Kinder CIS part 3 Flashcards

1
Q

Mixed Connective Tissue Disease

A
Overlap syndrome with clinical features of SLE, Scleroderma, and Polymyositis
*** + Anti-U1-RNP
80% are women
Peak ages teens to twenties
50% Hand edema and synovitis
33% Myositis
50% Decreased esophageal motility
50% fibrosing alveolitis
20% Pulmonary Hypertension
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2
Q

Mixed Connective Tissue Disease skin manifestations and some other

A
  • diffuse swelling of the hands classicallly described
Skin manifestations
- Sclerodactyly
- Scleroderma
- Calcinosis
- Telangiectasias
- Photosensitivty
- Malar rash
- Gottron rash
60% Pleruopericarditis
50% Sicca symptoms
25% Trigeminal neuralgia
25% Kidney involvement (membranous nephropathy)
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3
Q

Mixed Connective Tissue Disease symptoms

A

Raynaud phenomenon
* Diffuse swelling of the hands
Lupus or dermatomyositis like rashes
Diffuse systemic sclerosis-like skin changes and ischemic necrosis and ulceration of the fingertips
Polyarthralgias
Arthritis in 75%
Sometimes erosive arthritis and deformities
Proximal muscle weakness
Membranous nephropathy in 25% of patients – typically mild
Interstitial Lung Disease
Pulmonary Hypertension is a major cause of death
Heart failure
Sjögren syndrome

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4
Q

Mixed Connective Tissue Disease Labs

A

Elevated ANA in a speckled pattern
Elevated Anti-U1 RNP
Anti-Sm and anti-DS DNA are negative
RF and ESR may be elevated

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5
Q

Mixed Connective Tissue Disease Prognosis

A

10-year survival is 80%
Symptoms of scleroderma and polymyositis have worse prognosis
Causes of death include pulmonary hypertension, renal failure, MI, colonic perforation, disseminated infection, and cerebral hemorrhage

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6
Q

Mixed Connective Tissue Disease treatment

A

Based on the specific organ system involvement

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7
Q

Relapsing Polychondritis

A

Immune mediated condition

Auricular chondritis

Inflammation of other cartilaginous areas

  • Nose, joints, trachea, ribcage, and airways
  • Proteoglycan rich areas such as eyes and heart valves

Associations

  • Systemic vasculitis
  • Connective tissue disease
  • Myelodysplastic syndrome
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8
Q

Relapsing Polychondritis dx and associations

A

30% associated with other diseases

  • Systemic vasculitis – Wegener granulomatosis
  • Connective Tissue Disease – RA or SLE
  • Myelodysplastic Syndrome

Diagnosis

  • Cartilaginous inflammation in typical Areas
  • – Auricular, nasal bridge, costochondral joints
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9
Q

relapsing polychondritis clinical findings- ear and nose

A

Ear::
Inflammation and pain that spares the earlobe
Cartilaginous areas are erythematous and tender
Swelling of external canal can cause conductive earing loss
Sensorineural hearing loss of unknown cause

Nose:
Tenderness of the nasal bridge
Epistaxis
Saddle-nose deformity
Nasal septal perforation
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10
Q

relapsing polychondritis clinical findings-trachea, bronghi, airways, eyes

A

Trachea:
Subglottic stenosis
Tenderness to palpation of the anterior cervical trachea, thyroid cartilage, and larynx

Bronchi and Airways:
May mimic asthma
Mucociliary dysfunction leads to infections

Eyes:
Scleritis – pain and photophobia
Episcleritis and Conjunctivitis

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11
Q

relapsing polychondritis clinical findings- heart, joints, skin

A

Heart:
Aortic and Mitral Regurgitation
Cardiac conduction abnormalities

Joints:
Intermittent, migratory oligoarthritis
Occasionally symmetric polyarticular

Skin:
Aphthous ulcers, nodules, purpura, papules, and sterile pustules
MAGIC syndrome
Mouth and genital ulcers with inflamed cartilage

