Rheumatology Flashcards

1
Q

Juvenile idiopathic arthritis clinical symptoms

A
  • Morning stiffness, joint redness, swelling, pain, rash
  • Onset before 16 years of age
  • Must be present for at least 6 weeks in at least one joint
  • GIRLS are much more commonly affected than boys
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2
Q

Polyarticular JIA clinical signs

A
  • 5 or more joints in the first 6 months of disease
  • Small to medium joints and symmetrical
  • Rarely have systemic symptoms
  • Can be RF positive or RF negative
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3
Q

Oligoarticular JIA clinical signs

A
  • 4 or fewer joints affected in the first 6 months of disease
  • Medium to large joints, asymmetrical
  • ANA positive, RF often negative (but if positive means worse disease)
  • Common in young females (age 2-4)
  • Boys with this disease are HLA-B27 positive
  • MONITOR FOR CHRONIC UVEITIS (need ophtho consult, #1 treatable cause of blindness)
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4
Q

Systemic JIA

A
  • Affects males and females, usually ages 1-5
  • Systemic symptoms can occur before joint symptoms
  • High fevers daily for at least 2 weeks
  • Leukocytosis, elevated FERRITIN
  • Rash (salmon colored macules that come and go)
  • HSM, lymphadenopathy, pleuritis, pericarditis, serositis
  • Uveitis is rare in systemic JIA
  • RF and ANA often negative
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5
Q

Psoriatic arthritis

A
  • Rash, arthritis, nail pitting, dactlitis
  • More common in girls
  • HLA-B27 positive
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6
Q

3 main differences between JIA and leukemia

A
  1. Timing of symptoms: JIA pain in morning, leukemia pain awakens the kid at night and typically doesn’t involve a joint
  2. Progression of symptoms: JIA are periodic and waxing/waning, leukemia is persistent and worsening
  3. Heme abnormalities: more severe in leukemia
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7
Q

JIA long term consequences

A

Leg length discrepancy, joint contractures, joint destruction, blindness from chronic uveitis, active disease in adulthood

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8
Q

JIA treatment

A
  1. PT/OT
  2. NSAIDs (indomethacin, ibuprofen, naproxen)
  3. Steroids and immunosuppressive meds
    - Methotrexate (gold standard)
  4. Can use anti-IL-1 (anakinra) for systemic onset
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9
Q

Teenager with night pain and morning stiffness relieved by exercise, pain of large joints (knee), low grade fever/weight loss

A

Ankylosing spondylitis

  • HLA B27 antigen positive over 90% of the time
  • ANA and RF negative
  • Mainly affects sacroiliac joints
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10
Q

Ankylosing spondylitis imaging finding and associations

A
  • Bamboo spine on xray
  • Affects males
  • Can involve the eye (irits and uveitis –> acute anterior uveitis)
  • Associated with inflammatory bowel disease and aortitis (check for murmur)
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11
Q

School aged kid with rash on the face, scaly skin on extensor surfaces of the extremities and interphalangeal joints and large proximal muscle weakness

A

Juvenile dermatomyositis

  • Can also have periungual red bumps (nail fold telangectasias)
  • Heliotrope, violaceous, or butterfly malar rash
  • Rash can be very itchy
  • Gottron’s papules are the rash on the hands
  • Muscle sx: difficulty getting dressed, clumsy, trouble climbing steps
  • More common in females
  • Often have elevated CK
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12
Q

Most common systemic vasculitis in kids involving the skin, GI tract, joints, and kidneys

A

HSP (IgA vasculitis)

  • Slightly more common in boys
  • Often has preceeding bacterial or viral infection
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13
Q

SLE vs JDM rash differences

A
  • SLE rash on hands is between the joints, JDM is on the joints
  • Facial rash is pretty similar but JDM may involve a bit more on the eyelids
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14
Q

School aged kid with palpable purpura on lower extremities/buttocks with colicky abdominal pain and heme positive stools

A

HSP

  • Abd pain often from intussusception
  • Renal involvement: hematuria, proteinuria, HTN
  • Arthritis/arthralgias also common
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15
Q

HSP lab findings

A
  • Elevated creatinine/BUN
  • UA with protein and blood
  • NORMAL platelets and PT/PTT
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16
Q

Causes of monoartricular arthrtitis

A
  • Lyme/other infections
  • Legg-Calve-Perthes disease
  • Leukemia/bone tumors
17
Q

Causes of polyarticular arthritis

A

FIRE CAM

  • Fabry/Farber disease
  • Infections/IBD
  • Reactive arthrtiis
  • E lupus (SLE)
  • Connective tissue disease
  • And
  • Malignancy
18
Q

