on hx/ PE
- cultures to det infection
- CBC, CRP, UA, urine culture
- echo
- HIV pts higher risk
treatment of reactive arthritis
- based on sx
- treat infection
- NSAIDs
- steroids
- DMARDS if NSAIDs and steroids are ineffective
- 2/3 recover spontaneously
anterior uveitis
- occurs in pts with reactive arthritis
- one of leading causes of preventable vision loss
- progresses over a few hours
si/sx of anterior uveitis
- red eye
- pain worse with reading
- blurred vision
- photophobia
- excessive tear production
- abnormally shaped pupil
juvenile idiopathic arthritis (JIA)
- kids < 16 y/o
- chronic inflammation of connective tissue
- pain and swelling
- can impact one or many joints
- duration > 6 weeks
si/sx of JIA
- often have fever and rash that is similar to SLE
- joint inflammation
- contractures
- joint damage
- leads to decreased mobility, strength, endurace
- episodic- go from sx free to extreme pain quickly
subtypes of JIA
- systemic onset arthritis
- polyarticular arthritis
- oligoarticular arthritis
- enthesis
systemic onset arthritis
- subtype of JIA
- arthritis and fever > 103
- fluctuating rash
- inflammation of internal organs and joints
- anemia and leukocytosis
polyarticular arthritis
- subset of JIA
- arthritis in 5 or more joints
- constant pain
oligoarticular arthritis
- subset of JIA
- arthritis in 4 or fewer joints
- if dx before 7 have best chance of disease subsiding
- high risk uveitis
enthesis related arthritis
- subtyle of JIA
- involves ligaments as well as spine
- aka sondyloarthritis
treatment for JIA
- refer to rheum
- sx control
- intra-articular steroid injection if only a few joints involved
- PO prednisone
- DMARDS +/- biologics
polymyositis
- immune mediated
- inflammation of muscles and associated tissues in response to cell damage
- bilateral muscle weakness
associated diseases with polymyositis
- raynauds
- RA
- SLE
- Sjorgens
- CV disease
- cancer
sx of polymyositis
- proximal muscle weakness
- dysphagia
- bloating, constipation
- arrythmias and conduction defects
- pulmonary and kidney involvement
diagnosis of polymyositis
- CBC, ESR, elevated CK and LDH
- elevated liver enzymes
- ANA
- EMG
- muscle biopsy
treatment of polymyositis
- steroids 4-8 weeks
- monitor CK lev and muscle strenth
- immunosuppressives if no response to steroids or extra-skel manifestations
- IVIG
- TNF inhibitors
- consult
dermatomyositis
- heliotrope rash and grotton papules
- muscle weakness
- capillary loops at base of fingernails- “dirty look”
heliotrope rash
- sx of dermatomyositis
- purple discoloration on upper eyelids
- flat red rash on cheeks and trunk
gottron papules
- sx of dermatomyositis
- scaly rash on knuckles
muscle biopsy for dermatomyositis
- shows perivascular and perimysial inflammation
inclusion body myositis
- asymmetrical distal muscle weakness
- more common in older men
- falls common
- facial muscle weakness
- dysphagia and choking
- progresses slowly so may be confused with MD
treatment of inclusion body myositis
- resistant to immunotherapy
- axathioprine + prednisone often tried for a few months
treatment of dermatomyositis
- same as polymyositis
livedo reticularis
- reticular/ lace like pattern on skin
- cyanotic discoloration of skin
- found mainly on arms and legs
- d/t altered flow in BV feeding skin -> dilation of other vessels to compensate
giant cell arteritis
- aka temporal arteritis
- large vessel disease
- most common of systemic vasculitis
- in older adults
- strong association with PMR
clinical manifestations of giant cell arteritis
- new onset HA and temporal tenderness
- jaw claudication
- abrupt onset visual disturb
- thoracic aortic aneurysm possible
classification criteria for giant cell arteritis
- need at least 3 of the following 5:
- age > 50
- new onset localized HA
- tenderness or decreased pulse of temporal artery
- ESR > 50 (often >70)
- biopsy of temporal artery with necrotizing arteritis and giant cells
diagnosis of giant cell arteritis
- gold standard= temporal a. biopsy
- scheduling biopsy should not interfere with tx
- color doppler US, CT, MRI
treatment of giant cell arteritis without vision loss
- prednisone as single dose
- taper to d/c steroids- drop by 10%
- treatment can last 12-16 months
- also give low dose ASA
treatment of giant cell arteritis with vision loss
- methylprenisolone IV X 3 days then PO prednisone
- taper to d/c steroids- drop by 10%
- treatment can last 12-16 months
- also give low dose ASA
Takayasu Arteritis
- large vessel disease
- inflammation of aorta and its branches
- mainly females, usually younger
- classically asian woman in 40s
clinical presentation of takayasu arteritis
- look chronically ill with constitutional sx
- pulselessness
- extremities are cool, dev arm or leg claudication
