rheumatoid diseases (except RA and gout) Flashcards
systemic juvenile idiopathic osteoarthritis: what is this?
systemic arthritis in <16 y/o
presents with
1) arthritis (+2 joints)
2) salmon pink macular rash
3) daily spiking fevers
systemic juvenile idiopathic osteoarthritis: in who?
children <16 y/o
systemic juvenile idiopathic osteoarthritis: presentation?
arthritis commonly in +2 joints
daily spiking fevers
salmon pink macular rash
arthritis in +2 joints, pink macular rash, fever in 12 y/o. what is this suggestive of?
systemic juvenile idiopathic arthritis
what is systemic juvenile idiopathic arthritis associated with?
anterior uveitis
systemic juvenile idiopathic arthritis: lab?
leukocytosis, thrombocytosis, anemia
elevated ESR and CRP
systemic juvenile idiopathic arthritis: ESR and CRP?
elevated
systemic juvenile idiopathic arthritis: treatment?
NSAIDs
steroids
methotrexate
TNF inhibitors
Sjogren: what is this?
auto-immune disorder characterized by destruction of exocrine glands by lymphocytic infiltrates
Sjogren: what gets destructed?
exocrine glands, especially lacrimal and salivary
which glands gets most commonly destructed in Sjogren?
lacrimal and salivary
which cell type infiltrate glands in Sjogren?
lymphocytes
Sjogren: who gets mostly affected?
females 40-60 y/o
Sjogren: symptoms?
- inflammatory joint pain
- dry, sandy feeling eyes (keratoconjunctivitis sicca)
- dry mouth (xerostomia)
- bilateral parotid enlargement
what causes the dry eyes (keratoconjunctivitis sicca) in Sjogren?
decreased tear production and subsequent corneal damage
Other word for dry eyes in Sjogren?
keratoconjunctivitis sicca
Other word for dry mouth in Sjogren?
Xerostomia
joint pain with dry eyes/mouth and enlarged parotides. what can it be?
sjogren
sjogren: what is the cause of xerostomia?
decreased saliva production -> mucosal damage
what can you see on a tongue in sjogren?
fissuring
sjogren: which antibodies?
ANA +
RF + (can be positive without RA)
SS-A and SS-B
what are antiribonucleoprotein antibodies?
SS-A and SS-B
what kind of antibodies are SS-A and SS-B?
antiribonucleoprotein
in which diseases may SS-A and SS-B be seen?
sjogren and SLE
is sjogren common as a primary disorder?
yes
what can sjogren be secondary to?
RA, SLE, SS (systemic sclerosis)
sjogren: complications?
dental caries
MALT lymphoma
risk of giving birth of baby with neonatal lupus
MALT lymphoma: complication of what?
sjogren
dental caries: complication of what?
sjogren
sjogren: risk of having baby with what kind of disorder?
neonatal lupus
sjogren: how to confirm diagnosis?
labial salivary gland biopsy
(focal lymphocytic sialadenitis)
what do you see on the labial salivary gland biopsy in sjogren?
focial lymphocytic sialadenitis
other word for inflammation of a salivary gland?
sialadenitis
septic arthritis: common causes? (pathogens)
s. aureus
streptococcus
neisseria gonorrea
septic arthritis: mostly bilateral or unilateral?
unilateral
septic arthritis: presentation?
swollen, red, painful joint
synovial fluid purulent
septic arthritis: synovial fluid?
purulent (WBC >50,000/mm3)
septic arthritis: treatment?
antibiotics, aspiration (needle), drainage (cut) to prevent irreversible joint damage
what do you do in septic arthritis to prevent irreversible joint damage?
drainage (+/- debridement)
difference joint drainage and aspiration?
aspiration: needle to let fluids out
drainage: cut it open en let fluids come out
disseminated gonococcal infection: how does it get transmitted? how does it present?
STI that presents as either a
1) purulent arthritis (e.g. knee)
2) or triad from polyarthralgia, tenosynovitis (hand), dermatitis (pustules)
what is the triad of disseminated gonoccal infection?