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12
Q

relapsing polychondritis clinical findings-kidney and labs

A

Kidney
- Pauci-immune glomerulonephritis

Laboratory

  • No specific findings
  • Mild normochromic, normocytic anemia
  • Cytopenias
  • – Myelodysplasia
  • ANA and RF are negative
  • Complement normal
  • ANCA +
  • – Wegener granulomatosis
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13
Q

relapsing polychondritis - Testing

A

CT
- valuation of airways

Fiberoptic layngoscopy

Pulmonary Function Testing
- Determine extrathoracic verses intrathoracic obstruction

Biopsy
- Cautiously, can lead to complete airway obstruction

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14
Q

relapsing polychondritis - Differential Diagnosis

A

Aural Chondritis- Infection

Nasal inflammation with Saddle Nose deformity:
Wegener granulomatosis
Crohn disease
Syphilis
Leprosy
Lymphoma
Leishmaniasis
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15
Q

relapsing polychondritis - Complications

A

Cauliflower ear
Saddle-nose deformities
Extrathoracic airway obstruction can lead to tracheostomy
Postobstructive infections
Cardiac valvular regurgitation can lead to valve replacement

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16
Q

relapsing polychondritis - Treatment

A

Glucorticoids are treatment of choice for reduction of cartilagenous inflammation

Others
- Dapsone, colchicine, and NSAID to limit glucocorticoid use
Methotrexate – most common glucocorticoid sparing agent
Cyclophosphamide for glomerulonephritis

Airway disease

  • Direct steroid injection
  • Mechanical interventions
  • Stenting
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17
Q

Spondylarthritis types

A

Ankylosing spondylitis
Psoriatic arthritis
The arthritis of inflammatory bowel disease
Reactive Arthritis

Entheses – tendons, fascia, and ligaments insert into bone

  • Heel pain – achilles tendon
  • Foot pain – plantar aponeurosis
  • Digit – flexor or extensor tendons - dactylitis or sausage digit
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18
Q

Ankylosing Spondylitis

A
Inflammatory back pain in young adults
Radiographic evidence of sacroiliitis
Reduced spinal mobility, especially lumbar flexion
Anterior uveitis
HLA-B27 increased relative risk
Positive family history
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19
Q

Ankylosing Spondylitis and HLA B-27

A

85% of patients have HLA-B27
HLA-B27 present in 8-10% of white Americans with 0.1-0.2% having the disease

Child of an HLA B-27 positive parent with spondylitis has about a 10% chance of developing disease

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20
Q

Ankylosing Spondylitis Typical presentation

A

Young male 3:1
Pain/stiffness in buttocks, low back, and chest wall
Worse with rest and better with exercise
Reduced spinal mobility
Family History of ankylosing spondylitis
Oligoarticular/monoarticular large joint involvement (Hips and Shoulders)
History of eye pain, redness, blurry vision – anterior uveitis

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21
Q

tests for ankylosing spondylitis

A

Schober Test, Chest expansion, Occiput-to-wall measurement

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22
Q

Ankylosing Spondylitis- other symptoms re: arthritis and enthesitis and eyes

A

Peripheral Arthritis
Hips and Shoulders in 50% of patients

Enthesitis

  • Leads to spinal fusion and ankylosis
  • Costosternal joints – chest wall pain
  • Achilles tendon insertion
  • Plantar aponeurosis

Anterior uveitis in 30% of patients
- Abrupt onset, intense pain, and photophobia

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23
Q

Ankylosing Spondylitis cardiac and neurologic symptoms

A

Cardiac:
Less than 5% of patients
1st degree AV block
Higher degree blocks can require pacemaker
Aortic regurgitation due to inflammatory thickening of the aortic valve and root
Valve replacement may be required within 1-2 years of onset of murmur