- subclavian steel syndrome
- myalgias and lg joint synovitis
- bruits
- HTN
- elevated ESR and CRP
- HA and vision loss
dx of takayasu arteritis
- elevated ESR and CRP
- MRI, CT, angiogram
- normochromic normocytic anemia
treatment of takayasu arteritis
- acute- steroids
- chronic- surgical bypass once arterial stenosis has occured
polyarteritis nodosa (PAN)
- necrotizing vasculitis of medium +/- sm vessels
- systemic sx: kidneys*, skin, joints, muscles, nerves, GIT
- NO lung involvement*
- not associated with ANCA
- middle aged/ older men
- associated with HBV and HCV
clinical manifestations of PAN
- nonspecific constitutional sx
- often have HTN d/t renal disease
- skin rash/ ulcers: purpura, livedo reticularis
- peripheral neuropathies
- renal sx
- GIT sx
diagnosis of PAN
- elevated ESR
- UA shows proteinuria
- biopsy if skin, muscle, or nerve involvement
- nerve conduction study if nerve involvement
classification criteria for PAN
- need at least 3 of the following:
- unexplained wt loss > 4 kg
- myalgias/ weakness
- mono or polyneuropathy
- new onset diastolic > 90 mmHg
- elevated BUN
treatment for mild PAN
- prednisone PO X 4 weeks
- taper to d/c
treatment for mod-severe PAN
- prednisone PO plus cyclophosphamide Q 2 weeks for three doses
treatment for severe PAN
- methylprednisolone IV X 3 days then PO steroids
kawasaki disease
- medium vessel disease
- most common in kids*
- fever > 5 days*
- coronary artery involvement -> aneurysm if untreated
clinical presentation of kawasaki disease
- fever > 5 days
- bilat bulbar conjuctival injection
- oral mucous membrane changes
- polymorphous rash
- cervical LD
- peripheral extremity changes
- need at least four of five listed plus fever to dx
diagnosis of kawasaki
- fever plus 4/5 of sx
- get baseline echo + every 2-6 weeks
- no labs
treatment of kawasaki
- IVIG single infusion over 8-12 hours
- ASA 30-50 mg/kg X 4 doses until fever is gone for 48 hours
- continue with low dose ASA
- postpone live vaccines for at least 11 mo if pt has had IVIG
microscopic polyangiitis
- small vessel disease
- necrotizing vasculitis with few/no immune deposits
- +/- medium vessels
- most common cause of pulmonary renal syndrome
clinical presentation of microscopic polyangiitis
- purpura
- pulmonary hemorrhage*
- interstitial lung fibrosis
- ulcers
- splinter hemorrhages
diagnosis of microscopic polyangiitis
- elevated ESR
- positive ANCA
- UA- hematuria, proteinura, RBC casts
- CT
treatment of microscopic polyangiitis
- cyclophosphamide PO plus prednisone for 3-6 mo
- key to good outcome is early dx
- recurrence possible
granulomatosis with polyangiitis
- aka wegner’s disease
- necrotizing vasculitis of small vessels
- +/- medium vessels
- common in 40s/50s
- ANCA associated
clinical presentation of granulomatosis with polyangiitis
- triad of upper and lower respiratory sx and glomerulonephritis
- constitutional sx
- crusting, ulceration, bleeding, and perf of nasal septum
- proptosis, ptosis, opthalmoplegia
- scleritis
upper RT sx associated with granulomatosis with polyangiitis
- nasal congestion
- sinusitis
- otitis media
- mastoiditis
lower RT sx associated with granulomatosis with polyangiitis
- cough
- dyspnea
- hemoptysis
diagnosis of granulomatosis with polyangiitis
- elevated ESR and CRP
- slight anemia and leukocytosis
- UA shows proteinura and red cell casts
- CXR followed by chest CT
treatment of granulomatosis with polyangiitis
- cyclophosphamide plus prednisone
- continue for 3-6 mo
IgA vasculitis
- aka Henoch-Schonlein
- small vessel disease
- affects skin, GIT, kidneys
- found in kids**
- generally self limited, cause unknown
clinical presentation of IgA vasculitis
- purpura (all)
- arthralgia/arthritis in lower extremity large joints
- abdominal pain- possible intussusception
- renal disease- hematuria without RBC
classification of IgA vasculitis
- MUST have purpura and at least one of the following:
- abdominal pain
- arthritis
- renal involvement
- leukocytoclastic vasculitis or proliferative glomerulonephritis with IgA deposition
diagnosis of IgA vasculitis
- clinical
- based on purpura plus at least one other cardinal sx
treatment of IgA vasculitis
- supportive/ symptomatic
- oral hydration
- NSAIDs (naproxen) for joint/ abdominal pain
- if severe abdominal pain and decreased PO intake give them prednisone
behcet disease
- variable vessel disease
- triad of aphthous ulcers, genital ulcers, recurrent eye inflammation
- HLA-B51 risk factor
clinical presentation of behcet disease
- aphthous ulcers- numerous, frequent, more painful
- genital ulcers
- anterior and/or posterior uveitis
- lungs- aneurysms
- GIT ulcers
treatment of behcet disease
- steroids
- colchicine and thalidomide for mucocutaneous findings
- cyclophosphamide if severe ocular or CNS sx