(presents as this triad or as a purulent arthritis)
polyarthralgia
tenosynovitis (e.g. hand)
dermatitis (pustules)
Pain at multiple joints, pustules and a tenosynovitis of the hand. What is this suggestive of?
disseminated gonococcal infection
what are the types of seronegative spondyloarthritis?
psoriatic arthritis
ankylosing spondylitis
IBD
reactive arthritis
(PAIR)
psoriatic arthritis
ankylosing spondylitis
IBD
reactive arthritis
what are these?
seronegative spondyloarthritis
what characterizes seronegative spondyloarthritis?
no RF
association with HLA-B27
shared symptoms:
- inflammatory back pain
- peripheral arthritis,
- enthesitis
- dactylitis
- uveitis
HLA-B27 associated with what?
seronegative spondyloarthritis (bechterew, IBD, reactive, psoriatic arthritis)
- inflammatory back pain
- peripheral arthritis,
- enthesitis
- dactylitis
- uveitis
shared symptoms of what?
seronegative spondylarthritis
which type of arthritis are NEGATIVE for RF?
seronegative spondyloarthritis
(bechterew, reactive, psoriatic arthritis, IBD)
what is dactylitis?
swelling of fingers, ‘sausage’ fingers
what is enthesis?
inflamed insertion sites of tendons, e.g. Achilles
characteristics of back pain in seronegative spondylarthritis?
worse in morning, better with movement
Psoriatric arthitis: arthritis in combination with what?
skin psorias and nail lesions
psoriatric arthritis: involvement of joints symmetric or asymmetric?
asymmetric
psoriatric arthritis: xray?
dactylitis and pencil-in-a-cup deformity
pencil-in-a-cup deformity of DIP on xray. suggestive of what?
psoriatric arthritis
how often is psoriatric arthritis seen in psoriatric patients?
fewer than 1/3
ankylosing spondylitis: other name?
Bechterew
Bechterew: other name?
ankylosing spondylitis
ankylosing spondylitis: involvement of which joints?
spine and sacroiliac joints
ankylosing spondylitis: symmetric or asymmetric?
symmetric
complications ankylosing spondylitis?
joint fusion (ankylosis), uveitis, aortic regurgitation
restrictive lung disease
symmetric inflammation of sacroiliac joints. what kan it be?
ankylosing spondylitis (Bechterew)
ankylosing spondylitis: what happens to he spine?
vertebral fusion -> bamboo spine
ankylosing spondylitis: what can happen if costovertebral and costosternal ankylosis occurs?
restrictive lung disease
what can lead to restrictive lung disease in ankylosing spondylitis?
ankylosis of costovertebral and costosternal joints
how to assess disease severity of restrictive lung disease in ankylosing spondylitis?
degree of chest expansion
ankylosing spondylitis: more common in who?
males (20-40 y/o)
ankylosing spondylitis: age of onset?
20-40
which GI diseases are associated with seronegative spondylarthritis?
crohn and ulcerative colitis (IBD)
reactive arthritis: triad?
conjunctivitis
urethritis
arthritis
(can’t see, can’t pee, can’t bend my knee)
can’t see, can’t pee, can’t bend my knee
what is this?
reactive arthritis
(conjunctivitis, urethritis, arthritis)
reactive arthritis is what kind of arthritis?
seronegative spondyloarthritis
(RF negative, HLA-B27 associated)
psoriatic arthritis is what kind of arthritis?
seronegative spondyloarthritis
(RF negative, HLA-B27 associated)
bechterew is what kind of arthritis?
seronegative spondyloarthritis
(RF negative, HLA-B27 associated)
mnemonic reactive arthritis symptoms?
can’t see, can’t pee, can’t bend my knee
menmonic reactive arthritis bacteria?
She Caught Every Student Cheating Yesterday (and overREACted)
Shigella Campylobacter E.coli Salmonella Chlamydia Yersinia
which pathogens cause reactive arthritis?
Shigella Campylobacter E.coli Salmonella Chlamydia Yersinia
(she caught every student cheating yesterday and overreacted)
SLE: abbreviation for what?
systemic lupus erythematosus
SLE: what is it?
relapsing-remitting auto-immune disease
classic presentation: rash, joint pain, fever in female of reproductive age
SLE: which type of hypersensitivity reactions are involved?
type III: immune complex (=> most common)
type II: IgE, IgM
How fast is a type III hypersensivity reaction?
1-3 weeks
How fast is a type II hypersensitivity reaction?
hours to days
what is a type III hypersensitivity reaction?
immune complex
1-3 wks
what is a type II hypersensitivity reaction?
IgG, IgM
hours-days
which type of hypersensitivity reaction causes most organ damage in SLE?
type III immune complex
1-3 weeks
SLE is associated with a deficiency of what kind of proteins?
complement
(e.g. C1q, C4, C2)
-> reduced clearance of immune complexes
SLE is associated with a reduced clearance of what?
immune complexes
(due to complement deficiency)
what is an immune complex?
combination of an antibody with its antigen (can be multiple)
must be cleared, if not ->auto immune
rash, joint pain, fever in female of reproductive age
suggestive of?
