Rheuma COPY Flashcards
Differentials fever
Systemic JIA, SLE, vasculitis, acute rheumatic fever, sarcoidosis, MCTD
Malignancies, infections and post-infectious syndromes, inflammatory bowel disease, periodic fever (autoinflammatory) syndromes, Kawasaki disease, HSP
Differentials arthralgia
JIA, SLE, rheumatic fever, JDM, vasculitis, scleroderma, sarcoidosis
Hypothyroidism, trauma, endocarditis, other infections, pain syndromes, growing pains, malignancies, overuse syndromes
Differentials weakness
JDM, myositis secondary to SLE, MCTD, and deep localized scleroderma
Muscular dystrophies, metabolic and other myopathies, hypothyroidism
Differential chest pain
Juvenile rheumatoid arthritis, SLE (with associated pericarditis or costochondritis)
Costochondritis (isolated), rib fracture, viral pericarditis, panic attack, hyperventilation
Differentials back pain
Enthesitis related arthritis, juvenile ankylosing spondylitis
Vertebral compression fracture, diskitis, intraspinal tumor, spondylolysis, spondylolisthesis, bone marrow–occupying malignancy, pain syndromes, osteomyelitis, muscle spasm, injury
Differentials fatigue
SLE, JDM, MCTD, vasculitis, JIA
Pain syndromes, chronic infections, chronic fatigue syndrome, depression
Differentials malar rash
SLE, JDM
Sunburn, parvovirus B19 (fifth disease), Kawasaki disease
Differentials oral ulcer
SLE, Behçet disease
HSV infection, PFAPA syndrome
Differentials Purpuric rash
Vasculitis, e.g., ANCA-associated vasculitis, HSP
Meningococcemia, thrombocytopenia, clotting disorders
Gottron papules
Heliotrope rash, periungual telangiectasias
JDM
Differential Arthritis
Juvenile idiopathic arthritis, SLE, vasculitis, HSP, MCTD, scleroderma, acute rheumatic fever, reactive arthritis
Postviral arthritis, reactive arthritis, trauma, infection, Lyme disease, Kawasaki disease, malignancy, overuse syndromes
Differential Gottron papules non rheuma
Psoriasis, eczema
Moa nsiads
Inhibit Cox cyclooxygenase which is critical in production of prostaglandin
Nsaid use skin reaction
Small hypopigmented depressed scars in areas of minor skin trauma
Pseudoporphyria
Side effect of hydroxychloroquine
Retinal toxicity
Irreversible color blindness or loss of central vision
Serious side effect of rituximab
Multifocal leukoencephalopathy
Most common rheumatic problem in kids
Jia
Criteria JIA
Age at onset: <16 yr
Arthritis (swelling or effusion, or the presence of 2 or more of the following signs: limitation of range of motion, tenderness or pain on motion, increased heat) in ≥1 joint
Duration of disease: ≥6 wk
Onset type defined by type of articular involvement in the 1st 6 mo after onset:
Polyarthritis: ≥5 inflamed joints
Oligoarthritis: ≤4 inflamed joints
Systemic-onset disease: arthritis with rash and a characteristic quotidian fever
Exclusion of other forms of juvenile arthritis
arthritis fever hepatosplenomegaly lymphadenopathy serositis
systemic JIA
cutaneous hypersensitivity to trauma in jia
koebner phenomenon
rare but fatal complication of systemic jia
macrophage activation syndrome
or hemophagocytic syndrome
or hemophagocytic lymphohistiocytosis
acute profound anemia thrombocytopenia leukopenia high fever lymphadenopathy hepatosplenomegaly prolonged pt and ptt
macrophage activation syndrome
or hemophagocytic syndrome
or hemophagocytic lymphohistiocytosis
treatment of macrophage activation syndrome
or hemophagocytic syndrome
or hemophagocytic lymphohistiocytosis
mppt
cyclosporine
anikinra
quality of arthritis in ARF
exquisite joint pain
hip pain due to idiopathic avascular necrosis of femoral head
Legg–Calvé–Perthes disease
ANA + in kids with JIA is associated with
chronic uveitis
complications of anterior uveitis in jia
posterior synechiae, cataracts, band keratopathy, blindness
predictors of severe disease in jia
young age at onset RF + rheumatoid nodules \+ anti CCP antibodies large number of affected joints wrist/hands affected
