Rheumatology Flashcards

Terms Definitions
Infliximab
Remicade

DMARD
RA
causes of gout?
family
etoh
dehydration
overweight/overeat
crash diet/fasting
eating certain food
diuretic
kidney disease
causes of ra?
smoke
family hx
Cauda Equina
S&S:
Hyperreflexia
Upward babinski sign
Urinary Incontinence
Decreased rectal sphincter tone/incontinence
Is fibromyalgia a progressive condition?
no
3 main jts of pmr?
shoulder
neck
hip
Name a anti-b-cell antibody medication
rituximab
is Rheumatoid joint involvement symmetric or asymmetric?
symmetric
drug-induced lupus
reactions to hydralazine, isoniazid, procainamide and quinidine
presents with fever, arthritis, fatigue and rarely plurisy
severe symptoms of lupus are not seen including skin disease and photosensitivity
anti-histone antibodies are present
in hydralazine-induced lupus only 1/3 have anti-histone antibodies present
manage by withdrawing causing drug which confirms diagnosis when symptoms subside within 2 weeks
Is cancer risk greater with polymyositis or dermatomyositis?
Dermatomyositis
Clinical lupus diagnosis?
indirect immunofluorescence that detects generic ANAs
Can sacroiliitis occur in enteropathic arthritis?
yes
Secondary OA
Secondary to hemachromatosis, Wilsons Disease, acromegaly, congenital hip dislocation
Rheumatologic syndromes associated with hypothyroid
M-TRAP
Myxedematous arthropathy: synovial thickening, laxity, effusions
Tunnel (carpal) 7%
Raynaud's
Aching muscle
Proximal muscle weakness w/ elevated CK
an inflammed proliferating synovium is called a?
pannus
What is included in the non-pharmacologic management of RA?
Education
Physical/occupational therapies
Systemic rest
Articular rest
Exercise (joints/muscles/endurance)
Heat/Cold
assistive devices (hip/knee)
splints
weight loss
How is Polyarteritis Nodosa treated?
-High dose corticosteroids
-Cyclophosphamide (lowers mortality/morbidity)

patients with Hep B: short course of prednisone followed by lamivudine and plasmapheresis 3 x/week
How is polymyositis treated?
Prevent atrophy/contractures
treat dermatomyositis
Prednisone
Methotrexate
Azathioprine (Imuran)
IV immunoglobulin
Rituximab (B cell depletion therapy)
general advice?
talk to team, eg rheumatologist
early diagnosis and intervention
learn about condition, actively manage
manage pain
stay active
diet and lifestyle change
reduce stress, quit smoking
support
Lesch-Nyhan Syndrome presentation
severe mental retardation, self mutilation, gouty arthritis
What is a main toxicity of abatacept?
COPD
What are two trigger of Raynaud's?
cold
stress
Small vessel vasculitides are divided into two groups. Give the groups and the individual diseases.
Immune-complex:
Hypersensitivity vasculitis
HSP
Cryoglobulinemic

ANCA (paucimmune)
Wegener's
Churg-Strauss
Microscopic polyangiitis
Hep C is associated with what vasculitis?
cryoglobulinemia
What may high-dose corticosteroid therapy in the setting of scleroderma be associated with?
normontensive renal crisis
What is osteomalacia?
Also known as "rickets" in children, it is a disorder of defective mineralization of the organic matrix of the skeleton due to inadequate intake or metabolism of Vitamin D.
What are 10% of Polyarteritis Nodosa cases caused by?
hepatitis B
What type of disorder is SLE?
inflammatory autoimmune disorder
What is a significant factor in joint damage for RA?
inflammation
how to diagnose ankylosing spondylitis?
inflammation level
spine x-ray
refer to rheumatologist
NSAIDs in RA
modest symptomatic relief, but don’t prevent joint destruction, NOT GOOD FOR MONOTHERAPY
young female w/ arthritis, rash on the face, in the lab has leukopenia, anemia, what's the diagnosis?
SLE
What is a clinical feature of amyloidosis?
median neuropathy
What is the female:male ratio of CRPS in children?
4:1
What is the main toxicity of hydroxychloroquine?
macular damage
What is the demographic for Dupuytren's contracture?
white males 50-70
What percentage of RA patients are RA +?
85%
RA - Labs
RF positive (80%)
Anemia of Chronic Disease
Elevated ESR
Where is Bechet's most common?
"Marco Polo's Silk Route"
Japan, Turkey, Mediterranean basin
Rheumatoid Arthritis
- extra-articular manifestations
MSK: atrophy, contractures; rheumatoid nodules: olecranon, prox ulna, achilles, occiput (20-30%)
Resp: pleural disease, interstitial fibr, pleuro pulm nodules, pneumonitis, arteritis
Lympadenopathy, Splenomegaly
Vasculitis: can occur anywhere (neuro, MI, cut ulcers)
rare Heart disease
Eye (<1%): episcleritis, scleritis
What is the most common pediatric inflammatory myopathy?
Juvenile Dermatomyositis
rheumatoid arthritis etiology
unknown (most cases) or due to mycoplasma or parvovirus infection
How is reactive arthritis treated?
-NSAIDS are mainstay of treatment
-Antibiotics at time of initial STD may prevent development of reactive arthritis
-Tetracycline for 3 months can reduce duration of symptoms for reactive arthritis caused by chlamydia
-Tumor necrosis factor inhibitors for refractory cases
What happens with spondylitic heart disease?
AV conduction defects
Aortic insufficiency
five shared features of spondylarthropathies
axial skeleton involvement, asymmetric peripheral joint arthritis, enthesitis, seronegativity, HLA-B27 association
What areas are most commonly involved in psoriatic arthritis due to HIV?
foot and ankle
What finger joints are affected in Swan Neck?
MCP or PIP
How is PAN treated?
if no Hepatitis B:
high dose corticosteroids (prednisone, or if severe, methylprednisone)
Cyclophosphamide or other cytotoxics are added if there is major organ involvement

