Spondyloarthropathies, PMR, FM Flashcards

1
Q

What are spondyloarthropathies?

A

group of inflammatory arthropathies that share distinctive clinical, radiographic and genetic features:

  • inflammatory axial spine involvement
  • asymmetrical peripheral arthritis
  • enthesitis (inflammation of sites where tendons and ligaments attach to bone)
  • inflammatory eye disease
  • mucocutaneous features
  • negative RF
  • high freq of HLA B27 abs
  • familial aggregation

these are:

  • ankylosing spondylitis
  • reactive arthritis (Reiter’s syndrome)
  • psoriatic arthritis
  • enteropathic arthritis (crohns and UC)
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2
Q

Disease associations w/ HLA-B27?

A
  • ankylosing spondylitis: over 90%
  • reactive arthritis 85%
  • reiters syndrome 80%
  • IBD 50%
  • psoriatic arthritis 50%
  • whipple’s disease 30%
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3
Q

What is ankylosing spondylitis (AS)? Extra-articular manifestations?

A
  • chronic inflammatory disease of jts of the axial skeleton
  • higher incidence at higher latitudes, Scandinavian countries
  • changes seen in sacroiliac jts and hips
  • inflammation around enethesis
  • extra-articular manifestations:
    anterior uveitis
    aortic valvular disease
    restricted chest expansion
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4
Q

Diagnostic features of AS?

A
  • insidious onset low back pain for longer than 3 months
  • improves w/ exercise not rest
  • morning stiffness for longer than 30 min
  • awakened by pain during the 2nd half of the night
  • alternating buttock or posterior thigh pain
  • sites of enthesitis
  • sacroiliitis on x-ray
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5
Q

diff in characteristics of inflammatory back pain and mechanical back pain?

A
  • inflammatory: prolonged AM stiffness and max pain is early AM, exercise improves sxs, chronic duriation, 9-40 age at onset, on xray: sacroiliitis, vertebral ankylosis, syndesmophytes
  • mechanical: minor am stiffness (less than 45 min), late in day max pain, exercise worsen sxs, duration can be acute or chronic, age at onset: 20-65 yrs, X-ray: osteophytes, malalignment
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6
Q

What willl you see on radiograph of ankylosing spondylitis?

A
  • single most impt imaging technique for dx and f/u
    changes:
  • early changes are at sacral iliac jts: erosion and sclerosis
  • involvement of apophysial jts of spine
  • ossification of the annulus fibrosus
  • calcification of the anterior and lateral spinal ligaments
  • squaring and generalized demineralization of the vertebral bodies
  • radiographic changes of the spine and are referred to as the bamboo spine
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7
Q

Characteristics of AS?

A
  • typical pt is males aged 20-40
  • sxs appear gradually and are usually not specifc to AS, time to correct dx is 8.5-14 yrs
  • first sxs are typically chronic pain and stiffness in the middle spine assoc w/ referred to one or the other buttock or in the back of the thigh
  • assoc w/ morning stiffness that improves w/ exercise
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8
Q

What is the modified New York criteria for dx for AS?

A
  1. limited lumbar motion
  2. low back pain for longer than 3 months - improved w/ exercise, not relieved w/ rest
  3. reduced chest expansion
  4. bilateral grade 2-4 sacroiliitis on xray
  5. unilateral grade 3-4 sacroiliitis on xray
    * definite AS if: criteria 4 or 5 plus 1, 2 or 3
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9
Q

What is Enthesitis?

A

inflammation of the entheses, sites where tendons or ligaments insert into the bone

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10
Q

What are extra-articular manifestations that may occur in AS?

A
  • skin rashes (presents like psoriasis - onchylosis, nail pitting)
  • eye inflammation: esp uveitis
  • lung involvement
  • cardiac involvement: w/ aortic valve disease
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11
Q

How common is anterior uveitis in AS?

A
  • 30-40% of people w/ AS will experience iritis at least once
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12
Q

Early presentation of AS?

A
  • sxs: LBP, stiffness, fatigue
  • extra auricular manifestations: ocular skin/nail enthesitis
  • disease progression: sacroiliitis
  • morbidity/mortality: pain, fxnl limitation
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13
Q

Moderate AS presentation?

