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Rheumatology 2
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94
Medical
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11/19/2011

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Term
What is Sjogren's syndrome?
Definition
autoimmune disorder in which immune cells attack and destroy exocrine glands affecting the glands that form saliva and tears
Term
Who is more commonly affected by Sjogren's syndrome, males or females?
Definition
females
Term
What is the average age of diagnosis for Sjogren's syndrome?
Definition
late 40's
Term
What is the 2nt most common autoimmune rhematological disease?
Definition
Sjogren's syndrome
Term
What does Sjogren's syndrome cause?
Definition
dry mouth and dry eyes
Term
What test is used to diagnose Sjogren's syndrome?
Definition
Schirmer test

measures the amount of tear production over 5 minutes with a filter strip that is held in the lower eye lid and then the moisture is measured with a ruler

producing less than 5 mm is indicative of Sjogren's
Term
What imaging modalities can be used in Sjogren's syndrome to detect calculi and other duct problems?
Definition
ultrasound
radiographs with contrast dye (stenson duct)
Term
Who is scleroderma more common in, men or women?
Definition
women
Term
What is the most commonly diagnosed age group for scerloderma?
Definition
30-50
Term
What is scleroderma?
Definition
systemic autoimmune disease characterized by fibrosis, vascular alteration, and autoantibodies

kills the arterioles of the epithelial cells and they are replaced by collagen and other fibrotic material
Term
What do 90% of patients with scleroderma present with?
Definition
Raynaud's phenomenon
Term
What is scleroderma associated with?
Definition
CREST

calcinosis
Raynaud's phenomenon
esophageal dysfunction
sclerodactyly
telangiectasia
Term
What internal organs are most commonly affected by scleroderma?
Definition
kidneys
heart
lungs
esophagus
Term
Is there a cure for scleroderma?
Definition
no (only treat organ systems it affects)
Term
What complications cause most of the deaths due to scleroderma?
Definition
renal and pulmonary complications
Term
What is sarcoidosis?
Definition
abnormal collection of inflammatory cells form as nodules in many organs of the body
Term
What do the granulomas associated with sarcoidosis most often affect?
Definition
lungs and lymph nodes (but can affect any organ)
Term
Sarcoidosis is usually ___________.
Definition
asymptomatic
Term
What condition can cause lung scarring and infection which can lead to respiratory failure and death?
Definition
sarcoidosis
Term
What condition has a higher prevalence in non-smokers?
Definition
sarcoidosis
Term
What condition is a diagnosis of exclusion?
Definition
sarcoidosis
Term
What is the treatment for sarcoidosis if treatment is needed?
Definition
corticosteroids
DMARDs
cyclophasphomide
Term
What is first line treatment for sarcoidosis?
Definition
corticosteroids
(azathiopine, methotrexat also used)
Term
What annual exams need to be done in a patient with sarcoidosis?
Definition
EKG
ocular evaluation
LFTs
serum calcium
24 hour urine calcium
thyroid evaluations
Term
Most patients with sarcoidosis are cured in ___ years.
Definition
5
Term
What is Reiter's syndrome?
Definition
reactive arthritis in response to an infection elsewhere in the body

seronegative spondyloarthropathy
Term
What are the only autoimmune conditions discussed that are more common in men than women?
Definition
Reiter's syndrome
polyarteritis nodosa
Term
What gene is present in 75% of patients with Reiter's syndrome?
Definition
HLA-B27
Term
What is the triad of symptoms associated with Reiter's syndrome?
Definition
-inflammatory arthritis (usually polyarticular and asymmetric)
-inflammation of the eye (conjunctivitis, uveitis)
-urethritis/cervicitis
Term
What types of infections are usually the cause of Reiter's syndrome?
Definition
genitourinary or gastrointestinal
Term
What is the most common trigger for Reiter's syndrome?
Definition
chlamydia

also gonorrhea, salmonella, shigella, and camphylobacter
Term
If you suspect Reiter's syndrome, what must you get a good history on?
Definition
sexual hx
travel hx
Term
What is keratoderma blenorrhagica and what condition is it associated with?
Definition
Reiter's syndrome