Neurologic:
Cauda equina syndrome – rare
Atlantoaxial subluxation

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24
Q

Ankylosing Spondylitis renal and lung stuff

A

Renal:
Secondary renal amyloidosis
After many decades of persistent inflammatory disease
Proteinuria and nephrotic syndrome

Lung:
Apical pulmonary fibrosis
Rare and of no clinical consequence
Restrictive pattern on PFT due to costovertebral joint involvement and ankylosis of the thoracic spine

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25
Q

Ankylosing Spondylitis labs

A

ESR and CRP elevated in 50% of patients and more associated with peripheral disease
RF negative
ANA negative
Serum complement levels normal

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26
Q

Ankylosing Spondylitis Imaging

A

Sacroiliac Joints - Bilateral

  • Early radiographic changes
  • — Sclerosis on the iliac side of the SI joint margins
  • Erosions with pseudo widening of the SI joints
  • Eventual fusion
  • MRI is more sensitive for detecting early disease when the plain radiograph may still be normal

Spine

  • “shiny corners” or Romanus lesions
  • syndesmophytes
27
Q

Ankylosing Spondylitis Diagnosis

A

Modified New York Diagnostic Criteria

Clinical

  • Low back pain and stiffness for more than 3 months that improves with exercise but is not relieved with rest
  • Limitation of motion of the lumbar spine in the sagittal and frontal planes
  • Limitation of chest expansion to 2.5 cm or less, measured at the level of the 4th intercostal space

Radiographic
- Sacroiliitis

Assessment of Spondyloarthritis International Society Classification for Axial Spondyloarthritis in patients with back pain for ≥ 3 months and age at onset of < 45 years

Sacroiliitis on imaging + ≥ 1 Spondyloarthritis features
OR
HLA-B27 + ≥ 2 Spondyloarthritis features

28
Q

Ankylosing Spondylitis Treatment

A

Physical Therapy

  • Maintain an erect spine
  • Promote chest expansion
  • Swimming is an excellent exercise

NSAID
- If first drug not effective, should trial a second

Anti-TNF agents

  • Active Disease
  • Not responsive to two NSAIDs

Prognosis - Excellent

29
Q

Inflammatory Bowel Disease Associated Spondyloarthritis

A

20% of patients with Crohn disease and Ulcerative Colitis
Most commonly presents with peripheral arthritis that correlates with bowel diseases activity
Can present with spondylitis that progresses in the absence of active bowel disease – less common
Peripheral Arthritis is migratory and rarely erosive
Spondylitis usually more benign than ankylosing spondylitis
May present with sacroiliitis without spondylitis
Cutaneous Manifestations
- Erythema nodosum and pyoderma gangrenosa
No male predominance

30
Q

IBD Associated Spondyloarthropathy Treatment

A

Treatment of the underlying IBD
- Sulfasalazine

NSAID – avoided
- May exacerbate IBD

Anti-TNF agents
- If sulfasalazine has failed

31
Q

Reactive Arthritis

A

Inflammatory arthritis triggered by antecedent gastrointestinal or genitourinary infections
Asymmetric oligoarthritis most commonly affecting the lower extremities
Enthesitis and dactylitis

Extra-articular manifestations:
- Conjunctivitis, anterior uveitis, urethritis, circinate balanitis, oral ulcers, and keratoderma blennorrhagicum

Adults between 20-40 years of age
Post GI infection affects men and women equally
Post GU subtype affects men 9:1
Reiter Syndrome
- Peripheral arthritis
- Conjunctivitis
- Urethritis/Cervicitis
32
Q

Reactive Arthritis and diseases

A
Develops 1-4 weeks after gastroenteritis or sexually transmitted disease
Shigella
Salmonella
Campylobacter
Yersinia
Chlamydia
HIV – occasionally
25% of patients have no symptoms of infection
30-50% HLA-B27
33
Q

Reactive Arthritis Clinical Findings

A

Articular
- Asymmetric oligoarthritis of peripheral joints
Knees, ankles, and feet
- Inflammatory low back pain in 50%
- Unilateral sacroiliitis in 25% of patients