SLE
classic presentation of SLE?
fever, rash and joint pain in female of reproductive age
increased prevalence of SLE in which populations in the US?
black, hispanic, asian, caribbean
heart condition associated with SLE?
LSE (Libman Sacks Endocarditis)
sterile thrombotic vegetations
what is endocarditis?
inflammation of the inner lining of the heart’s chambers and valves
which important complications can be caused by SLE?
LSE and lupus nephritis
what is lupus nephritis?
glomerular deposition of DNA-anti-DNA immune complexes
which immune complexes are deposited in lupus nephritis?
DNA-anti-DNA
what is glomerular deposition of DNA-anti-DNA immune complexes?
lupus nephritis
symptoms of lupus nephritis?
hematuria and proteinuria
causes of death in SLE?
renal, infection and cardiovascular (accelerated CAD)
what is the most common cause of death in SLE?
renal disease
mnemonic causes of death in SLE?
patients die with Redness In their Cheeks
renal - infection - cardiovascular
SLE with anti-SSA: increased risk of giving birth to baby with what?
neonatal lupus
symptoms neonatal lupus?
neonatal heart block
periorbital/diffuse rash
transaminitis
cytopenia
(present at birth)
what kind of antibody in a person with SLE gives increased risk of neonatal lupus?
anti-SSA
mnemonic SLE symptoms?
RASH OR PAIN
Rash
Arthritis
Serositis
Hematological (cytopenia)
Oral/nasopharyngeal ulcers
Renal disease
Photosensitivity
ANA
Immunological
Neurological symptoms
is arthritis in SLE erosive?
no
what kind of serositis in SLE?
pleuritis, pericarditis
what kind of hematological symptoms in SLE?
cytopenias
what kind of rash in SLE?
malar or discoid
what kind of ulcers in SLE?
oral and nasopharyngeal ulcers, painless
what is ANA short for?
antinuclear antibodies
neurological symptoms in SLE?
seizures, psychosis
antibodies in SLE?
anti-nuclear antibody
anti-dsDNA
anti-Smith
antiphospholipid
ANA, anti-dsDNA, anti-Sm, antiphospholipid
which antibody is specific for SLE?
anti-Smith (anti-Sm)
is positive ANA always associated with an auto-immune disease?
no
mixed connective tissue disease: which antibody?
speckled ANA (=anti-U1 RNP)
mixed connective tissue disease: features of which diseases?
SLE, SS, polymyositis
antiphospholipid syndrome: if secondary, to what?
SLE
can antiphospholipid syndrom be a primary disease?
yes
antiphospholipid syndrome: diagnosis based on what?
clinical criteria:
1) history of thrombosis (arterial or venous)
OR
2) spontaneous abortion
with lab findings of antiphospholipid antibodies
which are the antiphospholipid proteins?
lupus anticoagulant
anti B2 glycoprotein I
anticardiolipin
anticardiolipin is what kind of protein?
antiphospholipid
anti b2 glycoprotein is what kind of protein?
antiphospholipid
lupus anticoagulant is what kind of protein?
antiphospholipid
antiphospholipid syndrom: treatment?
systemic anticoagulation
history of thrombosis and lupus anticoagulant positive
suggestive of?
antiphospholipd syndrome
history of spontaneous abortion and anticardiolipin positive
suggestive of?
antiphospholipid synrome
what test can anticardiolipin influence?
syphilis test (VDRL/RPR)
-> false positive
which test can lupus anticoagulant influence?
can cause prolonged PTT
not correct by addition of normal platelet free plasma
polymyalgia rheumatica: symptoms?
pain and stiffness in proximal muscles (shoulders, hip)
often with systemic symptoms (fever, malaise, weight loss)
does polymyalgia rheumatica cause muscle weakness?
no
what is polymyalgia rheumatica associated with?
giant cell (temporal) arteritis
polymyalgia rheumatica: most common in who?
females >50 y/o
woman 55 y/o, stiff and painful shoulders and hips. no weakness
what is this suggestive of?
