Rheumatology Flashcards
Juvenile idiopathic arthritis clinical symptoms
- 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
Polyarticular JIA clinical signs
- 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
Oligoarticular JIA clinical signs
- 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)
Systemic JIA
- 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
Psoriatic arthritis
- Rash, arthritis, nail pitting, dactlitis
- More common in girls
- HLA-B27 positive
3 main differences between JIA and leukemia
- Timing of symptoms: JIA pain in morning, leukemia pain awakens the kid at night and typically doesn’t involve a joint
- Progression of symptoms: JIA are periodic and waxing/waning, leukemia is persistent and worsening
- Heme abnormalities: more severe in leukemia
JIA long term consequences
Leg length discrepancy, joint contractures, joint destruction, blindness from chronic uveitis, active disease in adulthood
JIA treatment
- PT/OT
- NSAIDs (indomethacin, ibuprofen, naproxen)
- Steroids and immunosuppressive meds
- Methotrexate (gold standard) - Can use anti-IL-1 (anakinra) for systemic onset
Teenager with night pain and morning stiffness relieved by exercise, pain of large joints (knee), low grade fever/weight loss
Ankylosing spondylitis
- HLA B27 antigen positive over 90% of the time
- ANA and RF negative
- Mainly affects sacroiliac joints
Ankylosing spondylitis imaging finding and associations
- 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)
School aged kid with rash on the face, scaly skin on extensor surfaces of the extremities and interphalangeal joints and large proximal muscle weakness
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
Most common systemic vasculitis in kids involving the skin, GI tract, joints, and kidneys
HSP (IgA vasculitis)
- Slightly more common in boys
- Often has preceeding bacterial or viral infection
SLE vs JDM rash differences
- 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
School aged kid with palpable purpura on lower extremities/buttocks with colicky abdominal pain and heme positive stools
HSP
- Abd pain often from intussusception
- Renal involvement: hematuria, proteinuria, HTN
- Arthritis/arthralgias also common
HSP lab findings
- Elevated creatinine/BUN
- UA with protein and blood
- NORMAL platelets and PT/PTT
Causes of monoartricular arthrtitis
- Lyme/other infections
- Legg-Calve-Perthes disease
- Leukemia/bone tumors
Causes of polyarticular arthritis
FIRE CAM
- Fabry/Farber disease
- Infections/IBD
- Reactive arthrtiis
- E lupus (SLE)
- Connective tissue disease
- And
- Malignancy
Diagnostic criteria for Kawasaki disease
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
Kawasaki disease epidemiology
- 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
Kawasaki disease labs
- Thrombocytosis by 5th day of illness
- Leukocytosis by 12th day of illness
- Normocytic anemia
- Elevated acute phase reactants
- Sterile pyuria
Leading cause of acquired heart disease in developing/developed countries
- Developing: rheumatic heart disease
- Developed: kawasaki disease
Kawasaki disease treatment
- IVIG (2 g/kg)
- High dose aspirin until 24 hours fever free then lower dose for maintenance for 2 months
JIA vs Kawasaki difference
JIA fever will not be acute and the rash will be evanescent, reticular rash that appears when the fever peaks
Measles vs Kawasaki difference
- Measles has EXUDATIVE conjunctivitis
- Measles rash starts at the hairline and progresses downward to the extremities
Reactive arthritis symptoms and infections
- 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)
Afebrile African American child with easy fatigue, chronic cough, and hilar adenopathy
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
Linear hyperpigmented patch that becomes more and more fibrotic, shiny hypopigmented skin with a brown border
Localized linear scleroderma
- Tx: topical lubricants typically is enough, if widespread could use steroids or immunosuppressives (MTX)
Systemic symptoms of scleroderma
Sclerodactyly, pulmonary fibrosis, reflux/dysphagia from lower esophageal sphincter incompetence, Raynaud’s
- ANA is almost always positive
Symptoms of Lupus
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
Lab workup in Lupus
- 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
Heart block in a neonate
Neonatal lupus
- Can also present with rashes on the trunk, hydrops fetalis
- Test for Anti-SSA antibodies
Treatment of lupus
- Mild: NSAIDs, hydroxychloroquine, dapsone for skin, low dose prednisone
- Severe: immunosuppressants like cyclophosphamide and azathioprine
Methotrexate mechanism of action and side effects
- Folate antagonist
- Side effects include GI issues, oral ulcers, bone marrow suppression, and hpeatic transaminitis
- No live vaccines, also at risk for lymphoproliferative malignancies
Stickler’s syndrome symptoms
- AD inheritance (COL21A mutation)
- Joint laxity (early osteoarthritis)
- Craniofacial features: hearing loss, myopia, cleft palate, micrognathia, flat midface
Amplified pain syndrome diagnostic symptoms
- Generalized aches/pains at > 3 areas for > 3 months with normal workup
- Common in teenagers
- Associated with depression, anxiety, etc
