BRS Rheumatology and Ortho Flashcards
URI –> skin, GI, renal, and joint manifestations
- skin: petechiae and palpable purpura on buttocks and legs, edema of extremities and face
- joint: arthritis, arthralgia
- GI: colicky abd pain, GI bleeding, intussusception
- renal: ranges from mild hematuria to ESRD
henoch Schönlein purpura- IgA mediated vasculitis
how to dx HSP
what are the plts like?
H&P
may see IgA immune complexes in serum or kidney
**note: plt counts are normal! this is a nonthrombocytopenic purpura
how to tx HSP
prognosis?
supportive care
steroids for abd pain and arthritis
most recover in 4 weeks- some might recur, morbidity depends on severity of nephritis
median age of HSP
age 5 years
______ is an acute febrile vasculitis of childhood
most common cause of acquired heart disease in kids in US
-mean age of presentation? MC ethnicity affected?
kawasaki disease (mucocutaneous lymph node syndrome)
mean age 18-24 months
Asian
diagnostic criteria of kawasaki disease
- fever for at least 5 days
AND - 4/5 of the following:
-conjunctivitis- limbic sparing, no exudate
-oropharyngeal changes- red cracked swollen lips, strawberry tongue
-cervical adenopathy- unilateral, nonsuppurative
-rash- trunk
-changes in extremities- brawny edema, erythematous palms and soles –> peeling around nail beds
AND - it’s not caused by something else
other features of kawasaki disease
coronary artery aneurysm in the subacute phase of the disease (days 7-14) low grade myocarditis CHF urethritis (sterile pyuria) aseptic meningitis *hydrops of the gallbladder arthritis or arthralgias anterior uveitis
3 phases of kawasaki disease
- acute phase (1-2 weeks): fever, conjunctivitis, oropharyngeal changes, cervical adenopathy, rash, swollen hands
- increased ESR and CRP - subacute phase (weeks to months): defervescence of inflammation, peeling from nail beds or distal extremities, *coronary artery aneurysms
- decreasing ESR and CRP, increasing plt count - convalescent phase (weeks to years): gradual resolution of aneurysms
- normalization of labs
tx for kawasaki disease
acute phase: high dose IVIG, hight dose aspirin
subacute phase: low dose aspirin
convalescent phase: low dose aspirin only if aneurysms remain
prognosis of kawasaki disease
if coronary artery disease is absent, no long term sequelae occur
diagnostic criteria for JRA
- age of onset < 16 years
- arthritis in at least 1 joint (swelling or effusion OR limitation of motion, tenderness, increased warmth)
- duration of disease > 6 weeks
- exclusion of other causes of arthritis
mean age of onset of JRA
1-3 years
for the most part, JRA is more common in females except for the following two instances:
- M = F for systemic onset JRA
2. M > F for late-onset oligoarticular JRA
two types of oligoarticular JRA (less than or equal to 4 joints)
- early onset: females, majority are ANA+, high risk for developing chronic uveitis
- late onset: males, HLA-B27+, hips and SI joints involved
- in both types, arthritis is usually not symmetric and involves knees and hips most commonly
two types of polyarticular JRA (>4 joints)
- RF negative: both early and late childhood
- RF positive: more in older children, more severe than RF (-) disease, higher risk of severe arthritis
- symmetric polyarthritis of both small and large joints
- high spiking fevers that are worse at night
- transient salmon colored rash when fevers are present, nonpruritic
- HSM
- LAD
- fatigue, anorexia, weight loss, FTT, serositis, CNS involvement, myositis, tenosynovitis
systemic onset JRA (Still’s disease)
what type of anemia do you see in JRA
microcytic and hypochromic- anemia of chronic dz
when is ANA+ with JRA
75% of early-onset oligoarticular
50% of polyarticular
unlike adult inflammatory arthritis, in JRA, RF is ____ in the majority of patients
negative
how to tx JRA
- NSAIDs
- immunomodulatory meds (steroids, methotrexate, etc) for more severe dz
- PT and OT
- surgery if joint issues are awful
- psychosocial support
diagnostic criteria for SLE
4/11 of the following (SOAP BRAIN MD):
- Serositis (pleurites or pericardial inflammation)
- Oral or nasal mucocutaneous ulcers
- Arthritis, nonerosive
- Photosensitivity
- Blood cytopenias (leukopenia, hemolytic anemia, thrombocytopenia)
- Renal disease (hematuria, proteinuria, HTN)
- ANA positive
- Immunoserology- dsDNA, Smith, false + RPR or VDRL
- Neurologic sxs- seizures, psychosis, encephalopathy
- Malar rash
- Discoid lupus
demographic for SLE
teen girls
describe the arthritis in SLE
transient and migratory
rarely causes joint deformity or erosion (unlike JRA)
what are libman-sacks endocarditis?
sterile valvular vegetations
can be seen in SLE
neonates born to mothers with SLE might have this
congenital heart block
which rheum factors are often (+) in SLE but are not specific?
ANA
RF
_____ are specific for SLE and can be used as markers for active disease (esp nephritis)
anti-dsDNA
_______ are very very specific for SLE but are less prevalent
anti-Smith antibodies
these are NOT used as a measure of disease activity
_____ found in SLE predispose you to thrombotic events
antiphospholipid antibodies
these complement findings are present in lupus
decreased C3 and C4
how to tx SLE
-NSAIDs for myalgias and arthralgias
-steroids are the mainstay of therapy!!
