Rheumatology Flashcards
difference between CRP and ESR
Inflammatory markers
CRP -autoinflammatory - elevates quick and normalizes quick - cytokines (IL-I and IL-VI) drive CRP up ….think liver
ESR (erythrocyte sedimentation Rate) - Elevates slow and normalizes slow (remember this is the one that measures how many erythrocytes fall in an hour….things that make this heavy have higher ESR
things that elevate ESR….
-fibrinogen
-IgG - in Lupus your IgG is elevated which drives up your ESR
-High lipids
-SCD
Viral or Bacterial
High CRP
Low ESR
viral
which part of immune system is autoinflammatory
innate
which part of immune system is autoimmune
adaptive
Periodic fevers
Autoimmune or autoinflammatory
autoinflammatory so innate immune system
B cells and T cells
part of innate or adaptive
adaptive
Cytokines
part of innate of adaptive
innate
Anakinra is a ___ blocker
IL-I blocker
ANA positive….pt is at risk for
uveitis
+RF or CCP increases risk for
destructive arthritis
PFAPA
Periodic
Fever - 38.5-41C 2-7 days - can almost be predicted
Aphthus stomatitis - round gray oral ulcers
Adenitis
no cause so must be strep neg
non specific increase in CBC, CRP and ESR during attack. normal in between
Med to treat PFAPA
Steroid during flares (prednisolone)
if needed - Colchicine
50% resolve after tonsillectomy
FMF
Familial Mediterranean Fever
Fever
Serousitis - think where you have serous fluid
Risk for amyloidosis (renal)
If ESR is elevated, what else can you order
Fibrinogen
IgG
If IgG is elevated - Rheum cares about this
Medications for JIA
Scheduled NSAIDS- so BID - takes 2-3 weeks to hit peak antiinflammatory properties
then either Methotrexate or Humira
Also intra-articular steroid injections
difference in Methotrexate and Humira for JIA treatment
Methotrexate is 1x per week for about 2 years
2-3 months before you can fail therapy
PO or subq injection however the injection is more effective and the po pills is 2.5mg each. Average dosing is 20-25 mg so it is a lot of pills
Also day after you feel tired and n/v. oral. The injection has a smaller side effect profile
folic acid helps with side effects because the med depletes the folic acid
Humira
injection is 1x every 2 weeks
This is a biologic
more effective
less side effects
black box warding for males with IBD there is an increased risk of lymphoma
JIA diagnostic criterion
> 6 weeks in 1 joint
systemic JIA is autoimmune or autoinflammatory
autoinflammatory
what can you recommend for hair loss in JIA
Folic acid and Biotin
CRASH & BURN
Kawasaki
Conjunctivitis (without exudate) * Also limbic sparing is a big clue - red eyes with clear halo around pupil
Rash (not vesicular or bullous)
Adenopathy (must be at least one lymph node in the anterior chain at least 1.5)
Strawberry tongue (mucositis - fissuring on lips)
Hand or Feet swelling or erythema
Burn (5 days of fever)
age group not to trust in kawasaki
12 mos and younger. Can be an incomplete picture
Thrombocytopenia worsening during kawasaki
Not a sign of disease worsening……it is normal progression
What is most concerning in kawasaki
Coronary dilation
-increase risk of heart attack at a young age
After giving IVIG for kawasaki, how long do we allow fever for before we treat again
36 hours
Treatment for Kawasaki
Options
Pulse steroids (30mg/kg, max 1g) - up to 3 days
IVIG
can repeat if still have fever after 36 hours
Infliximab (Remicade)
Conical teeth on exam —think…
This is a red flag.
Needs genetic testing
Rheum emergency in Hemophagocytic lymphohistiocytosis (HLH)
Iron >500
What is considered B symptoms
Fevers
night sweats
Weight loss
Causes of JDM (Juvenile dermatomyositis
Infections
-viral
-bacterial
-parasitic
Non-infectious
-Muscular dystrophies
-Multiple sclerosis
-Paraneoplastic myopathy
-Metabolic myopathy
Immunologic
-inflammatory
-Lupus
-Guinane barre syndrome
-other connective tissue disease
other
-drug induced
-toxin
Hyperreflexia and hypertonia
upper or lower motor neuron?
