Seronegative Spondyloarthropathies (SpA) Flashcards

1
Q

What are the different types of seronegative spondyloarthropathies?

A

reactive arthritis (ReA)

ankylosing spondylitis (AS)

psoriatic arthritis (PsA)

spondylitis associated with inflammatory bowel disease

undifferentiated spondyloarthropathy

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2
Q

What are the environmental triggers of SpA reactive arthritis?

A

enteric infections - shigella, salmonella, yersinia, campylobacter, clostridium

urogenital infection (urethritis) - chlamydia t.

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3
Q

What is HLA-B27?

A

an antigen presenting protein

interacts with receptor on NK and/or T cells

leads to cytokine production/memory or effector function

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4
Q

What are common features of seronegative spondyloarthropathies?

A

HLA-B27 positive

enthesopathy/enthesitis (inflammation of tendon sheaths at tendon insertions into bone)

sacroiliitis (tender SI joints)

conjunctivitis

anterior uveitis/iritis

mucocutaneous disease

arthritis/synovitis

absence of rheumatoid factor (“seronegative” spondyloarthropathy)

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5
Q

What is inflammatory enthesopathy?

A

subchondral bone inflammation and resorption

periosteal new bone formation

often quite painful, especially in the morning

can result in reactive bone formation

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6
Q

What is sacroiliitis?

A

inflammation at the sacroiliac joints

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7
Q

What are the eye diseases in seronegative spondyloarthropathies?

A

anterior uveitis

conjunctivitis

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8
Q

What is acute anterior uveitis?

A

swelling and irritation fo the anterior portion of the uveal tract (middle layer of the eye)

ciliary injection and irregularity of the pupil

painful, abnormal vision, photophobia

emergency - need to refer to opthalmology within 24h

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9
Q

What is conjunctivitis?

A

erythema and exudate on the bulbar and palpebral/tarsal conjunctivae

minimal pain and photophobia and normal vision

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10
Q

What are the mucocutaneous diseases in seronegative spondyloarthropathies?

A

oral ulcers (painless, superficial erosion)

sharply demarcated erosion of the hard palate (most common)

onycholysis

meatitis

circinate balanitis

keratoderma blennorrhagicum

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11
Q

What is onycholysis?

A

separation of the distal portion of the nail from nailbed

accumulation of subungual material and periungual scaling

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12
Q

meatitis and circinate balanitis

A

shallow, painless ulcers on the glans penis

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13
Q

What is keratoderma blennorrhagicum

A

discrete, scaly, and plaque-like skin lesions on the hands and feet

crusts = collection of debris on the skin surface

pustules = a small collection of pus in the top layer of skin (epidermis) or beneath it in the dermis

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14
Q

additive vs. migratory joint pattern for articular disease

A

additive pattern - joints become involved in an additive fashion, a knee then an ankle as opposed to a migratory pattern

migratory pattern - a knee hurts then gets better followed by another joint as in rheumatic fever

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15
Q

What is the presentation of inflammatory arthritis in seronegative spondyloarthropathies?

A

asymmetric oligoarthritis

inflammatory arthritis

tumor necrosis factor in human SI joint infiltrate

MRI improvement with infliximab (monoclonal antibody vs. TNF-alpha) - also reduced inflammation

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16
Q

What are the important features of reactive arthritis?

A

acute inflammatory arthritis following GI or GU infection

affects men more than women - self-limited, may be recurrent or chronic

relapses in 15%

chronic destructive course in 15%

mortality infrequent

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17
Q

What are the articular features of reactive arthritis?

A

additive, asymmetric mono- or olig-arthritis involving more commonly large lower extremity joints

enthesopathy (diffusely swollen digits, “sausage toe” or finger)

enthesitis (heel pain at tendon insertion)

inflammatory low back pain (sacroiliitis)

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18
Q

What are the extraarticular features of reactive arthritis?

A

keratoderma blennorrhagicum

mucosal lesions

thickened, opacified nails

conjunctivitis and acute anterior uveitis

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19
Q

Whis the pathophysiology behind reactive arthritis?