reactive arthritis occurs after
Salmonella sp., Shigella exneri, Yersinia enterocolitica, Campylobacter jejuni, Chlamydia trachomatis
arthritis
uveitis
urethritis
Reiter syndrome
rubella
hepa B
typically affect __
small joints
mumps
varicella
typically affect _
large joints
preceded by respiratory infection
severe hip pain
normal ESR, WBC in cbc
on utz noted effusion/widening of joint space
transient synovitis
or toxic synovitis
charcs of biopsy of sle lesion
hyperkeratosis
follicular plugging
infiltration of mononuclear cells into the dermal/epidermal junction
immunofluorescence of both affected and not affected skin reveal disposition of immune complexes w/in
dermal-epidermal junction
lupus band test
drugs associated with drug induced lupus
Minocycline, procainamide, hydralazine, isoniazid, penicillamine, diltiazem, interferon-α, methyldopa, chlorpromazine, etanercept, infliximab, adalimumab
how to diagnose sle
> or 4 (at least 1 clinical and 1 lab) OR biopsy proven lupus nephritis with positive ANA or anti DNA
clinical criteria: acute/chronic lupus oral ulcers alopecia arthritis serositis renal neuro hemolytic anemia leukopenia thrombocytopenia
immuno critera: ANA anti DNA anti SM antiphospholipid antibody low complement (c3, c4, ch50) direct coombs
t or f
ana levels correlate with disease severity
false
what lab marker correlates with disease severity
anti dsDNA levels
particularly anti Smith antibody
most common complications/death
infection
complication of glomerulonephritis
neuropsychiatric disorders
side effects of cyclophosphamide
hemorrhagic cystitis
premature gonadal failure
malignancy
conduction abnormalities in neonatal lupus detected at. ____ aog
16wks aog
which maternal antobodies are transferred to fetus
anti Ro anti La (SSB)
__% recurrence with congenital heart block in next baby (if prev baby has heart block)
15%
proximal muscle weakness
heliotrope rash
juvenile dermatomyositis
rash in JDM over neck and chest due to photosensitivity
shawl sign
thickened erythematous scaly rash on palms
anti Jo1 antibodies
JDM
mechanic’s hands
proximal muscle weakness symptoms
difficulty climbing combing hair getting out of bed postiive gowers sign head lag
associated with longstanding disease
JDM
lipodystrophy
calcinosis
what deposited in calcinosis
JDM
calcium phosphate
hydroxyapatite
fluoroapatite
findings EMG NCV in JDM
EMG myopathy and denervation with increased insertional activity, fibrillations, and sharp waves
NCV normal
diagnosis of JDM
Classic rash: Heliotrope rash of the eyelids Gottron papules
Plus 3 of the following:
Weakness: Symmetric, Proximal
Muscle enzyme elevation (≥1): CK, Aspartate aminotransferase, Lactate dehydrogenase, Aldolase
Electromyographic changes: Short, small polyphasic motor unit potentials, Fibrillation, Positive sharp waves, Insertional irritability, Bizarre, high-frequency repetitive discharges
Muscle biopsy: Necrosis, inflammation
what is given to reduce toxicity and side effects from folate inhibition in methotrexate use
folic acid
most common visceral manifestation of scleroderma
pulmonary
previously known as CREST syndrom
CREST - calcinosis cutis, raynaud, esophageal dysfxn, telangiectasia
now systemic sclerosis
order of raynauds in cold/stress
blanching -> cyanosis -> erythema
how to diagnose juvenile systemic sclerosis
major criterion
MAJOR CRITERION (REQUIRED)* Proximal skin sclerosis/induration of the skin proximal to metacarpophalangeal or metatarsophalangeal joints
how to diagnose juvenile systemic sclerosis
minor criterion
AT LEAST 2 REQUIRED
Cutaneous: sclerodactyly
Peripheral vascular: Raynaud phenomenon, nailfold capillary abnormalities (telangiectasias), digital tip ulcers
Gastrointestinal: dysphagia, gastroesophageal reflux
Cardiac: Arrhythmias, heart failure
Renal: Renal crisis, new-onset arterial hypertension