upper
Hyporeflexia and hypotonia
upper or lower motor neuorn
lower
Juvenile Dermatomyositis buzzwords
Difficulty
-going upstairs
-Combing hair
-Standing up
Symmetric
intact sensation
positive Gower’s sign
Trendelenburg gait
dosing for pulsing steroids
30mg/kg - max 1 g
Ash leaf spot think
tubular sclerosis
hypomelanic macules are called
ash leaf spots
Ash leaf spots (Tubular sclerosis)
heliotrope rash
seen in Dermatomyositis
what happens in Juvenile Dermatomyositis
This is a vasculopathy where immune cells attack vessels
Risks in JDM
Bowel perforation
in kids, JDM do not have associated cancer risk
symptoms of JDM
Proximal weakness> distal symmetric weakness
+/- tenderness
Limb /girdle muscles
Ant neck flexors back and abd muscles
can affect voice
Gottron Papules
seen in Juvenile Dermatomyositis
Hypopigmented red papules over knuckles
Gottron papules
Dry dark and red dry skin
extensor erythema
Juvenile dermatomyositis
Inverse Gottron papules
Think…
They are on the palms instead of dorsal side of hand
Juvenile Dermatomyositis
Anti MDA5 type
Causes aggressive interstitial lung disease
which type of JDM causes bowel perforation
JDM
Anti-MJ
What type of JDM causes interstitial lung disease
Anti MDA-5
tell me about JDM with anti-p155/140 autoantibodies
Most patients are white and have extensive photosensitive skin rashes.
also associated with a chronic course of illness and generalized lipodystrophy
tell me about Juvenile Dermatomyositis with Anti-MJ autoantibodies
Most patients are white. Anti-MJ autoantibodies are associated with muscle cramps, muscle atrophy, joint contractures, dysphonia, and an absence of truncal rashes. Patients with anti-MJ autoantibodies tended to be weaker and have decreased physical function
also gastro perforation
Cutaneous signs in Juvenile Dermatomyositis
V-neck distribution
Shawl sign
Holster sign
Cuticles and gums - look for prominent or tortuous capilaries
calcinosis in JDM, what should you order
Xray - see if it could be deeper or more
what associated problems associated with JDM
insulin resistance
HTN
increased bMI
Metabolic syndrome
Macrophage activation syndrome
Anemia
Osteopenia
Osteoporosis
what labs test for muscle specific inflammatory markers
AST
Aldolase
CK
LDH
which muscle inflammatory markers respond last and which respond first
first
AST and CK
Last
Aldolase and LDH
what would warrant hospitalization in JDM
-Loss of ambulation
-Inability to raise head against gravity
-Bulbar symptoms
—Difficulty chewing
—Difficulty swallowing
—Difficulty talking
-Dyspnea
-Significant abd pain
Treatment for JDM
Sunscreen and Sun protection -> Sun flairs disease
1st line
-Methotrexate (muscle)
-steroids
-IVIG
-Hydroxychloroquine (skin)
2nd line
-Mycophenalate mofetil
-Rituximab
-Tacro
-Cyclosporine
Severe disease
-Cyclosporine
How long do you avoid live vaccines after IVIG and Methotrexate
11 months
term for capillaries inflamed in nails
nailfold capilaropathy
what rheum disease do you see rash in between knuckles and not on them
lupus
Infliximab is what type of medication
TNF inhibitor
what test before can start Humira
Quantiferon gold
Arthritis in a flair can show an _______ D-Dimer
elevated
polyarteritis nodosa (PAN) clinical symptoms
fever
malaise
weight loss
+ signs of multiorgan involvement
a systemic necrotizing vasculitis with aneurysm formation affects mall and/or med sized vessels
Polyarteritis Nodosa (PAN)
pan dx
must have abnormalities in sm/md A. (necrotizing vasculitis, aneurysm, stenosis or occlusions)
+ 1/5 systemic
1) skin involvement
2) myalgias
3) HTN
4) Peripheral neuropathy
5) Kidney involvement
steroids does what to WBC on labs
can drive it up
Lupus uses ____ criteria for dx
SLICC
what lab is most specific for lupus
Anti-smith
antiphospholipids during illness
can be falsely elevated. re-test in 3 months
Anti-ds-DNA is specific to
nephritis
Complements (C3, C4, CH50) are ______ in kidney disease
low
direct combs looks for
hemolytic anemia
when is CRP not reliable
with decreased liver fx….CRP is made in the liver
what does your body do with ferritin when your sick
stores/hoards iron if body thinks there is infection
> 500 ferritin think….