A
  • sxs: spinal limitation, fxnl limits, night pain
  • extra-articular manifestations: chronic uveitis, IBD
  • disease progression: hip involvement, spondylitis
  • morbidity/mortality: AS complications, drug toxicity, comorbidities
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14
Q

Severe AS presentation?

A
  • sxs: spinal immobility
  • extra-articular manifestations: aortitis, retristrictive lung, heart block
  • disease progression: bamboo spine
  • morbidity/mortality: fracture, death
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15
Q

What is Reactive arthritis? Triad? Diff types? Complications?

A
  • acute inflammatory arthritis occurring 1-3 wks after infectious event (GU, GI, idiopathic)
  • triad: arthritis+urethritis (cervicitis)+ conjunctivitis (classic triad found in less than 1/3 of pts)
  • post-veneral onset (Reiters): MC and more common in males 5:1
  • post-dysenteric: less common, equal in M and F
  • course: usually self limiting (less than 6 mos), can become chronic w/o tx
  • complications: acute anterior uveitis 5%, myocarditis, fasciitis
  • decreasing incidence in HIV era (condom use)
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16
Q

Presentation of Reiter’s syndrome?

A
msk signs and sxs:
- arthritis
- enthesitis- heel tendonitis
- dactylitis 
extra-articular signs and sxs: 
- GU: dysuria and pelvic pain
- conjunctivitis
- oral ulcers, tongue lesions 
- palate erosion 
- rashes- pustules, keratoderma blenorrhagica 
- nail changes - dystophy 
- genital lesions 
- plantar periostitis
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17
Q

Infectious triggers for reactive arthritis?

A
- enteric infections:
shigella
salmonella
yersina enterocolotica
campylobacter
- urogenital infections:
chlamydia trachomatis, C. pneumoniae 
ureaplasma urealyticum
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18
Q

What is psoriatic arthritis? How common is this? Presentation and course?

A
  • chronic inflammatory arthropathy in setting of psoriasis
  • etiology and genotype unclear
  • 1-5% of US pop has psoriasis: 5-42% of these develop psoriatic arthritis (skin usually preceded jts):
    frequency of PsA increases w/ disease severity and duration, est 350-400,000 pts in USA
  • nail changes: pitting, dystrophy, onycholysis
  • course: chronic, destructive arthritis in 30-50%
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19
Q

Clinical characteristics of psoriatic arthritis?

A
  • inflammatory arthritis in DIPs, PIPs (pencil and cup deformity)
  • asymmetric arthritis
  • sausage digits
  • nail pitting (onycholysis)
  • no rheumatoid nodules
  • RF test negative
  • erosive arthritis w/o osteopenia
  • sacroiliitis, often asx
  • paravertebral ossification
  • enthesopathy
  • rash
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20
Q

Tx for all the spondyloarthropathies?

A
  • tx sxs w/ NSAIDs initially
  • PT, stretching and exercises to preserve spine and jt fxn
  • maintain good posture
  • sulfasalazine, methotrexate found to be beneficial
  • anti-TNF aka TNF inhibitors (Remicade, Humira, Enbrel)
  • prevent eye complications by early recognition and tx
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21
Q

Use of NSAIDs in tx spondyloarthropathies?

A
  • effective for: inflammatory back pain, spinal stiffness, peripheral arthritis, enthesopathy
  • no evidence that they inhibit disease progression
  • FDA approved NSAIDs for AS: indomethacin, indomethacin-SR, EC ASA, naproxen, sulindac, diclofenac
  • anecdotal reports and few studies suggest that specific NSAIDs may be more effective:
    phenylbutazone: limited availability: risk of agranulocytosis
    indomethacin: esp in long acting form
    diclofenac: as effective as Indocin
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22
Q

When should you consider DMARDs for spondyloarthropathies?

A
when:
- antiinflammatory therapy is insufficient to control sxs
- progression of inflammatory axial disease noted
- active persistent polyarthritis 
- uncontrolled extra-articular disease:
TNF inhibitors
sulfasalazine
methotrexate
23
Q

What is Polymyalgia Rheumatica?

A
  • PMR is an inflammatory condition of unknown etiology
  • characterized by aching, stiffness in shoulder and pelvic girdles and the neck
  • occurs in people older than 50
  • usually responds to low doses of steroids
  • is related to GCA, w/ bx proven GCA present in 4-21%
24
Q

Epidemiology of PMR?