Respiratory: pulmonary fibrosis (high-resolution computed
tomography/radiography), decreased diffusing capacity for carbon monoxide, pulmonary arterial hypertension
Neurologic: neuropathy, carpal tunnel syndrome
Musculoskeletal: tendon friction rubs, arthritis, myositis
Serologic: antinuclear antibodies—SSc-selective autoantibodies (anticentromere, antitopoisomerase I [Scl-70], anti brillarin, anti-PM/Scl, anti brillin or anti-RNA polymerase I or III
history of recurrent painful shallow that don’t scar (if buccal, lips, tonge) or scar (genitals)
uveitis
erythema nodosum/pseudofolliculitis
behcet
autosomal recessive
bried acute fever episodes
polyserositis
assoc with AA amyloidosis
familial mediterranean fever
progressive shrinkage and degeneration of the tissues beneath the skin, usually on only one side of the face (hemifacial atrophy)
form of localized scleroderma
Parry–Romberg syndrome
autosomal recessive severe mental retardation ataxia myopathy cataracts failure to thrive decreased mevalonate kinase recurrent fever lasting 3-7days ab pain diarrhea nausea vomiting arthalgia ulcer elevated igD
dutch fever
or hyperimmunoglobulinemia D syndrome
autosomal dominant periodic fever 4-6 days severe ab pain nausea vomiting arthritis myalgia rash conjunctivitis unilateral periorbital edema TNF receptor AA amyloidosis develops in 25%
TRAPS
tumor necrosis factor receptor associated periodic syndrome
autosomal dominant CIAS1 gene -> cryopyrin periodic fever skin rash on biopsy noted perivascular infiltrates of PMN leukocytes polyarthralgia triggered by cold exposure
familial cold autoinflammatory syndrome
periodic fever aphthous stomatitis pharyngititis adenitis 2-5 yrs old ab pain arthralgia
marshall syndrome
autosomal dominant
granulomatous deposition in skin, eye,joint
blau syndrome
cardinal feature of sarcoidosis
noncaseating epitheloid granulomatous lesions
what are located in center of granulomatous lesions in sarcoidosis
macrophage
epitheliod
multinucleated giant cell
cd4 t lymphocye
associated lab finding in sarcoidosis
hypercalcemia
vit D excess 1,25 OH VitD
other name for kawasaki
mucocutaneous lymph node syndrome
infantile polyarteritis nodosa
leading cause of acquired heart disease in developed countries
kawasaki
kawasaki affect __ sized arteries
medium
histopath exam of kawasaki
edema of endothelial and smooth muscle cells with intense inflammatory infiltration of the vascular wall
initially by pmn then by macrophages, lymphocytes and plasma cells
how to diagnose kawasaki
FEVER PLUS 4: bilateral non exudative conjunctival injection with limbal sparing strawberry tongue (erythema of mucosa) dry cracked lips edema and erythema of hands and feet rash nonsuppurative LAD unilateral >1.5cm
giant coronary aneurysm __mm internal diameter
8mm
greatest risk of rupture
3 clinical phases of kawasaki
acute febrile. 1-2 weeks
subacute 2 wk
convalescent 6-8
which phase of kawaski has highest risk of death due to development of aneurysms
subacute
classification of aneurysm in kawasaki
small <5mm
medium 508mm
giant >8mm
when is 2d echo done in kawasaki
at diagnosis
again after 2-3 weeks of illness
1 yr later (if normal 2decho)
then f/u every 5 yr
MMR vaccine must be deferred __ mo after IVIG
11 months
most common vasculitis in kids
HSP
characteristic of HSP
leukocytoclastic vasculitis and igA deposition in the small vessels of skin, joints, gi, kidney
skin biopsy of HSP
vasculitis of the dermal capillaries and post capillary venules
diagnosis of HSP (american)
Two of the following criteria must be present:
• Palpable purpura
• Age at onset ≤20 yr
• Bowel angina (postprandial abdominal pain, bloody diarrhea)
• Biopsy demonstrating intramural granulocytes in small arterioles and/or venules
diagnosis HSP (european)
Palpable purpura (in absence of coagulopathy or thrombocytopenia) and
1 MORE:
• Abdominal pain (acute, diffuse, colicky pain)
