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
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
26
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
27
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
28
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
29
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
30
IBD Associated Spondyloarthropathy Treatment
Treatment of the underlying IBD - Sulfasalazine NSAID – avoided - May exacerbate IBD Anti-TNF agents - If sulfasalazine has failed
31
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 ```
32
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 ```
33
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
34
Reactive Arthritis Treatment
NSAIDs treatment of choice for articular manifestations Treat active infection with antibiotics Sulfasalazine Methotrexate
35
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
36
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
37
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
38
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
39
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
40
Psoriatic Arthritis Clinical Findings- Labs
20% with hyperuricemia
41
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
42
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
43
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
44
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
45
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
46
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
47
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
48
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
49
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
50
septic arthritis organisims
mostly staph auerus some strep (A) Gram neg bacilli rarely anaerobes, staph epidermidis
51
Septic Arthritis Imaging
``` Helpful for joints that are difficult to palpate and aspirate Hip Shoulder Sternoclavicular SI joints ```
52
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
53
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%
54
finkelstein tests for what?
de quervain's tendonitis
55
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
56
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
57
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
58
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
59
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
60
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
61
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
62
Prosthetic Joint Infection Imaging
Serial x-rays - New subperiosteal bone growth - Transcortical sinus tracts - Radiolucency at the bone-cement interface to suggest joint loosening
63
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 ```
64
Prosthetic Joint Infections - complications
Prosthesis failure - Even after successful treatment, these revised joints often require early replacement compared to non-infected joints Recurrent infection