Enthesitis

  • Achilles tendon
  • Plantar Fascia
  • Pelvic Bones

Dactylitis

  • Sausage digit, toes more common than fingers
  • Reactive arthritis and Psoriatic Arthritis

Mucocutaneous Lesions

  • Circinate balanitis
  • Keratoderma blennorrhagicum
  • Urethritis/Cervicitis
  • Aphthous ulcers
  • Onchodystrophy

Ocular Inflammation

  • Conjunctivitis, iritis, scleritis, episcleritis, and keratitis
  • 25% of cases
  • Intermittent eye pain and visual changes

Heart – rare

  • Aortitis
  • Valvular heart disease – Aortic regurgitation
  • Complete Heart Block

Synovial Fluid Analysis

  • 5000-50,000 cells/mcL
  • Sterile
34
Q

Reactive Arthritis Treatment

A

NSAIDs treatment of choice for articular manifestations
Treat active infection with antibiotics
Sulfasalazine
Methotrexate

35
Q

Psoriatic Arthritis

A

Chronic inflammatory arthritis associated with skin and nail psoriasis

Symmetric polyarthritis or asymmetric oligoarthritis of peripheral joints
- Frequent DIP involvement

Spondylitis and enthesitis may occur

History of psoriasis or family history of psoriasis

Dactylitis and nail dystrophy
RF negative

Erosion and osteolytic destruction of interphalangeal joints and juxta-articular new bone formation

36
Q

Psoriatic Arthritis - who gets it

A

Mean age of onset 30-55 years old
Men and women equally affected
20-30% of adults with psoriasis
Group A streptococcal infections implicated in guttate psoriasis
HIV associated with psoriasis and psoriatic psoriasis
Physical trauma implicated
Arthritis can proceed psoriasis in 15-20% of cases

37
Q

Psoriatic Arthritis Clinical Findings- ARticular

A

Symmetric polyarthritis
Asymmetric oligoarthritis of the hands and feet
DIP joints in asymmetric fashion
Other joints
- Knees, hips, and sternoclavicular joints
Joint deformities, juxta-articular erosions, joint space narrowing, and bone ankylosis
Arthritis mutilans
- Complete subluxation and telescoping

38
Q

Psoriatic Arthritis Clinical Findings- fomgers amd temdpms

A

Dactylitis

  • Sausage digit
  • 30-50% of psoriatic arthritis patients
  • Toes more often than fingers

Enthesitis

  • Inflammation occurring at the site of tendon insertions into bone
  • Achilles tendon
  • Plantar Fascia
  • Pelvic bones
39
Q

Psoriatic Arthritis Clinical Findings- skin, nails, spondylarthropathy

A

Skin and Nail Changes
- Psoriasis lesions
Can be subtle and degree does not correlate with arthritis
Hairline, scalp, external auditory canal, periumbilical area, and gluteal cleft

  • Nails
    Ridging, pitting, onycholysis, and hyperkeratosis
    Nail of DIP joint affected

Spondylarthopathy

  • Less common than peripheral
  • Unilateral sacroiliac involvement
  • Cervical spine common
  • – Atlaontoaxial instability
  • Affects vertebra in a noncontiguous fashion
40
Q

Psoriatic Arthritis Clinical Findings- Labs

A

20% with hyperuricemia

41
Q

Patterns of Arthritis in Psoriatic Arthritis

A
1.Asymmetric 
Oligoarthritis 
2. SI 
3. Symmetric 
polyarthritis 
4. DIP 
5. Opera glass
“arthritis mutilans”

** pencil in cup = arthritis mutilans

42
Q

Ankylosing Spondylitis Treatment

A

NSAID

DMARD
Methotrexate, sulfasalazine, azathioprine, hydroxychloroquine, and cyclosporine