A
  • prevalence of 52.5 cases/100,000 persons aged 50years and older
  • incidence increases w/ age, peaks at 70-80yrs
  • females greater than males in all age groups (2:1)
  • higher incidence at higher latitudes, scandinavian countries
  • rarely reported in blacks, but appears to have same presentation course and response to tx
25
Q

Dx criteria for PMR?

A

criteria set a:

  • pts 50yo or older
  • bilateral aching and stiffness for more than 1 month and involving 2 of following areas: neck or torso, shoulders or proximal regions of the arms, hips or proximal aspects of the thighs
  • ESR greater than 40mm/hr
  • exclusion of other dx except for GCA
  • presence of all tehse criteria defines PMR

criteria set b:
same as above, + morning stiffness lasting more than 1 hr, rapid response to prednisone

criteria set c:
criteria from above + depression and/or wt loss, bilateral upper arm tenderness, dx of probable PMR is made if any 3 or more of these criteria are fulfulled, presence of any 3 or more criteria yields a sensitivity of 92% and specificity of 80%

26
Q

Etiology of PMR?

A
  • probably polygenic in which mult enviro and genetic factors influence susceptibility and severity
  • possible infectious triggers:
    viruses: adenovirus, RSV, parvovirus, parainfluenza
    bacteria: mycoplasma, chlamydia pneumoniae
  • genetic component probable:
    HLA-DRB104 and 101 appear to be most assoc w/ susceptibility to PMR
    -genetic polymorphisms of additional genes involved in initiation and regulation of inflammatory rxn
  • possible subclinical vasculitis
27
Q

Clinical manifestations of PMR?

A
  • persistent pain (for at least 1 month): aching and morning stiffness in neck, shoulder and pelvic girdles lasting 30 min, discomfort is bilateral, worse w/ movement, and usually interferes w/ ADLs
  • shoulder pain is presenting sign in 70-95%, hips and neck 50-70%, pain usually radiates distally towards elbows and knees
  • systemic signs seen in 1/3: fever, malaise, fatigue, anorexia, and wt loss
  • exam reveals little evidence of proximal jt swelling or tenderness, MRI studies have shown subdeltoid and subacromial bursitis are more prominent than actual jt synovitis
  • distal manifestations also seen in 1/2 of cases:
    nonerosive, self-limiting, asymmetric peripheral arthritis (knee/wrist), carpal tunnel, distal extremity swelling and pitting edema over dorsum of hands and wrists, ankles and feet
28
Q

Labs for PMR?

A
  • ESR will be above 40mm/hr (nl in up to 20%)
  • CRP less influenced by other factors, may be more sensitive and direct measure
  • modest anemia of chronic disease in 2/3
  • mildly abnormal LFTs in 1/3
  • rheum factor and ANA usually negative
  • CK and CPK enzymes are normal
29
Q

DDx for PMR?

A
  • SLE: look for pleuritic and pericarditis, leukopenia and thrombocytopenia, check for anti-dsDNA and anti-ENA abs
  • RA: small jts of hands/feet, only partially responsive to steroids, considerable overlap b/t PMR and seronegative RA
  • Polymyositis: symmetric proximal muscle weakness, pain not prominent, elevated CK, ALP, abnorm EMG, myositis on muscle bx
  • fibromyalgia
  • late onset spondyloarthropathy
  • malignancy: solid (kidney, ovary, stomach), heme (Myeloma, primary amyloidosis)
  • infection: bacterial endocarditis
  • lack of adequate response to prednisone and presence of atypical features should make one consider these
30
Q

Tx and course of PMR?

A
  • corticosteroids are DOC (10-20 mg/day) for 2-4 wks, then taper off
  • trial of NSAIDs for 2-4 wks if mild
  • complete or nearly complete resolution of sx is seen in a few days - absence of improvement should cause one to ? dx
  • relapses do occur, more frequent in 1st 1-2 yrs
  • follow ESR or CRP
  • tx for 1-2 yrs is often reqd, sometimes longer
  • watch for corticosteroid adverse effects
  • methotrexate being used in refractory cases
  • methylprednisolone had similar efficacy and fewer adverse effects
31
Q

What is GCA?

A
  • chronic vasculitis of medium and large vessels
  • more common in people older than 50, incidence peaks at 70-80
  • affects women more often 2:1
32
Q

PP of GCA?