+/- cyclophosphamide and other more potent immunomodulators
2 major complications of SLE
- if there’s thrombosis and antiphospholipid antibodies –> LMWH or warfarin
- renal failure –> tx like any other renal failure
prognosis of SLE
survival rate 90% at 5-10 years
major causes of mortality: infection, renal failure, CNS complications
demographic of dermatomyositis in kids
6 year old female
- fatigue, malaise, weight loss
- periorbital violaceous heliotrope rash
- gottron’s papules
- proximal muscle weakness with (+) Gower’s sign
dermatomyositis
neck flexor weakness calcinosis nail bed telangiectasias constipation dysphagia cardiac involvement
dermatomyositis
how to dx dermatomyositis
H&P
abnormal EMG and muscle bx
increased muscle enzymes (CPK, LDH, aldolase)
how to tx dermatomyositis
steroids are the mainstay
vitamin D and Ca to prevent osteopenia
may need more intense immunosuppressants
3 complications of dermatomyositis
aspiration pneumonia 2/2 decreased gag reflex
intestinal perforation 2/2 GI vasculitis
osteopenia 2/2 steroids and muscle weakness
with pediatric dermatomyositis, you must look for a malignancy (T/F)
F
prognosis of dermatomyositis is in general better in kids
rheumatic fever is caused by _______
GABHS strains that cause pharyngitis (the skin infection strains don’t cause rheumatic fever)
Jones criteria for rheumatic fever
2 major or 1 major and 2 minor
major:
- joints
- carditis
- nodules
- erythema marginatum
- sydenham chorea
minor: fever, arthralgia, previous rheumatic fever, leukocytosis, elevated ESR, elevated CRP, prolonged PR interval
cardiac manifestations in rheumatic fever
- endocarditis (most common) –> insufficiency of left sided valves
- myocarditis- tachycardia out of proportion to extent of fever
- pericarditis less common
describe the arthritis in rheumatic fever
migratory asymmetric and painful polyarthritis
-does not result in chronic joint disease
sydenham chorea caused by rheumatic fever is 2/2 involvement of these 2 brain structures
basal gnaglia and caudate nuclei
pink to red macules –> coalesce and spread centripetally with central clearing over the trunk and proximal limbs
erythema marginatum of rheumatic fever
how to look for GABHS infection in rheumatic fever
ASO
anti-DNase
anti-hyaluronidase
how to tx rheumatic fever
- benzathine PCN (IM) one dose or PCN (PO) for 10 days
- NSAIDs for joint pain and swelling, but only give these after the dx is for sure
- steroids if severe cardiac involvement
- tx the CHF if present, tx chorea if severe with haldol
what to do long term for rheumatic fever
long term antimicrobial ppx to prevent further episodes
watch out for heart valve dysfunction
Lyme disease is caused by _______, which is carried on ______
borrelia burgdorferi
deer ticks- ixodes scapularis
infected tick must be attached at least ______ for high chance of lyme transmission
36-48 hours
stages of Lyme disease
early disease (1-4 months)
-early localized
-early disseminated
late disease (5-12 months)
early localized Lyme disease
erythema migrans- annular and taret-like with variable central clearing
constitutional symptoms
early disseminated Lyme disease
- multiple secondary erythema migrans
- constitutional sxs
- neuro: aseptic meningitis, facial nerve palsy (uni and bilateral), encephalitis
- carditis is rare but could cause heart block
late disease of Lyme disease
5-12 months after transmisssion
hallmark is arthritis
how to dx lyme
classic rash
serology- ELISA then confirm with Western blot
how to tx Lyme disease
prognosis
- early localized or late disease with arthritis only: doxycycline (age >9 years) or amoxicillin
- carditis and meningitis require IV ceftriaxone or PCN
prognosis is excellent if treated, even if it’s late tx
triad of arthritis, urethritis, and conjunctivitis
what is it and what precedes it
reiter’s disease (a type of reactive arthritis)
usually preceded by chalmydia trachomatis infection
arthritis of small and large joints
scaly skin plaques
nail pitting
onycholysis (separation of nail from nailbed)
psoriatic arthritis
characterized by enthesitis
males with late onset oligoarticular JRA are at high risk for this later on
ankylosing spondylitis (HLA B27)
IBD pts may have this type of arthritis
HLA B27 positive, involvement of axial skeleton that looks exactly like ankylosing spondylitis
large vessel vasculitis affecting young Asian females
-aneurysmal dilation or thrombosis of the aorta, carotid, or subclavian arteries
takayasu’s arteritis
aneurysms and thrombosis of the small and medium sized vessels
a type of vasculitis
polyarteritis nodosa (PAN)
necrotizing granulomas in multiple organs
-severe sinusitis, hemoptysis, and glomerulonephritis
wegener’s granulomatosis
what is crest syndrome
calcinosis Raynaud's phenomenon esophageal involvement sclerosis of the skin telangiectasias
skin thickening (tightened skin), loss of dermal ridges everywhere in the body
systemic sclerosis
most brachial plexus injuries are during ______
the birthing process
_____ is a brachial plexus palsy affecting the ___ and _____ roots
it is the most common brachial plexus injury
what are some clinical features?
erb’s palsy, C5 and C6 roots
flaccid arm and asymmetric moro reflex
waiter’s tip