• Shuffle
    Toggle On
    Toggle Off
  • Alphabetize
    Toggle On
    Toggle Off
  • Front First
    Toggle On
    Toggle Off
  • Both Sides
    Toggle On
    Toggle Off
  • Read
    Toggle On
    Toggle Off
Reading...
Front

Card Range To Study

through

image

Play button

image

Play button

image

Progress

1/284

Click to flip

Use LEFT and RIGHT arrow keys to navigate between flashcards;

Use UP and DOWN arrow keys to flip the card;

H to show hint;

A reads text to speech;

284 Cards in this Set

  • Front
  • Back
Rheumatoid arthritis affects mainly ___ membranes of ___ joints.
synovial
multiple
Is RA more common in men or women?
women 3:1
What is the usual age of onset for RA?
20-40 years
Is susceptibility of RA genetically determined?
yes
Pathologic findings of RA include ___ synovitis with ___ formation.
chronic
pannus
What is fibrous ankylosis?
stiffening or fixation of a joint that can be seen in chronic stages of RA
What is a significant factor in joint damage for RA?
inflammation
What type of nodules are generally seen with RA?
subcutaneous (20%)
What does the prodrome of RA include? (4)
malaise
weight loss
vague periarticular pain
stiffness
What are the S/S of RA?
*symmetric joint swelling with stiffness, warmth, tenderness, and pain
When is stiffness with RA the worst?
in the morning (generally greater than 30 mins) and subsides throughout the day
How is RA differentiated from osteoarthritis?
RA
-morning stiffness usually lasts >30 mins (mornings the worst)

osteoarthritis
-mornings the best (gets worse throughout the day)
What are the most common sites of RA?
PIP and MCP joints of the fingers
Wrists
Knees
Ankles
Toes
What is the most destructive element affecting joints in the patient with rheumatoid arthritis?
pannus
Is RA monoarticular?
not usually, but can be early in the disease
Does splenomegaly and lymph node enlargement generally occur with RA?
no (few patients have these S/S)
What are 4 common deformities associated with RA?
Ulnar deviation of the fingers
Boutonniere deformity (PIP)
“Swan-neck” deformity (DIP)
Valgus deformity of the knees
What happens to the skin/muscle with RA?
atrophy
With RA, are the mucous membranes wet or dry?
dry
What are 5 conditions that may be associated with RA?
episcleritis
scleromalacia
pericarditis
pleural disease
aortitis (rare late complication that can result in aortic regurg. or rupture)
In what percentage of patients is rheumatoid factor present with RA?
75%
In what percentage of patients is ANA present w/ RA?
20%
Is the sed rate and gamma globulins elevated, normal, or decreased?
elevated
What type of anemia may be present with RA?
hypochromic, normocytic
Is the platelet count with RA elevated, normal, or decreased?
elevated
What is the most sensitive imaging technique done with RA?
x-ray (but not sensitive in first 6 months)
What x-ray changes may appear with RA?
-Earliest changes occur in the wrists or feet
-Erosions usually first seen at ulnar styloid
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
What should a patient w/ RA know about splints?
-Night splints for hands/wrists/knee/ankle
-Should be applied for shortest time period needed
-Corrective splints for contractures
What is the first line drug used for RA?
NSAIDs (analgesic and anti-inflammatory effects)
-can also use selective COX-2 (Celibrex)
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 are DMARDs?
Disease-modifying anti-rheumatic drugs
When should DMARDs be started?
as soon as the diagnosis of RA is certain
What is the DMARD treatment of choice?
Methotrexate (beneficial effect w/in 2-6wks)
What 2 conditions is Methotrexate contraindicated in?
chronic hepatitis
pregnancy
What type of DMARD drugs are given for RA if patient has no response to Methotrexate?
tumor necrosis factor inhibitors
-Etanercept (Enbrel, given subq) and Infliximab (Remicade, given IV)
What is the downfall of tumor necrosis factor inhibitors?
high cost (more than $10,000 per year)
Tumor necrosis factor inhibitors work faster than ____ and may become the drug of choice after ___ in the future.
Methotrexate
NSAIDs
What is the most common antimalarial DMARD used for RA?
Hydroxychloroquine sulfate
What is the most important potential reaction of Hydroxychloroquine sulfate?
pigmentary retinitis, which can lead to visual loss
When is Hydroxychloroquine sulfate useful for RA?
mild disease