Biologics
Anti-TNF

Glucocorticoids

  • Intra-articular injections effective for 1-2 joints
  • Systemic may lead to an exacerbation of severe pustular psoriasis on taper
43
Q

Septic Arthritis

A

Acute onset of painful, warm, and swollen joint
Usually monoarticular
Typically affects large weight-bearing joints
Cell count > 50,000 cells/mcL with over 80% neutrophils
Positive culture
Staphylococcus aureus is the most common etiology in native joints

44
Q

Septic Arthritis- medical emergency

A

25-50% rate of permanent joint damage
Fatality Rate 11% in monoarticular bacterial arthritis

Risk Factors for bacterial arthritis

  • Chronic arthritis
  • Prosthetic joints
  • Parenteral drug use
  • Extremes of age
  • Diabetes
  • Immunocompromised conditions
45
Q

Septic Arthritis pathogenesis

A

Pathogenesis

  • Hematogenous Spread in more than 50% of cases
  • Direct inoculation
  • Spread from adjacent bony or soft tissue infections

Skin infections are the most common predisposing infections

Other infections
- Transient bacteremia from respiratory, GI, or GU can also cause infections

46
Q

Septic Arthritis Clinical Manifestations

A

Abrupt onset of painful, warm, and swollen joint
Signs of Septic Arthritis
- Joint effusion
- Moderate to severe joint tenderness to palpation
- Marked restriction of both passive and active range of motion

Monoarticular Arthritis is considered septic arthritis until proven otherwise

Polyarticular septic arthritis is more common in patients with rheumatoid arthritis, connective tissue diseases or overwhelming sepsis

47
Q

joints of septic arthritis

A

mostly knee
hip
shoulder

also wrist, ankle, elbow, small joints (rare)

Sternoclavicular Joint
21% of cases associated with IV drug abusers
Less common risk factors include diabetes, trauma, infected central lines, and other distant infections

48
Q

Septic Arthritis Clinical Manifestations

A

Fever in 60-80% of patients
Shaking chills in 20% of those with fever

Symptoms of antecedent infection

  • Cough
  • GI symptoms
  • Dysuria

50% of patients will have an identified source of infection
- Pneumonia, otitis, bronchitis, pharyngitis, cutaneous, GI, or GU

49
Q

Septic Arthritis labs

A

Laboratory

  • Elevated white count
  • ESR and CRP elevated
  • Bacteremia in 40-50%

Synovial Fluid Analysis

  • Cloudy
  • White count > 50,000 WBC/mcL in 50-70% of cases
  • Gram stain positive in 50-75%
  • Culture positive in 70-90%
  • Micro Staph aureus in 60-70% of cases
50
Q

septic arthritis organisims

A

mostly staph auerus
some strep (A)
Gram neg bacilli

rarely anaerobes, staph epidermidis

51
Q

Septic Arthritis Imaging

A
Helpful for joints that are difficult to palpate and aspirate
Hip
Shoulder
Sternoclavicular
SI joints
52
Q

Septic Arthritis Treatment

A

Drainage

  • Initially performed at the time of synovial fluid analysis
  • Arthroscopic lavage, debridement, and drain insertion
  • Open surgical debridement may be necessary in some circumstances

IV antibiotics

  • Most treated for 4 week minimum combination of IV and oral
  • Some more difficult infections treated with 4 weeks IV antbiotics

Progressive joint mobilization

53
Q

Septic Arthritis Complications

A

Osteomyelitis
Persistent or recurrent infection

Marked decrease in joint mobility

Ankylosis

Persistent pain

70-85% of patients with group A Streptococcal infections recover without residual symptoms

50% of patients with Staphylococcal aureus or gram-negative rods have residual joint damage

Patients with rheumatoid arthritis and polyarticular infection have a survival rate less than 50%

54
Q

finkelstein tests for what?