MAS (macrophage activation syndrome)
HLH
Primary vs secondary HLH is driven by
genetic causes - can’t turn off inflammation when there is an infection
secondary - trigger by infection (EBV, cancer)
what IL involved in fever
IL-I and IL -VI
IN HLH, what will CRP and ESR look like
CRP elevated
ESR low
Big red flag
is JIA painful
no
HLH/MAS treatment
IVMP pulse 30mg/kg (max 1g)
+/- anakinra
why is anakinra nice
stays in system for less than 24 hours
criteria for Behcett’s
patients with recurrent oral aphthae (at least three times in one year) plus two of the following clinical features:
●Recurrent genital aphthae (aphthous ulceration or scarring).
●Eye lesions (including anterior or posterior uveitis, cells in vitreous on slit lamp examination, or retinal vasculitis observed by an ophthalmologist).
●Skin lesions (including erythema nodosum, pseudofolliculitis, papulopustular lesions, or acneiform nodules consistent with Behçet syndrome).
●A positive pathergy test. Pathergy is defined by a papule 2 mm or more in size developing 24 to 48 hours after oblique insertion of a 20-gauge needle 5 mm into the skin, generally performed on the forearm.
Rheumatologic differential for retinal vasculitis (+/- chorioretinitis) includes
Behçet’s, lupus, polyarteritis nodosa, granulomatous polyangiitis, sarcoidosis, HLA-B27 associated disease, inflammatory bowel disease, relapsing polychondritis, and VKH
what does CH50 tell us?
the activity level of the complements
lg vessel vasculites
(aorta)
Takayasu arteritis
giant cell arteritis (adult only)
med vessel vasculitis
(vessels going into organs)
Kawasaki
PAN
small vessel vasculites
(vessels in organs)
ANCA
HSP
Pulmonary capillaritis
If you have a large vessel vasculitis, can you have a small vessel vasculites? vise versa?
If you have large, you can have any of them. If you have small, you would not also have large.
what affects arteries and veins?
lupus
behcets
Behcets
when a patient presents with bleeding and a prolonged activated partial thromboplastin and prothrombin time, in combination with a lupus anticoagulant
Lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS)
vv ecmo
vs
VA ECMO
VV - bypass only lungs
VA - bypass heart and lungs
nailbed capillaritis can also be called
periungal errythema
buzz words on MRI for arthritis
Synovial enhancement
or uptake
_______ can present with monoarticular effusion
lyme
it can also be polyarticular
IVIG can cause fever for
36 hours
acute rheumatic fever diagnosis
either 2 major or 1major with 2 minor
major
carditits
arthritis (poly only)
chorea
erythema marginatum
subcutaneous nodules
minor
polyarthralgia
fever >=38.5
ESR >=60 in the first hour and or CRP>= 3.0
prolonged PR interval
evidence of GAS infection for either
MPO AB
Autoantibodies to MPO (MPO antineutrophil cytoplasmic antibodies: ANCA) occur in several diseases and may be involved in the pathogenesis of vascular inflammation in patients with microscopic polyangiitis (MPA).(1,2) Patients with MPA often develop MPO ANCA and may present with azotemia secondary to glomerulonephritis (pauci-immune necrotizing glomerulonephritis). MPO ANCA are not specific for MPA, and also may be detected in patients with systemic lupus erythematosus with or without lupus nephritis, Goodpasture syndrome and Churg-Strauss syndrome.
PR3AB
Evaluating patients suspected of having Wegener granulomatosis (WG) now called C-ANCA
Distinguishing between WG and other forms of vasculitis, in conjunction with:
-MPO / Myeloperoxidase Antibodies, IgG, Serum
-ANCA / Cytoplasmic Neutrophil Antibodies, Serum (may be obtained as VASC / Antineutrophil Cytoplasmic Antibodies Vasculitis Panel, Serum)
ANCA
Evaluating patients suspected of having antineutrophil cytoplasmic antibody-associated vasculitis (granulomatosis with polyangiitis and microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis)
Antineutrophil cytoplasmic antibodies (ANCA) can occur in patients with small blood vessel vasculitis, including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), or eosinophilic granulomatosis with polyangiitis (EGPA), collectively referred to as ANCA-associated vasculitis (AAV).(2)
c3
Assessing disease activity in systemic lupus erythematosus (SLE)
Decreased C3 may be associated with acute glomerulonephritis, membranoproliferative glomerulonephritis, immune complex disease, active systemic lupus erythematosus, septic shock, and end-stage liver disease.
decreased C3 means
Decreased C3 may be associated with acute glomerulonephritis, membranoproliferative glomerulonephritis, immune complex disease, active systemic lupus erythematosus, septic shock, and end-stage liver disease.
