Kinder CIS part 3 Flashcards
Mixed Connective Tissue Disease
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
Mixed Connective Tissue Disease skin manifestations and some other
- 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)
Mixed Connective Tissue Disease symptoms
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
Mixed Connective Tissue Disease Labs
Elevated ANA in a speckled pattern
Elevated Anti-U1 RNP
Anti-Sm and anti-DS DNA are negative
RF and ESR may be elevated
Mixed Connective Tissue Disease Prognosis
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
Mixed Connective Tissue Disease treatment
Based on the specific organ system involvement
Relapsing Polychondritis
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
Relapsing Polychondritis dx and associations
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
relapsing polychondritis clinical findings- ear and nose
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
relapsing polychondritis clinical findings-trachea, bronghi, airways, eyes
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
relapsing polychondritis clinical findings- heart, joints, skin
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
relapsing polychondritis clinical findings-kidney and labs
Kidney
- Pauci-immune glomerulonephritis
Laboratory
- No specific findings
- Mild normochromic, normocytic anemia
- Cytopenias
- – Myelodysplasia
- ANA and RF are negative
- Complement normal
- ANCA +
- – Wegener granulomatosis
relapsing polychondritis - Testing
CT
- valuation of airways
Fiberoptic layngoscopy
Pulmonary Function Testing
- Determine extrathoracic verses intrathoracic obstruction
Biopsy
- Cautiously, can lead to complete airway obstruction
relapsing polychondritis - Differential Diagnosis
Aural Chondritis- Infection
Nasal inflammation with Saddle Nose deformity: Wegener granulomatosis Crohn disease Syphilis Leprosy Lymphoma Leishmaniasis
relapsing polychondritis - Complications
Cauliflower ear
Saddle-nose deformities
Extrathoracic airway obstruction can lead to tracheostomy
Postobstructive infections
Cardiac valvular regurgitation can lead to valve replacement
relapsing polychondritis - Treatment
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
Spondylarthritis types
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
Ankylosing Spondylitis
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
Ankylosing Spondylitis and HLA B-27
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
Ankylosing Spondylitis Typical presentation
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
tests for ankylosing spondylitis
Schober Test, Chest expansion, Occiput-to-wall measurement
Ankylosing Spondylitis- other symptoms re: arthritis and enthesitis and eyes
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
Ankylosing Spondylitis cardiac and neurologic symptoms
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
Ankylosing Spondylitis renal and lung stuff
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
Ankylosing Spondylitis labs
ESR and CRP elevated in 50% of patients and more associated with peripheral disease
RF negative
ANA negative
Serum complement levels normal
Ankylosing Spondylitis Imaging
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
Ankylosing Spondylitis Diagnosis
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
Ankylosing Spondylitis Treatment
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
Inflammatory Bowel Disease Associated Spondyloarthritis
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
IBD Associated Spondyloarthropathy Treatment
Treatment of the underlying IBD
- Sulfasalazine
NSAID – avoided
- May exacerbate IBD
Anti-TNF agents
- If sulfasalazine has failed
Reactive Arthritis
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
Reactive Arthritis and diseases
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
Reactive Arthritis Clinical Findings
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
Reactive Arthritis Treatment
NSAIDs treatment of choice for articular manifestations
Treat active infection with antibiotics
Sulfasalazine
Methotrexate
Psoriatic Arthritis
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
Psoriatic Arthritis - who gets it
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
Psoriatic Arthritis Clinical Findings- ARticular
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
Psoriatic Arthritis Clinical Findings- fomgers amd temdpms
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
Psoriatic Arthritis Clinical Findings- skin, nails, spondylarthropathy
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
Psoriatic Arthritis Clinical Findings- Labs
20% with hyperuricemia
Patterns of Arthritis in Psoriatic Arthritis
1.Asymmetric Oligoarthritis 2. SI 3. Symmetric polyarthritis 4. DIP 5. Opera glass “arthritis mutilans”
** pencil in cup = arthritis mutilans
Ankylosing Spondylitis Treatment
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
Septic Arthritis
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
Septic Arthritis- medical emergency
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
Septic Arthritis pathogenesis
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
Septic Arthritis Clinical Manifestations
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
joints of septic arthritis
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
Septic Arthritis Clinical Manifestations
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
Septic Arthritis labs
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
septic arthritis organisims
mostly staph auerus
some strep (A)
Gram neg bacilli
rarely anaerobes, staph epidermidis
Septic Arthritis Imaging
Helpful for joints that are difficult to palpate and aspirate Hip Shoulder Sternoclavicular SI joints
Septic Arthritis Treatment
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
Septic Arthritis Complications
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%
finkelstein tests for what?
de quervain’s tendonitis
Gonococcal Arthritis
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
Gonococcal Arthritis - who gets it
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
Gonococcal Arthritis Labs
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
Gonococcal Arthritis Treatment
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
Prosthetic Joint Infections
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
Prosthetic Joint Infections- early vs late
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
Prosthetic Joint Infections Labs
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
Prosthetic Joint Infection Imaging
Serial x-rays
- New subperiosteal bone growth
- Transcortical sinus tracts
- Radiolucency at the bone-cement interface to suggest joint loosening
Prosthetic Joint Infection Treatment- late vs early
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
Prosthetic Joint Infections - complications
Prosthesis failure
- Even after successful treatment, these revised joints often require early replacement compared to non-infected joints
Recurrent infection