visco-pustular waxy lesion with a yellow-brown color

less frequent on palms
Term
What must you think about in a patient who presents with balanitis (inflammation of the glans penis)?
Definition
Reiter's syndrome
Term
What type of discharge may be present in a patient with Reiter's syndrome?
Definition
fowl smelling or purulent
Term
How long does Reiter's syndrome last?
Definition
days to months
Term
What is the treatment for Reiter's syndrome?
Definition
treat underlying organism
NSAIDs
methotrexate (reduce joint destruction)
Term
What is dermatomyositis?
Definition
connective tissue disease related to polymyositis that is characterized by inflammation of the muscles and skin
Term
What do many patients have a history of with dermatomyositis?
Definition
EBV
Term
How does polymyositis present?
Definition
bilateral proximal muscle weakness, usually in the upper legs

first c/o fatigue with walking

also occurs in the upper arms
Term
What are patients with dermatomyositis and polymyositis at risk for?
Definition
interstitial lung disease
Term
How is dermatomyositis and polymyositis diagnosed?
Definition
increased creatine kinase (not specific)
muscle biopsy
Term
What might x-rays show in a patient with dermatomyositis and polymyositis?
Definition
calcifications in the muscles
Term
What antibodies are present in >65% of diagnosed patients with dermatomyositis and polymyositis if there is also lung disease present?
Definition
anti Jo antibodies
Term
What is the treatment for dermatomyositis and polymyositis?
Definition
high dose corticosteroids
strength usually improves in 4-6 weeks

if unresponsive, then immunosuppressants
Term
What is death associated with dermatomyositis and polymyositis usually due to?
Definition
cancer or lung problems
Term
What is polymyalgia rheumatica?
Definition
inflammatory disorder that present as pain and stiffness in the hips and shoulders
Term
How does polymyalgia rheumatica present?
Definition
quickly over a few days

symptoms worse with increased inactivity or first thing in the morning

low grade fever, depression, fatigue, unintentional weight loss, loss of appetitie
Term
What condition is associated with giant cell arteritis?
Definition
polymyalgia rheumatica
Term
What condition can cause headache, jaw pain, scalp tenderness, and vision impairment?
Definition
giant cell arteritis
Term
Patients with what condition may find it hard to do simple activities like put on a coat or tend to simple activities of daily living?
Definition
polymyalgia rheumatica

hygiene may be poor
Term
What is the treatment for polymyalgia rheumatica?
Definition
low dose corticosteroids

pt. usually improves in a few days (if not, think about another diagnosis)
Term
How is polymyalgia rheumatica diagnosed?
Definition
normal creatine kinase level
negative RF
negative muscle biopsy
normochromic normocytic anemia (50%)
elevated ESR
mild elevated LFTs
nonspecific mild synovitis
Term
What is vasculitis?
Definition
group of disorders characterized by inflammatory destruction of blood vessels
Term
What vessels are involved in vasculitis?
Definition
both arteries and veins
Term
What conditions are consider vasculitis?
Definition
Buerger's disease
Kawasaki disease
polyarteritis nodosa
Behcet's syndrome
Henoch-Schonlen purpura
Term
What is another name for Buerger's disease?
Definition
thromboangiitis obliterans
Term
What vessels are obstructed in Buerger's disease?
Definition
hands and feet
Term
What demographic is primarily affected by Buerger's disease?
Definition
male smokers between 20 and 40
Term
What are the signs and symptoms of Buerger's disease?
Definition
pain in hands and feet (esp. arches)
claudication in legs
ulcers in the digits
pale, red, bluish hands and feet
symptoms worse with cold/stress
Term
What is the treatment for Buerger's disease?
Definition
control the symptoms
smoking cessation
amputation if gangrene sets in
Term
How can Buerger's disease be prevented?
Definition
don't smoke (especially patients with hx of Raynaud's)
Term
What condition can cause inflammation of blood vessels in children?
Definition
Kawasaki disease
Term
What characterizes the 1st phase of Kawasaki disease?
Definition
2 weeks
persistent fever >104 for 5 days
Term
What characterizes the 2nd phase of Kawasaki disease?
Definition
skin on hands and feet may peel in large pieces
Term
What are signs and symptoms of Kawasaki disease?
Definition
joint pain, abdominal pain, nausea, vomiting