A
  • vasculitis of extracranial branches of aorta, spares intracranial branches
  • transmural inflammation leads to intimal hyperplasia - luminal occlusion
  • sxs are due to end organ ischemia
33
Q

Etiology of GCA?

A
  • likely influenced by multiple genetic and enviro factors closely tied to PMR
  • 1 pt may have both: 50% w/ GCA have PMR, 10% w/ PMR have GCA
  • no evidence of autoabs
  • cellular immune response involving T cells, APCs, macrophages
34
Q

Presentation of GCA?

A
  • onset usually gradual but may be abrupt
  • most frequent sx: HA (2/3)
  • usually accompanied by syndrome of systemic inflammation: fever, malaise, wt loss, anorexia (40%)
35
Q

Sxs of GCA?

A
  • HA (68%)
  • jaw claudication (45%)
  • transient visual sxs (16%)
  • fixed visual sxs (14%)
  • CNS abnormalities
  • swallowing claudication/dysphagia (8%)
  • tongue claudication (6%)
  • limb claudication (4%)
36
Q

Clinical signs of GCA?

A
  • wt loss or anorexia (50%)
  • decreased temporal artery pulsations (46%)
  • fever (42%)
  • artery tenderness (27%)
  • erythematous or swollen scalp arteries (23%)
  • large artery bruits (21%)
  • fundoscopic abnormalities (18%)
37
Q

Complications of GCA?

A
  • blindness
  • aortic aneurysms
  • stroke
38
Q

Lab findings of GCA?

A
  • ESR over 50
  • 22% of pts w/ bx-provven GCA have normal ESR
  • therefore, normal ESR doesn’t rule out GCA
  • mild-moderate anemia of chronic disease
  • elevated LFTs (1/3)
39
Q

How do you dx GCA?

A
  • temporal artery bx should be performed in all pts w/ suspected GCA
  • sensitivity of unilateral bx: 88%
  • negative predictive value: 91%
  • bilateral bxs increase sensitivity, may be prudent
  • imaging: MRI/MRA may be used to dx large-vessel GCA. Also may help guide bx by localizing inflammation, other possible imaging modalities include arteriography, US, and PET
40
Q

Tx of GCA?

A
  • glucocorticoids are the est tx
  • initial dose: 40-60 mg prednisone daily
  • slow taper after 2-4 wks if sxs controlled
  • prolonged tx often reqd to avoid relapse
  • IV pulse methylprednisolone (1000 mg IV dailyx 3 days) if vision loss present
  • anti-platelet agents reduce ischemic events - low dose ASA recommended
  • if clinical suspicion is high, tx shouldn’t be delayed for bx
  • temporal artery bx should be peformed as soon as possible after tx is initiated but bx results shouldn’t be affected for several weeks
41
Q

What is Fibromyalgia Syndrome (FMS)?

A
  • clinical syndrome characterized by widespread muscular pain (usually chronic), fatigue and muscle tenderness
  • not an inflammatory condition
  • caused by abnormal sensory processing in the CNS
  • people w/ FMS may be extremely sensitive to pain and other unpleasant sensations
42
Q

Additional sxs w/ FMS?

A
  • poor sleep (almost always(
  • HAs
  • IBS
  • cognitive and memory problems
  • numbness and tingling in fingers and toes
  • irritable bladder
  • TMJ disorder
  • dry eyes and dry mouth
  • morning stiffness
  • anxiety and depression
  • sxs including pain may wax and wane over time
43
Q

What causes FMS?

A
  • unknown
  • abnormally high levels of substance P in spinal fluid in some pts
  • substance P impt in transmission and amplification of pain signals to and from the brain
  • “volume control” is turned up too high in brain’s pain centers
  • familial tendency to develop FMS suggests genetic role
  • can be triggered by physical, emotional or enviro stressors such as car accidents, repetitive injuries and certain diseases
  • pts w/ RA and SLE are more likely to develop FMS
  • lyme disease and OSA have been assoc w/ FMS
  • sleep deprivation w/ disruption of delta-wave sleep (non-REM stage IV) is assoc w/ day-time fatigue and FMS
44
Q

Who gets FMS?