PMR
polymyalgia rheumatica: lab values?
elevated ESR and CRP
normal CK
polymyalgia rheumatica: creatinine kinase elevated?
no
polymyalgia rheumatica: treatment?
low dose glucocorticosteroids
polymyalgia rheumatica: how fast is the response to low dose corticosteroids?
fast
fibromyalgia: most common in who?
females 20-50 y/o
fibromyalgia: symptoms?
chronic, widespread musculoskeletal pain
associated with ‘tender points, stiffness, paresthesias, poor sleep, fatigue, cognitive disturbances ‘fibro fog’
which rheumatic disease is associated with ‘tender points’?
fibromyalgia
45 y/o woman with diffuse chronic pain in joints with tender points, poor sleep and fatigue
suggestive of?
fibromyalgie
fibromyalgie: how are the inflammatory markers (ESR, CRP)?
normal
Fibromyalgia: treatment?
exercise, antidepressants (TCA), neuropathic pain agents (gabapentin)
what is gabapentin?
neuropathic pain agent
used in fibromyalgia
what is a neuropathic pain agent used in fibromyalgia?
gabapentin
Polymyositis/dermatomyositis: nonspecific antibodies/lab values
ANA +
elevated CK
polymyositis/dermatomyositis: specific antibodies
anti-Jo-1 (histidyl-tRNA synthetase)
anti-SRP (signal recognition particle)
anti-Mi-2 (helicase)
anti-Jo-1: antibody against?
histidyl-tRNA synthetase
anti-SRP: antibody against?
signal recognition particle
anti-Mi-2: antibody against?
helicase
anti-Jo-1: specific for which diseases?
polymyositis/dermatomyositis
anti-SRP: specific for what?
polymyositis/dermatomyositis
anti-Mi-2: specific for what?
polymyositis/dermatomyositis
Polymyositis: symptoms?
progressive symmetric proximal muscle weakness
polymyositis: symmetric or asymmetric?
symmetric
polymyositis: which muscles most often?
proximal muscles, most often shoulders
polymyositis: underlying pathology?
endomysial inflammation with CD8+ cells
endomysium: surrounds each individual muscle fiber/cell
what is the endomysium?
the sheath surrounding each individual muscle fiber
polymyositis: weakness?
yes
person with progressive symmetrical weakness in shoulders
no skin involvement
suggestive of?
polymyositis
dermatomyositis: symptoms?
similar to polymyositis (progressive symmetric proximal muscle weakness) with skin involvement:
- Gottron papules
- facial erythema
- shawl and face rash
- mechanic’s hands
dermatomyositis: dermatological symptoms?
- Gottron papules
- facial erythema (eyelids)
- face and shawl rash
- mechanic’s hands
what are gottron papules?
red, dry/flaky, bumps overlying the knuckles of the fingers
pathognomic for dermatomyositis
how does the facial erythema in dermatomyositis commonly look?
heliotrope (violet) edema of the eyelids
face and shawl edema
what color is heliotrope edema?
violet
heliotrope edema of the eyelids often seen in dermatomyositis
what are ‘mechanic’s hands’?
thickening, cracked, irregular, ‘dirty’ skin of fingers due to hyperkeratosis
often seen in dermatomyositis
dermatomyositis: pathology?
perimysial inflammation and atrophy with CD4+ cell involvement
perimysium: sheet of connective tissue surrounding a bundle of muscle fibers
what cells are involved in polymyositis?
CD8+
what cells are involved in dermatomyositis?
CD4+
which sheet in a muscle is involved in polymyositis?
endomysium
(surrounds individual muscle cell)
which sheet in a muscle is involved in dermatomyositis?
perimysium
(surrounds bundle of muscle cells)
dermatomyositis gives increased risk of what other disease?
occult malignancy
(can’t find the primary tumor)
person with progressive, symmetric weakness of the shoulders and red bumps on joints of the fingers
suggestive of?
dermatomyositis
myositis ossificans: what is this?
heterotopic ossification in skeletal muscle (e.g. quadriceps)
heterotopic: meaning?
in the wrong place
heterotopic ossification: what is this?
extraskeletal bone in muscle and soft tissue
myositis ossificans: associated with what?
blunt muscle trauma
myositis ossificans: presentation?
painful soft tissue mass
myositis ossificans: imaging?
eggshell calcification
myositis ossificans: histology?
metaplastic bone surrounding area of fibroplastic proliferation
myositis ossificans: benign or malignant?
benign
what may myositis ossificans be mistaken for?
osteosarcoma
(myositis ossificans is benign)
person develops hard, painful mass in quadriceps after blunt trauma
imaging: eggshell calcification near femur
what benign condition can this be?
myositis ossificans
RA: most commonly affected joints?
hands, wrists, and knees
osteoarthritis: most commonly affected joints?
hands, hips, and knees