if Hepatitis B, steroids alone allow virus to persist, so add:
plasmapheresis to remove immune complexes,
antivirals against hep B: interferon-2a, lamivudine
#1 clinical sx of Takayasu's
bruits

more common than uneven pulses, etc.
What are dermatomyositis , inclusion body myositis and polymyositis in older patients associated with?
malignancy
Should repeat screening every 6-12 months
Which NSAID is thought to be the most effective for ankylosing spondylitis?
Indocin (Indomethacin)
What are the 3 components of the immune system overload ("the cup runneth over")?
stressful episode
injury
lingering illness
What are 3 complications that may occur due to xerostomia associated with Sjogren's syndrome?
Difficulty swallowing dry foods
Increased thirst
*Increased dental caries
Who does Sjogren's syndrome commonly occur in?
women > men (9:1)

greatest incidence from 40-60 years
complications of gout?
last for 1 week
if not manage well, will turn chronic - constant inflammation, tophi, kidney stones, permanent joint damage
in RA, cytokines lead to:
synovial cell production of mediators of inflammation and matrix metalloproteases
What are the top 4 sites of AS in order of descending incidence?
sacroiliac
lumbar
thoracic
cervical
Where is the main defect resulting in ulnar deviation of fingers?
weakening of ECU
What three areas are affected by Wegener's granulomatosis?
nasal/sinus/oral ulcers (chronic sinusitis, epistaxis, saddle-nose)
pulmonary (nodules, cavitation, fixed infiltrate on Xray)
kidneys (microhematuria or RBC casts)
Aphthous ulcers are 70% idiopathic. What is the most common known cause?
B12, folate, or iron deficiency
Osteoarthritis
- signs and symptoms
- DIP, PIP, knee, cerv/lumb spine, feet, glenohumoral, acromioclavicular, tibiotalar, sacroiliac, TMJ
- mono or asymm oligo/polyarthritis
- joint pain: deep ache, worse by use/better @rest; stiffness/gelling with inactivity <20min, tenderness; can have swelling/bony enlargement, ↓ROM
- periarticular muscle wasting/varus/vagus deform
-Hands: Heberden's/Bouchard's, squared thumb base
-Knee: palpable osteophytes, crepitus, shrug sign (pat fem)
-Feet: hallux valgus/rigidus; hammer/mallet/claw toes
What clinical features should raise suspicion for CPPD?
Involvement in atypical locations, such as the wrist, elbow, metacarpophalangeal, or shoulder joints, in the absence of an inciting trauma
What are the possible complications of antiphospholipid syndrome?
Venous and arterial thrombotic events
History of miscarriages
Leg ulcers
Raynaud's phenomenon
Livido reticularis
How does the skin change with scleroderma?
subcutaneous edema, then thickening and loss of folds
Function of collagen II and proteoglycans in cartilage
provide elasticity and high tensile strength
in OA, chemical shift in cartilage
less keratan sulfate and more chondroitin 4-sulfate
How long does hand/wrist morning stiffness last in RA? OA?
>1-2 hours for RA
<30 minutes for OA
What are the main bacterial agents that can trigger a reactive arthritis?
SSYC + Chlamydia
Salmonella, Shigella, Yersinia, Campy J