A

de quervain’s tendonitis

55
Q

Gonococcal Arthritis

A

Sexually active young person without prior joint disease

Typical Triad

  • Polyarthritis
  • Tenosynovitis
  • Dermatitis

Synovial fluid Gram stain and cultures often negative

Urethral, cervical, pharyngeal, and rectal testing for Neisseria gonorrhoeae
- Positive in up to 90% of cases

56
Q

Gonococcal Arthritis - who gets it

A

Most common cause of acute septic arthritis in young sexually active persons in the United States
1 day to 2 months after sexual contact
25% manifest GU or pharyngeal symptoms
Fevers, chills, and migratory polyarthralgias
Progresses to monoarthritis or polyarthritis
- Knees, ankles, or wrists
Tenosynovitis in 2/3 of patients
- Dorsum of hand, wrist, ankle, or knee
Skin lesions

57
Q

Gonococcal Arthritis Labs

A

Blood cultures
- Positive in 20-30%

Synovial Fluid Analysis

  • Cell count 30,000-60,000 white blood cells/mcL
  • Gram stain positive in 25%
  • Culture positive in 20-50%

Urethral, Cervical, pharyngeal and Rectal specimens
- Positive in up to 90% of untreated patients

58
Q

Gonococcal Arthritis Treatment

A

Drainage

Antibiotics

  • IV cephalosporin antibiotic for 2-4 days followed by oral therapy for 7-10 days
  • Chlamydia should be treated also with Azithromycin or Doxycycline
  • Sex partners should be treated

Prognosis
Excellent with treatment

59
Q

Prosthetic Joint Infections

A

Prosthetic joints are at increased risk for infection

Early infection (under 4 weeks) after implantation or infections secondary to hematogenous seeding present with fever, pain, warmth, and swelling
- Often can be treated with joint salvage

Late infection (>4 weeks) from low virulence organisms introduced at the time of surgery have insidious onset of pain and often don’t have other signs of infection

  • Often require joint replacement
  • Coagulase negative staphylococci, Propionibacterium acnes, diphtheroids
60
Q

Prosthetic Joint Infections- early vs late

A

Early Prosthetic Joint Infections (< 4 weeks)

  • Virulent organisms
  • Classic symptoms
  • – Fever, pain, erythema, effusion, and warmth beyond the post-operative period

Late Prosthetic Joint Infections (> 4 weeks)

  • Less virulent organisms
  • Indolent course
  • – Increasing pain, implant loosening, sometimes drainage
  • – Usually no fever or leukocytosis
61
Q

Prosthetic Joint Infections Labs

A

White Count

  • Elevated in early infection
  • May be normal in late infection

Blood Cultures
- May be positive in early infections

Synovial Fluid Analysis
WBC count > 1700 WBC/mcL

Neutrophil Percentage > 65%

Gram stain
- Positive stain is diagnostic, but negative stain does not rule out infection

Culture
- More frequently positive in early infection

Microbiology

  • Early – Staphycolococcus aureus, coag-negative staphylococcus
  • Late – coagulase negative staphylococcus, P acnes, or diphtherioids
  • Surgical debridement with culture of periprosthetic tissue
62
Q

Prosthetic Joint Infection Imaging

A

Serial x-rays

  • New subperiosteal bone growth
  • Transcortical sinus tracts
  • Radiolucency at the bone-cement interface to suggest joint loosening
63
Q

Prosthetic Joint Infection Treatment- late vs early

A

Early Infection
- Prosthesis Salvage
Surgical debridement
Antibiotic for 3-6 months
— Oral Ciprofloxacin/Rifampin for 3-6 months in culture proven Staphylococcus aureus infection
— Rifampin penetrates tissues and biofilms

Late Infection
- Two stage exchange
Prosthesis removed, area debrided, and cement spacer inserted
Antibiotics for 6 weeks
Revision arthroplasty performed
64
Q

Prosthetic Joint Infections - complications

A

Prosthesis failure
- Even after successful treatment, these revised joints often require early replacement compared to non-infected joints

Recurrent infection