c4
Assessing disease activity in systemic lupus erythematosus, proliferative glomerulonephritis, rheumatoid arthritis, and autoimmune hemolytic anemia
decreased C4
C4 may be decreased in systemic lupus erythematosus, early glomerulonephritis, immune complex disease, cryoglobulinemia, hereditary angioedema, and congenital C4 deficiency.
positive ASO can last how long after strep
1 year
anti-DNase B titer
Demonstration of acute or recent streptococcal infection using anti-DNase B titer
titer to detect a more recent strep infection
ASO titer
If pt with suspected HSP is >10yrs old. What else should you be suspicious of
ANCA
IBS
dosing for Plaquenil
3-5mk/kg/day
higher doses associated with retinal injury
If you pull too much fluid off of a pt it will increase or decrease their creatinine
creatinine bump up
IVIG can cause headaches. what can you do to help
hydrate…have them drink lots of water to help prevent
Increased aldolase is an indicator of
myositis
scoring scale to grade their flare in lupus
SLEDEI
what enzyme looks at metabolizer ….lab should be ran before starting azathioprine
TPMT enzyme
Lupus with + _____ antibiody is at higher risk for vascular changes
antiphospholibid antibody
Lupus with + _____ antibiody is at higher risk for vascular changes
antiphospholipid antibody
HLH triggered by EBV,,,,give what med
Ritux bc knocks out B cells
after IVIG…vaccines
no live vaccines for 11 months
normal age for HSP?
older kids check what
<10yrs
ANCA and IBS
Photosensitivity occurs in ___% of patients with JDM
50%
Sun exposure has been associated with exacerbation
Name at least 3 inflammatory myopathies resulting from infectious etilogies mimicking myositis of JDM
Viral: enterovirus, influenza, coxsackie, echovirus, parvovirus, Hep B, Human T lymphotropic virus 1
Bacterial: staph, strep, Lyme
Parasitic: trichinosis and toxoplasmosis
what is the cause of Trendelenburg sign? Weakness of what muscles
weakness of the hip abductors
At what age is a Gower sign developmentally appropriate
Up to age 3
Rash on outside of thigh….erythematous papules on lateral aspect of the thighs
Holster sign
what type of PT should be initiated at the time of diagnosis (JDM)
Passive ROM 2-3 times per day to prevent loss of ROM using gentle stretching to regain lost range of motion
Death in JDM occurs in ___% of children
1-2%: most often within 2 years of disease onset and associated with progressive skin and muscle disease that is unresponsive to tx
Death often resulting from resp insufficiency or ILD, myocarditis, acute GI ulceration w/perforation or bleeding
Provide at lease 3 examples that would classify with severe disability as related to JDM
Inability to get out of bed
CMAS score of 15 or MMT8 score of 30
Parenchymal lung disease
gut vasculitis
other autoimmune or mimicking disease
need for ICU management
Age <1 yr
Aspiration or dysphagia
what biopsy findings are associated with poor prognosis in JDM
extensive myopathic changes
muscle infarction/necrosis
lymphocytic infiltrates with follicle like structures including follicular dendritic cells
high endothelial venules
After how many months of initiating hydroxychloroquine is it expected to improve skin rash associated with JDM according to brief study by olson and lindsley (only 9 children)
3 months
what sex is most commonly afflicted by JDM
Females >males
Aside from pathognomonic classic skin changes, how many other criteria by Bohan and Peter are needed to make a probable diagnosis of JDM
2
Provide most effective dose and method of providing methotrexate for JDM
up to 15mg/m2 dose per week as a subcutaneous injection
What findings/organ involvement would be active necessitating the use of cyclophosphamide therapy for JDM
Severe ulcerative skin disease
cardiomyopathy
ILD
Severe GI involvement with vasculitis
The presence of TNF alpha 308A allele a risk factor for which 2 disease associations and JDM
Calcinosis
poor course of disease
Mechanic hands associated with what AB in JDM
Anti-Jo AB
most common age distribution in children with JDM
Median age of presentation
5-14
7
non infectious exposures that have been implicated in etiology and pathogenesis of JDM
Meds
immunizations
stressful life events
unusual sun exposure
animal contact
weight training
Types of lipodystrophy associated with JDM
Generalized
partial
focal
metabolic features associated with acquired lipodystrohy in jDM
hypertryglyceride
insulin resistance
abnormal gtt/dm
hypertension
NASH
generalized lipodystrophy
-hypertrichosis
-hyperpigmentation
-enlarged cliteris
Normal liver synthetic function indicated by what labs
normal albumin, glucose, PT/INR