strawberry tongue

swollen lymph nodes

rash on trunk and genitals

bilateral conjunctivitis without exudate
Term
If treatment if delayed beyond ____ days of the start of the fever associated with Kawasaki disease, heart problems may ensue.
Definition
10 days
Term
What is the treatment for Kawasaki disease?
Definition
IV gamma globulin
ASA to reduce heart problems
Term
What vessels are affected by polyarteritis nodosa?
Definition
small and medium sized vessels
spares the aorta and its bronches and the small vessels including the capillaries and arterioles
Term
Where do the microaneurysms associated with polyarteritis nodosa occur?
Definition
bifurcations or branch points of vessels
Term
What organs are affected by polyarteritis nodosa?
Definition
skin
heart
kidneys
nervous system
musculoskeltal system
GU
eyes
brain
GI
Term
What condition is associated with necrotizing inflammatory lesions in the blood vessels?
Definition
polyarteritis nodosa
Term
What are the complications of polyarteritis nodosa?
Definition
organ infarction
gangrene of a limb
aneurysm rupture causing intra-organ bleeding
Term
How many of the 10 criteria must be present to diagnose polyarteritis nodosa?
Definition
3
Term
How is polyarteritis nodosa treated?
Definition
corticosteroids

severe disease--cyclophosphamides
surgery for bowel complications

need multi-specialty involvement with multi-organ involvement
Term
What vessels are affected by Behcets syndrome?
Definition
small (capillaries, arterioles, venules)
Term
What is the triple symptom complex associated with Behcets syndrome?
Definition
recurrent oral apthous ulcers
genital ulcers
uveitis
Term
What is the most common vasculitis in children and teens?
Definition
Henoch-Schonlein purpura
Term
What causes Henoch-Schonlein purpura?
Definition
deposition of IgA in the skin and kidneys
Term
What is the classic triad associated with Henoch-Schonlein purpura
Definition
purpura (palpable hemorrhages in the skin)
joint pain
abdominal pain
Term
What is the prognosis/treatment for Henoch-Schonlein purpura?
Definition
usually self-limiting
most of the time just treat symptoms

1/3 may develop kidney problems which can lead to ESRF
Term
How is the diagnosis of Henoch-Schonlein purpura made?
Definition
classic triad
Term
What is there a risk of in children under 10 who have Henoch-Schonlein purpura?
Definition
1/3 chance of recurrence within 4 months of the first attack

recurrence is more common in older children and adults
Term
Who is end stage renal failure more common in with patients who have Henoch-Schonlein purpura?
Definition
adults
Term
What is chronic myofascial pain syndrome?
Definition
painful condition affecting the muscle and fascia
Term
What is a common presentation of chronic myofascial pain syndrome?
Definition
trigger points
twitching of the muscle
referred pain

sleep disturbance
muscle weakness
tingling
stiffness
Term
What sign might a patient with chronic myofascial pain syndrome exhibit?
Definition
"jump sign" upon trigger point palpation
Term
What is the treatment for chronic myofascial pain syndrome?
Definition
NSAIDs
PT
massage
trigger point injections (-caine, Botox, dry needle)
correcting posture, leg length, workplace ergonomics, depression/anxiety
Term
What does fibromyalgia stem from?
Definition
dysfunction of neurotransmitters and hormones that transfer information from tissue to tissue
Term
What condition produces general aching?
Definition
fibromyalgia
Term
How does fibromyalgia differ from chronic myofascial pain?
Definition
fibromyalgia--tender points
myofascial pain--trigger points with radiating pain
Term
How is a diagnosis of fibromyalgia made?
Definition
3 months of widespread pain and tenderness at 11 of the 18 tender points
Term
What are components of treatment for fibromyalgia?
Definition
gentle exercise and stretching
support groups
patient education
balanced diet
acupuncture
no high impact aerobics/weight lifting
medications
Term
What medications can be used for the treatment of fibromyalgia?
Definition
Lyrica
Cymbalta
Savella
anti-inflammatories
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