Diagnostic criteria for Kawasaki disease

A

CRASH and Burn: need at least 4 plus the fevers

  • Conjunctivits
  • Rash
  • Adenopathy (cervical common)
  • Strawberry tounge
  • Hands/feet (desquamation/swelling)
  • Fever for 5 days
  • Other symptoms: sterile pyruia, thrombocytosis, CNS symptoms, polyarthritis, coronary artery aneurysms/enlargment
19
Q

Kawasaki disease epidemiology

A
  • Most common around age 2 but usually less than age 4
  • More common in winter/spring
  • more common in males and in patients of Asian descent
20
Q

Kawasaki disease labs

A
  • Thrombocytosis by 5th day of illness
  • Leukocytosis by 12th day of illness
  • Normocytic anemia
  • Elevated acute phase reactants
  • Sterile pyuria
21
Q

Leading cause of acquired heart disease in developing/developed countries

A
  • Developing: rheumatic heart disease

- Developed: kawasaki disease

22
Q

Kawasaki disease treatment

A
  • IVIG (2 g/kg)

- High dose aspirin until 24 hours fever free then lower dose for maintenance for 2 months

23
Q

JIA vs Kawasaki difference

A

JIA fever will not be acute and the rash will be evanescent, reticular rash that appears when the fever peaks

24
Q

Measles vs Kawasaki difference

A
  • Measles has EXUDATIVE conjunctivitis

- Measles rash starts at the hairline and progresses downward to the extremities

25
Q

Reactive arthritis symptoms and infections

A
  • Urethritis, iritis, arthritis (can’t see, can’t pee, can’t climb a tree)
  • Mono/oligo arthritis mostly in lower extremities and in large joints
  • Following a variety of infections (STI, GI, URI): Yersinia, Shigella, Camylobacter, Salmonella, Chlamydia, gonorrhea
  • Happens days to weeks after infection
  • ANA/RF normal but can be HLA-B27 positive
  • Tx is supportive (NSAIDs)
26
Q

Afebrile African American child with easy fatigue, chronic cough, and hilar adenopathy

A

Sarcoidosis

  • CXR: noncaseating granulomas with bilateral peribronchial thickening
  • Renal disease due to hypercalcemia and hyperclacuria
  • Uveitis, can involve the heart as well

LOOKS LIKE TB BUT TB TEST NEGATIVE

27
Q

Linear hyperpigmented patch that becomes more and more fibrotic, shiny hypopigmented skin with a brown border

A

Localized linear scleroderma

- Tx: topical lubricants typically is enough, if widespread could use steroids or immunosuppressives (MTX)

28
Q

Systemic symptoms of scleroderma

A

Sclerodactyly, pulmonary fibrosis, reflux/dysphagia from lower esophageal sphincter incompetence, Raynaud’s
- ANA is almost always positive

29
Q

Symptoms of Lupus

A

Need 4 or more:

  • Malar rash
  • Discoid lesions
  • Photosensitivity
  • Oral ulcerations (painless)
  • Arthritis
  • Serositis (pleuritis, pericarditis)
  • Heme abnormalities (leukopenia, hemolytic anemia, thrombocytopenia)
  • Renal abnormalities (protineuria, hematuria, casts)
  • Presence of antibodies
  • Psychosis/other neuro sx (seizures, headaches)
  • Positive ANA
30
Q

Lab workup in Lupus

A
  • Anti-ds DNA is very SPECIFIC for SLE
  • Low C3/C4
  • Anti-smith is also specific for SLE but don’t go down when disease activity is well controlled
  • ANA is very SENSITIVE but not specific
31
Q

Heart block in a neonate

A

Neonatal lupus

  • Can also present with rashes on the trunk, hydrops fetalis
  • Test for Anti-SSA antibodies
32
Q

Treatment of lupus

A
  • Mild: NSAIDs, hydroxychloroquine, dapsone for skin, low dose prednisone
  • Severe: immunosuppressants like cyclophosphamide and azathioprine
33
Q

Methotrexate mechanism of action and side effects

A
  • Folate antagonist
  • Side effects include GI issues, oral ulcers, bone marrow suppression, and hpeatic transaminitis
  • No live vaccines, also at risk for lymphoproliferative malignancies
34
Q

Stickler’s syndrome symptoms

A
  • AD inheritance (COL21A mutation)
  • Joint laxity (early osteoarthritis)
  • Craniofacial features: hearing loss, myopia, cleft palate, micrognathia, flat midface
35
Q

Amplified pain syndrome diagnostic symptoms

A
  • Generalized aches/pains at > 3 areas for > 3 months with normal workup
  • Common in teenagers
  • Associated with depression, anxiety, etc