• Arthritis or arthralgia
• Biopsy of affected tissue demonstrating predominant
immunoglobulin A deposition
• Renal involvement (proteinuria >3 grams/24 hr), hematuria or red cell casts
age of onset takayasu
10 to 40yrs old
pulseless disease
takayasu arteritis
most commonly involed vessels in takayasu arteritis
subclaian
renal
carotid
proposed classification of pedia takayasu arteritis
Angiographic abnormalities (conventional, CT, or magnetic resonance angiography) of the aorta or its main branches and ONE OF FF:
• Decreased peripheral artery pulse(s) and/or claudication of extremities
• Blood pressure difference between arms or legs of >10 mm Hg
• Bruits over the aorta and/or its major branches
• Hypertension (de ned by childhood normative data)
• Elevated acute phase reactant (erythrocyte sedimentation rate or C-reactive protein)
gold standard in diagnosis of takayasu
arteriography of aorta and major branches: carotid, subclavina, pulmo, renal, mesenteric
biopsy result in PAN
necrotizing vasculitis with granulocytes and monocytes infiltrating
gold standard imaging for PAN
finding?
arteriography
beads on a string
three forms of ANCA (antineutrophil circulating antibodies) asoociated vasculitis
wegener granulomatosis
microscopic polyangiitis
churg strauss syndrome
mean age diagnosis wegener
male or female predominance?
14 yrs
female
biopsy in wegener granulomatosis
necrotizing vasculitis
kidney: cresentic GN with little to no immune complex deposition
granulomatous inflammation
nasal ulceration -> saddle nose deformity epistaxis pulmo hemorrhage hematuria, proteinuria, hypertension conjunctivitis, uveitis, optic neuritis
wegener granulomatosis
treatment of ANCA associated vasculitis
corticosteroids
oral cyclophosphamide (if kidney)
methotrexate (if pulmo only)
peak age of growing pains
4-8 yrs old
diffuse musculoskeletal pain in 3 different areas in 3 months
juvenile primary fibromyalgia syndrome
ongoing burning limb that is subsequent to an injury, immobilization
complex regional pain syndrome
or previously Reflex sympathetic dystrophy
key features of complex regional pain syndrome
allodynia
hyperalgesia
autonomic dysfxn (cyanosis, mottling, hyperhidrosis)
age of onset complex regional pain syndrome
predominance
9-15yr
females 6:1
treatment for complex regional pain syndrome
amytriptylline
episodic cartilage inflammation causing destruction
relapsing polychondritis
diagnosis psoriatic arthritis
arthritis and psoriasis or arthritis and AT LEAST TWO of following: (1) dactylitis, (2) nail pitting or onycholysis, (3) psoriasis in a 1st-degree relative
diagnosis of Juvenile Ankylosing Spondylitis
xray evidence of sacroiliitis (sacroiliitis of grade 2 or greater bilaterally or at least grade 3 unilaterally)
PLUS ONE of FF:
inflammatory back pain
limitation of motion in the lumbar spine
limitation of chest expansion
erythema nodosum, pyoderma gangrenosum, fever, weight loss, or anorexia in a child with chronic arthritis
inflammatory Bowel Disease
Sclerosis typically starts on the __ side
iliac
Squaring of the corners of the vertebral bodies and syndesmophyte formation
bamboo spine
Ankylosing Spondylitis
AA amyloidosis has also been associated with
granulomatous dis- eases such as sarcoidosis, cystic brosis, Crohn disease, malignancies such as mesothelioma and Hodgkin diseases, intravenous drug abuse, and other infections, such as bronchiectasis and HIV.
hereditary amyloidosis genes involved
transthyretin and apolipoprotein A
fevers, myalgias, arthralgias, urticarial-like rash, and progressive sensorineural hearing loss
Muckle-Wells syndrome
homogeneous eosinophilic material that stains with Congo red dye and demonstrates the pathognomonic “apple-green birefringence” in polarized light
amyloid bodies
___ is the underlying cause of death in 40-60% of patients with amyloidosis
End-stage renal failure