but the full list is long
In whom does osteonecrosis commonly develop?
Patients with SLE, rarely with RA
What is Ankylosing Spondylitis and who is likely to get it?
A chronic and progressive inflammatory disease that most commonly affects the spinal, sacroiliac and hip joints. Other Sx may include uveitis and aortitis. Men in the third decade of life are the most frequently affected.
What is Gottron's sign, which is associated with polymyositis?
scaly reddish discoloration over the knuckles, elbows and knees
time course of reactive arthritis symptoms
sx develop weeks after urethritis or diarrhea, joint stiffness and low back pain are early sx, wax and wane over weeks to months
Describe 3 finger joint positions of Swan Neck
1. flexion contracture of MCP
2. Hyperextension of PIP
3. Flexion of DIP
What tests should be performed on exam and labs for suspected Raynaud's?
Nailfold capillary exam with ophthalmoscope set at 40 diopters: if abnormal, then rheumatologic cause is suspected
--Adson's test for thoracic outlet syndrome
--look for scleroderma signs
--check pulses for vasculitis, obstruction
---heart exam for cx that throws clots

LABS:
primary may have ANA
coag studies
TSH
anti-centromere, anti-topoisomerase
cryoglobulins
What is the most common joints involved in osteoarthritis?
knee, hip, distal and proximal interphalangeal, and first carpometacarpal
________ are required to control the disease of SLE in the majority of pts.
corticosteroids; & then should be tapered to the minimum dose
What are the S/S of Polymyalgia Rheumatica?
-pain and stiffness of the shoulder and pelvic girdle
-Fever, malaise, and weight loss
-No muscular weakness
whent is allopurinol used instead of probenacide for gout.
if patient has kidney stone, chronic kidney disease (creatinin >2), serum uric acid level >11, if gouty tophi is present
T/F: Raynaud's can be diagnosed by putting patient's hands in ice water.
False--this may work, but it is unreliable and dangerous.
How long does stiffness usually last with osteoarthritis? Rhematoid arthritis?
- < 30 min
- > 45 min
What is the downfall of tumor necrosis factor inhibitors?
high cost (more than $10,000 per year)
What test is done at birth to test for congenital hip dislocation?
Barlow - dislocation
Ortolani - relocation
What are the most common side effects of NSAIDs with RA?
GI side effects (can be serious and lead to death)
-PPI’s sometimes used to decrease GI problems
What lab values are seen in Still's disease?
ESR > 50
CRP can be 10x upper limit of normal
leukocytosis 12-40K, neutrophils > 80%
EXTREMELY ELEVATED FERRITIN (cause unknown - acute phase reactant)

can have elevated LFTs
Adalimumab
Humira

DMARD
RA
Feltys
RA
Splenomegaly
Leukopenia (decreased WBC)
causes of ankylosing spondylitis/psoriatic arthritis?
hla-b27
Should angiotensin-converting enzyme inhibitor therapy continue with scleroderma renal crisis even if hemodialysis required?
yes
What is scleroderma?
autoimmune widespread thickening/hardening of the skin
how to manage gout?
nsaids
corticosteroid
colchicine
limit walking if big toe affected
SLE...
- Autoantibodies causing multi-organ inflammation
- Peripheral polyarthritis, symmetric involvement of small and large joints
- Non-erosive
OCP can exacerbate. So can anticonvulsants, hydralazine, procainamide.
More common in blacks/Asians
What are the seronegative spondyloarthropathies?
PAIR:
Psoriasis
Ankylosing spondylitis
Inflammatory Bowel Disease
Reiter syndrome
Sjogren syndrome
lymphocytic infiltration of exocrine glands seen alone or in association with RA, PBC, SLE

presents with itchy eyes with sandy feeling (keratoconjunctivitis sicca) and difficulty swallowing food, parotid enlargement and dental caries

advanced stages affects lungs and kidneys and predisposes to malignant lymphoma

screen with anti-Ro/La antibodies
confirm with biopsy of salivary glands
Are Giant Cell Arteritis & Polymyalgia Rheumatica
a spectrum of the same disease?
yes
What are 2 rheumatology conditions that Raynaud's phenomenon may occur in?
SLE
scleroderma
Presentation of seronegative spondyloarthropathies
inflammatory peripheral/axial arthritis, tendon inflammation
Is enteropathic arthritis symmetric or asymmetric?
asymmetric
What antibiotics are used in adults/children with Lyme's disease?
doxycycline/amoxicillin
T/F: PAN will usually show thrombocytosis and low albumin.
true.
Classic weird symptom of Wegener's
saddle nose
limited scleroderma presentation
CREST syndrome: calcinosis, raynaud, esophageal dysmotility, sclerodactily, telangiectasia