A

trigger is often a GI or GU infection

synovial fluid cultures negative

antigens from inciting organisms detected within intrasynovial cells

T-cells within synovial fluid with antigenic specificity for specific microbial trigger

antibiotic therapy disappointing

molecular mimicry

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20
Q

What are the laboratory features of reactive arthritis?

A

anemia - increased ESR (sed rate), CRP (C-reactive protein) and platelet count

positive HLA-B27

rheumatoid factor and ANA negative

inflammation in the synovial fluid (WBC 2,000-30,000) but sterile

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21
Q

What is the clinical course and prognosis of reactive arthritis?

A

usually runs a self-limited course from 3-12 months

relapse occur in up to 15% of patients

another 15% or more have chronic destructive disease

22
Q

What is the treatment for reactive arthritis?

A

NSAIDs and corticosteroids

DMARDs - sulfasalazine (peripheral, not axial disease such as sacroiliitis)

  • biologics such as ethanercept, infliximab, adalimumab

antibiotic therapy - indicated only for acute infections and has no long-term use

physical therapy - very important, every patient gets referred

23
Q

What are the common features of psoriatic arthritis (PsA)

A

men = women

affects both peripheral and axial skeleton (facet/apophyseal joints) assymetrically

dactylitis/sausage digits (more commonly toes)

6-30% of people with psoriasis develop PsA

nail pits, onycholysis

enthesitis and arthritis mutlans (completely destructive arthritis)

commonly sacroiliac and DIP invovement

24
Q

What is the pathophysiology behind PsA?

A

may be associated with streptococcal pharyngitis and tonsillitis in children

Koebner phenomenon - psoriatic lesions arising at sites of trauma

deep Koebner phenomenon - psoriatic arthritic changes in previously injured joint