A
  • 1/50 Americans
  • MC in middle aged women
  • men and kids may also develop this
  • pts w/ RA, SLE, and AS have higher incidence
  • women w a family member w/ FMS are more likely to develop it
45
Q

Dx FMS?

A
  • widespread chronic pain (longer than 3 months) and
  • 11/18 reproducible tender pts
    or
    at least 4 of: generalized fatigue, HA, sleep disturbance, neuropsych complaints, numbness/tingling, IBS
    and
    explained by no other condition
    -at least 4 of the following sxs:
  • generalized fatigue
  • HAs
  • sleep disturbance
  • neuropsych complaints
  • numbness, tingling sensations
  • Irritable bowel sxs
  • xrays, blood tests, specialized scans such as nuclear medicine and CT, muscle bxs are all normal
  • objective markers of inflammation such as ESR, CPR are normal
  • must be distinguished from other common diffuse pain conditions such as RA, SLE, hypothyroidism and PMR
46
Q

What are the problems in defining fibromyalgia?

A
  • is it real if no clear pathophysiologic basis?
  • gold std is expert opinion
  • tender pts, sxs are subjective
  • fewer than 11 tender pts?
  • sxs are not clearly defined
  • same dx criteria and dilemma for any illness lacking objective biologic markers (depression, migraine, IBS, CFS - chronic fatigue syndrome)
47
Q

relationship b/t FMS and mood disorders?

A
  • at time of FM dx, mood disorders are present in 30-50%, primarily depression
  • increased lifetime and family hx of mood disorders in FM vs RA
  • fibromyalgia co-aggregates w/ major mood disorder in families
48
Q

Shared features of FM and depression?

A
  • both have strong genetic predisposition and similar co-morbidity
  • similar sleep disturbances
  • similar cognitive disturbances
  • orthostatic features, ANS dysfxn
  • childhood abuse, stress
  • can be debilitating
  • imaging studies - unremarkable
  • neuroendocrine studies: unremarkable
49
Q

Is FMS a medical or psych illness?

A
  • harmful and unproductive argument
  • fruitless quandry to work out what came first
  • for all pts, sxs are real and can be disabling
  • need a dual tx approach targeting both physical and psych sxs
50
Q

Tx guidelines for FMS?

A
  • confirm dx, ID impt sx domains, their severity, and level of pt fxn
  • eval for comorbid medical and psych disorders - assess psychosocial stressors, levels of fitness, barriers to tx - provide education about FMS, may reqr referral to specialist for full eval for psych or sleep clinic
  • give all pts reassurance, give explanation - psychological factors, promote returnt to normal activities, exercise
51
Q

Pharm tx for FMS?

A
  • most pts: med trial (antidepressants, anticonvulsants), CBT, counseling, physical rehab
  • no definitive cure
  • best meds: tx pain and improve sleep disturbance:
    TCAs: amitriptyline, cyclobenzoprine, anticonvulsants - pregabalin, gabapentin, SNRIs - duloxetine, milnacipran
  • NSAIDs uneffective, long actign opioids ineffective
  • benzos (diazepam and clonazepam) may be useful for pts w/ RLS or very severe sleep disturbance who haven’t responded to other therapies
  • trial of additional analgesics such as tramadol
52
Q

Alt therapies for FM?

A
  • alt therapies have been used but not well studied
  • therapeutic massage
  • myofascial release therapy
  • acupuncture
  • pt self management:
    schedule time to relax, including deep breathing and meditation, est routine for going to bed and waking up, aerobic exercise on regular basis
  • avoid inactivity
  • non-pharm tx - trigger pt injections
  • stretching, strengthening
  • self education: arthitis foundation
  • support group
  • CBT
53
Q

FM - prognosis and typical outcome?

A
  • kids and individuals tx in primary care settings and those w/ recent onset of sxs generally have better prognosis
  • FM doesn’t herald onset of systemic disease
  • there is no progressive, structural or organ damage
  • primary care pts more commonly report complete remission of sxs
  • most pts continue to work, but 10-15% are disabled
  • there is often adverse impact on work and leisure activities
  • most pts quality of life improves w/ medical management
54
Q

Who should tx FM?

A
  • more than 80% of visits are to PCP
  • currently, 16% of FM visits are to rheumatologists
  • the ACR suggest that rheumatologists serve as consultants
  • other specialists should include mental health professionals, physiatrists and pain management experts