skin involvment is in distal extremities
pulmonary artery hypertension in 25-50%
interstitial lung disease in 10%
anticentromere antibodies are present
Is the platelet count with RA elevated, normal, or decreased?
elevated
Pathologic findings of RA include ___ synovitis with ___ formation.
chronic
pannus
fibromyalgia is common in people with?
inflammatory arthritis
illness/infection
pain from injury/trauma
emotional stress/depression
Initial symptoms of RA:
malaise, fatigue, generalized musculoskeletal pain
ankylosing spondyloarthritis disease progression
tendinoligamentous insertion site inflammation, ossification, bony outgrowths, ankylosis, spinal immobility
Sicke Cell causes what percentage of femeral/humeral heads to go onto osteonecrosis?
33%/25%
With what condition can reactive arthritis be confused?
plantar fasciitis
What two anti-TNFs work best against psoriatic arthritis?
adalimumab
infliximab
Effusions are common in amyloidosis in what joint?
shoulders
Name 5 HLA-B27 + diseases,
Ankylosing spondylitis
Enteropathic arthropathy
Pauciarticular JRA
Psoriatic arthritis
Reiter's syndrome
What kind of syndesmophytes are seen in Reiter's?
"jug-handle"
What is the least reliable antiphospholipid for establishing the syndrome?
anticardiolipin antibody
______ antibodies are the most common antibodies associated with hypercoagulability in SLE.
antiphospholipid
rheumatoid arthritis presentation
need four of the following criteria:
morning stiffness > 1h
swelling of wrists, MCPs and PIPs
swelling of three joints
symmetric involvement
joint erosions on x-rays
RF positive
rheumatoid nodules
constitutional symptoms: fatigue, anorexia, weight loss, generalized wekness
DIPs and lower back joints are not involved

signs: radial deviation of wrist with ulnar deviations of digits
Boutonniere and Swan-neck deformities
nodules are usually on olecranon, occiput or Achilles tendon

Felty syndrome (RA, splenomegaly, neutropenia)
Caplan syndrome (RA, pneumoconiosis)
What may SS-A antibodies with Sjogren's syndrome be associated with?
more extra-glandular manifestations
What joint may be involved w/ psoriatic arthritis?
sacroiliac joint
prognosis of oa?
very positive - usually mild,
surgery if needed, is very effective
What can cause secondary osteoarthritis?
macrotrauma, repeated microtrauma (also obesity), congenity deformity in joint, underlying system disease (diabetes, ochronosis, Wilson's disease, hemochromatosis)
2 spinal complications of ankylosing spondy
fractures, C1-C2 subluxation
What are 4 findings in juvenile dermatomyositis?
heliotrope rash
clumsiness
vasculitis
responds well to steroids
Darkening of tissue parts is called what?
ochronosis (alkaptonuria)
What type of pinning is done in SCFE?
Knowles
What are the three main pulmonary-renal syndromes?
Wegener's granulomatosis
Goodpasture's disease
SLE
Tx of PMR
prednisone 15-20 mg/day should cause rapid resolution; if it doesn't, it's probably not PMR

Taper fairly quickly to 5 mg, may need extended tx; observe for one year
What is is associated with dactylitis and asymmetrical distal interphalangeal joint inflammation?
Psoriatic arthritis
Which rheumatologic condition(s) causes pulmonary lung disease?
*Systemic scleroderma can cause pulmonary fibrosis
*SLE can cause atelectasis, pleural effusions, & pulmonary infiltrates
chronic gout management
low purine diet, limitation of alcohol and diuretics

if recurrent attacks: probenecid in undersecretors or allopurinol in undersecretors or overproducers or renal failure

goal is to lower uric acid levels so monitor plasma uric acid
What can Mesenteric vasculitis associated with Polyarteritis Nodosa cause?
acute abdomen
-abdominal pain w/ N/V
how to diagnose ra?
blood tests for inflammation
rf - negative does not exclude ra
x-ray - negative does not exclude ra
refer to rheumatologist
which antibodies are diagnostic of SLE (2)?
anti-dsDNA and anti-Sm
What 2 surgical treatments are used in LCPD?
epiphysiodesis/valugus osteotomy
How is amyloidosis dx and tx?
UPEP, SPEP, amyloidosis
biopsy (abdominal fat pat, rectal mucosa, bone marrow), Congo red stain, apple-greem birefringence

tx:
primary amyloidosis:
cytotoxic agents: melphalan > colchicine
stem cell transplants