the synovium more vascular than RA

association with HLA-B27 and others

25
What is the clinical course and prognosis of PsA?
67% of patients have joint erosions at the time of initial presentation to clinic disease progression over time with an increased incidence of polyarthritis and an increase in joint damage both clinically and radiographically poor prognosis related to polyarticular disease and high medication level at presentation
26
What are the features of axial articular involvement in PsA?
sacroiliitis with asymmetric joint involvement apophyseal (facet) joint involvement with skip lesions syndesmophytes may be seen spine/back pain and stiffness is not as severe compared to AS
27
What are the features of extraarticular manifestations in PsA?
skin disease - psoriasis is a prerequisite, but may come after joint disease or occur at the same time nail dystrophy - thickened, opacified onycholysis and pitting hyperkeratosis (hypertrophy of skin under the nail) conjunctivitis and acute anterior uveitis/iritis is more common in patients with axial disease uretheritis is common as well as non-specific colitis or subclinical gut disease dilation of base of aortic arch resulting in aortic insufficiency
28
What is the treatment for PsA?
NSAIDs and corticosteroids for symptom relief MTX, sulfasalazine for peripheral TNFalpha inhibitors for axial involvement
29
What is fingernail pitting pathomneumonic of?
psoriasis
30
What is the presentation of psoriatic arthritis?
additive, asymmetric pattern, oligoarticular or polyarticular bluish/purple discoloration over inflamed joints skin disease typically precedes joint disease by up to 10 years 10% will present with joint disease first no correlation between severity of skin and joint disease, although presence of joint disease is more likely with severe skin disease nail findings associated with joint disease
31
What is ankylosing spondylitis?
chronic, systemic inflammatory arthritis with both axial and peripheral involvement - especially sacroiliac joints and spine may have extra-articular manifestations (skin, eye, bowels, and heart) strongly associated with HLA-B27 (~90%) ~80% male, young adults
32
What is the pathophysiology of ankylosing spondylitis?
can be due to Klebsiella aeruginosa bacterial infection, but no conclusive evidence strong association with HLA-B27 positivity inflammatory disease
33
What is the presentation of ankylosing spondylitis?
often a late diagnosis, although symptoms typically begin in 20s - children may present with peripheral disease presenting symptoms: - inflammatory back pain and stiffness - sacroiliitis - oligoarthritis - enthesitis - systemic symptoms (fatigue, imparied sleep)
34
What is the dagger sign?
found in ankylosing spondylosis ossification of spinous ligaments syndesmophytes (calcium deposites in posterior spinous ligament) are diffuse
35
What is bamboo spine?
found in ankylosing spondylitis presence of bridging syndesmophytes - intervertebral discs and spinal ligaments become calcified
36
What are the presenting symptoms of inflammtory bowel disease related arthritis?
inflammatory back pain and stiffness, ascend up the spin and results in loss of lumber lordosis sacroiliitis oligoarthritis enthesitis dactylitis systemic symptoms (fatugue, impaired sleep, fever, weight loss, loss of libido) dagger sign and bamboo spine \*\*also has diarrhea, which separates from ankylosing spondylitis
37
What is the classic presentation of ankylosing spondylitis?
young man with inflammatory back pain (better with motion) and worse at night, and insidious in onset pain is worsened by rest and improved with exercise
38
What are the extraarticular manifestations of ankylosing spondylitis?
conjunctivitis, acute anterior uveitis/iritis - not related to joint flares dilatation of base of aortic arch resulting in aortic insufficiency conduction abonormalities associated with peripheral joint involvemen inflammatory bowel disease present subclinically in 60% of patients 1% develop progressive upper lobe fibrosis or restrictive lung disease spinal fractuers, deformities, and disc disease can impinge nerves psoriatic lesions in 10-25%
39
type I peripheral arthritis
pauciarticular, large joint arthritis (self-limited, flares with IBD activity)
40
type 2 peripheral arthritis
polyarticular arthritis typically symmetrical involvement of small joints of hands independent of IBD activity
41
What are the treatment options for SpA?
**exercise/physical therapy (most important)** NSAIDs corticosteroids sulfasalazine methotrexate TNFalpha antagonists
42
What is ankylosis associated with inflammatory bowel diseases?
accompanies ulcerative colitis (UC) and Chron's disease (CD) periphearl joint pain/disease typically mirrors the gut disease, but this is not true for axial joint disease colectomy for UC can lead to complete resolution of peripheral joint disease although this is not the case for CD
43
What are the clinical and laboratory features of ankylosis associated with inflammatory bowel disease?
sacroiliitis with symmetric joint involvement like AS asymmetric oligoarthritis that commonly involves the lower extremity (hips, knees, ankles, MTP are most commonly affected) enthesitis conjunctivitis, acute anterior uveitis/iritis erythema nodosum (panniculitis, pyoderma gangrenosum, oral ulcers
44
What is the treatment for ankylosis associated with inflammatory bowel disease?
NSAIDs or corticosteroids sulfasalasine etanercept infliximab adalimumab
45
Etanercept (Enbrel)
TNFalpha inhibitor useful for skin, jjoint, and extraarticular manifestations of PsA administered SC
46
Infliximab (Remicade)
chimeric (part mouse) monoclonal antib-TNFalpha antibody that is useful for skinm joint, an extraarticular manifestations of PsA has been shown to prevent radiographic progression of PsA administered IV
47
Adalimumab (Humira)
fully human anti-TNFalpha monoclonal antibody administered SC
48
Alefacept (Amevive)
fully human fusion protein that blocks interaction between LFA-3 on the antigen-presenting cell and CD2 on the T-cell or by attracting natural killer T-cells to interact with CD2 to yield apoptosis of particular T-cell clones useful for PsA and is administered IM
49
Pamidronate
IV bisphosphonate that has been shown to improve bone metabolism, inflammation, and immune regulation
50
Thalidomide
glutamic acid derivvative that produces anti-inflammatory and immunomodulatory effects , including a reduction of TNFalpha production
51
Sulfasalazine (Azulfidine)
drug that is mtabolized to sulfapyridine, which is thought to have antibiotic porperties the other metabolite, 5-aminosalicylic acid (5-ASA) is thought to have the drug's anti-inflammatory therapeutic effect