secondary amyloidosis:
control underlying inflammatory dz
DMSO, colchicine
chlorambucil for JRA
cytotoxics are controversial for secondary form
How should patients taking prednisone, ≥5 mg/d, for more than 3 months be treated, if no contraindications?
Calcium, Vitamin D, bisphosphonate
What is the most common rheumatic disease in children in the US?
Juvenile arthritis (JA)
In childhood dermatomyositis, what can develop in the fatty layer of the skin?
hard lumps of calcium deposits
how is gca diagnosed?
microscopy of biopsy of blood vessel on the side of the head
lupus patient has proximal muscle weakness, CPK is elevated. what's the diangnosis. what's the next test you want to order.
mixed connective tissue disorder. order anti-RNP.
What are some other extraskeletal conditions associated with AS?
aortitis leading to conduction defects
Pulmonary fibrosis
amyloidosis
cauda equina syndrome
What type of CRPS is seen after a specific nerve injury?
Type 2
What are the most common organ symptoms of PAN?
>50%: focal necrotizing glomerulonephritis
severe hypertension (due to GN)
mononeuritis muliplex

50%: myalgia, arthralgia
palpable purpura, livedo

<30% liver function abnormalities
abdominal pain
testicular pain

more rare:
Name the five types of psoriatic arthritis and their clinical features.
1. asymmetric oligoarthritis: dactylitis
2. predominant DIP: nail changes
3. arthritis mutilans: telescoping of digits
4. rheumatoid like: fusion of wrists
5. axial: asymmetric SI and syndesmophytes
What does therapy for scleroderma involve?
systematic management of end-organ involvement (CCB for Raynaud's and omeprazole for reflux)
diseases associated with symmetrical polyarthritis
RA (involves mostly the joints), SLE, scleroderma, Sjogren
also parvoB19 and hepB
Does splenomegaly and lymph node enlargement generally occur with RA?
no (few patients have these S/S)
Who is polymyositis more common in?
females
middle childhood and 20s and elderly (bimodal distribution)
Which cells are involved in RA?
CD4+ T cells and B cells
55 y.o. female w/ difficulty climbing stairs w/ purplish rash around the eye. what's the diagnosis and what's the next test you want to order? how do you confirm diagnosis?
dermatomyositis order CPK levels. confirm w/ muscle biopsy.
What 2 x-ray views are required in SCFE?
AP and Frog Leg
What is the treatment for Churg-Strauss?
high dose prednisone, 60 mg/day
Add cyclophosphamide if severe
What is the pharm tx of Reiter's?
Eliminate trigger infection with antibiotics, especially if Chlamydia.
Topicals for skin; refer to ophtho if uveitis
arthritic: Indomethacin 150 mg/day, do not use aspirin or ibuprofen
steroids injections for SI, but systemic steroids are ineffective
if refractory the drug of choice is SULFASALAZINE
What important factor is it important to consider in taking a clinical hx on a child w/arthritis? Why?
*age & developmental level

*b/c arthritis in children can begin at any age from 8 months through adolescence
What can heart and lung involvement with polymyositis lead to?
irregular heart rhythm, heart failure, and shortness of breath
Which joints involved in RA (specifically)?
MCP, PIP, MTP, followed by wrists, ankles, elbows, knees
Name 4 main toxicities of anit-TNF agents.
1. reactivation of TB
2. demyelinating disease
3. CHF
4. Autoimmunity
What is the course of Still's disease?
1/3 self-limiting within 9 months
1/3 with recurrent flares, usually only one
1/3 chronic
What should be suspected in a patient with uveitis, diplopia, asymmetrical inflammatory arthritis, and weight loss?
Whipple's disease (mimics systemic autoimmune disorder)... perform small bowel biopsy
what is the most specific test used to dx RA?
Anti-CCP (anti-citrullinated cyclic peptide)
What x-ray changes may appear with RA?
-Earliest changes occur in the wrists or feet
-Erosions usually first seen at ulnar styloid
What unique medicine is used for acute gout and what is its MOA?
cochicine - inhibits phagocytosis of urate crystals
What are the most common cancer mets to joints?
What is the most common site?
lung and breast cancer
knee most common site
In the absence of an infection, when should adult-onset Still's disease be suspected?
ferritin levels >3000 ng/mL with signs and symptoms compatible with this condition (fever, rash & arthritis)
Is the kidneys and GI tract involved with limited scleroderma?
no involvement of kidneys, but does have involvement of GI tract
What is main defect in boutonniere's deformity?
Rupture of central slip causing PIP to protrude
In what 2 joints does the arthritis of hemochromotosis most commonly occur?
2nd and 3rd MCP and PIP joints
Is it possible for one rash to be the only one that causes a specific rheumatic disease?
NO... BS! B/c all rheumatic diseases cause rashes
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