also has a low toxicity
When are corticosteroids used for RA?
-Immediate and dramatic anti-inflammatory effect
-May be able to slow rate of bony destruction
**Not good long-term medication
-Intra-articular steroids may be beneficial, can be used 4 times per year
What may Sulfasalazine cause?
Second line agent that may cause neutropenia or thrombocytopenia
Which DMARD is a pyrimidine synthesis inhibitor?
Leflunomide
Leflunomide can be used alone or with ___.
Methotrexate
What is the downfall of using Lefludomide for RA?
drug is carcinogenic, teratogenic
Is Azathioprine (Imuran) mainly effective for mild or severe RA?
severe
What can Azathioprine (Imuran) cause?
immunosuppression
Is Minocycline used in mild or severe cases of RA?
early, mild cases (few side effects)
When is combination therapy used for RA?
when fail to respond to single agents
What are the DMARDs that can be used for RA?
-Methotrexate
-tumor necrosis factor inhibitor (Etanercept, Infliximab)
-antimalarials (hydroxychloroquine sulfate)
-corticosteroids (ex. prednisone)
-Sulfasalazine
-Leflunomide
-Azathiprine
-Minocycline
-Gold salts & Penicillamine (now rarely used)
-combo. therapy
Can surgical measure be used to correct deformities with RA?
yes
How many years early do patients with RA generally die?
10-15 years earlier
What are the most common causes of death with RA?
infection, heart disease, respiratory failure, renal failure and GI disease
Is ankylosing spondylitis more common in men or women?
men
Does ankylosing spondylitis generally have a young or elderly onset?
young
Where is the pain with ankylosing spondylitis?
chronic, low back pain
What are the progressive limitations with ankylosing spondylitis?
back motion and chest expansion
Where are diagnostic x-ray changes seen with ankylosing spondylitis?
sacroiliac joint
What type of eye condition may show up with RA?
anterior uveitis (20-25%)
What do the labs for ankylosing spondylitis show?
elevated sed rate
negative RF
HLA-B27 positive (genetic)
Does ankylosing spondylitis have an acute or gradual onset?
gradual
Where may ankylosing spondylitis radiate?
down thighs
What type of condition is "bamboo spine" associated with?
ankylosing spondylitis
What happens with spondylitic heart disease?
AV conduction defects
Aortic insufficiency
With ankylosing spondylitis, what may occur with the upper lobes of the lungs?
pulmonary fibrosis
Are constitutional symptoms usually present with ankylosing spondylitis?
no
How is ankylosing spondylitis treated?
Postural/breathing exercises
NSAIDS
Physical therapy
Which NSAID is thought to be the most effective for ankylosing spondylitis?
Indocin (Indomethacin)
What 3 meds. can be used for ankylosing spondylitis?
Indocin (indomethacin)
Sulfasalazine
Tumor necrosis factor inhibitors (for refractory symptoms)
What is the prognosis for ankylosing spondylitis?
-Almost all patients have long term symptoms
-Rarely there is long term remission
What percentage of patients with ankylosing spondylitis will have work disability after 10 years?
10%
When is the worst prognosis seen with ankylosing spondylitis?
if hip disease develops within the first 2 years of disease onset
Psoriasis precedes ___ in 80% of cases.
arthritis
Is psoriatic arthritis symmetric or asymmetric?
asymmetric
What joint may be involved w/ psoriatic arthritis?
sacroiliac joint
What type of appearance do the finger and toes have with psoriatic arthritis?
"sausage"
Psoriatic arthritis is at least ___ times more common in patients with severe skin disease.
5
What may happen to the nails with psoriatic arthritis?
nail pitting
What do the labs for psoriatic arthritis show?
elevated sed rate
RF negative
uric acid levels may be high
What is the most helpful imaging modality for psoriatic arthritis?
x-rays
What may show up on x-ray of psoriatic arthritis?
-Marginal erosions of bone
-Irregular destruction of joints and bone
-“Sharpened pencil” appearance of phalanges
-Fluffy periosteal new bone
-Paravertebral ossification
How is the paravertebral ossification of psoriatic arthritis different from ankylosing spondylitis?
no paravertebral ossification in the anterior aspect of the spine with psoriatic arthritis
How is psoriatic arthritis treated?
-Symptomatic treatment
-NSAIDS for mild cases
-Corticosteroids less effective
-Methotrexate may be helpful in resistant cases
-Tumor necrosis factor inhibitors for disease refractory to methotrexate
-PUVA therapy may be helpful
What type of arthritis was formerly known as Reiter's Syndrome?
reactive arthritis
What does the clinical tetrad for reactive arthritis include?
urethritis
conjunctivitis (sometimes uveitis)
mucocutaneous lesions
aseptic arthritis
Who is reactive arthritis most common in?
young men
What percentage of patients with reactive arthritis are HLA-B27 positive?
50-80%
What does reactive arthritis often follow?
dysentery (male=female) or STD (males>females 9:1)
What types of "bugs" can cause dysentery which can lead to reactive arthritis? (4)
shigella, salmonella, yersinia, campylobacter
What types of "bugs" can cause a STD which can lead to reactive arthritis? (2)
chlamydia or Ureasplasma urealyticum
Are the affected joint cultures of reactive arthritis positive or negative?
negative
Is reactive arthritis commonly symmetric or asymmetric?
asymmetric
What joints does reactive arthritis commonly affect?
large weight-bearing joints (knee or ankle)

sacroiliitis in 20% of patients
What are the S/S of reactive arthritis?
-Systemic symptoms may include fever and weight loss
-Mucocutaneous lesions (balanitis, stomatitis)
-Fingernails may pit like psoriatic arthritis
-May see “sausage” fingers and toes
-Carditis and aortic regurgitation
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 3 rheumatology conditions have sacro-iliac joint association?
ankylosing spondylitis
psoriatic arthritis
reactive arthritis
What 2 rheumatology conditions have asymmetric joint involvement?
psoriatic arthritis
reactive arthritis
What 2 conditions may "sausage" fingers and toes appear?
psoriatic arthritis
reactive arthritis
What is SLE?
systemic lupus erythematosis aka Lupus
Who does SLE most commonly affect?
young women
Where does the rash with SLE generally appear?
over sun-exposed areas
What percentage of patients with SLE have joint symptoms?
90%
Is hemoglobin, WBCs, and platelets increased or decreased with SLE?
decreased
What are the 2 major causes of morbidity with SLE?
CNS involvement and glomerulonephritis
How often is ANA positive with SLE?
*100%*
What type of disorder is SLE?
inflammatory autoimmune disorder
What happens to the antigen-antibody complexes with SLE?
they become trapped in capillaries
What happens to the host cells with SLE?
*Autoantibody-mediated destruction
Does SLE have a hereditary link?
yes
During what stage of a woman is SLE more common?
after menarche and before menopause
What must SLE be differentiated from?
drug-induced lupus-like syndrome
What are the 3 most common drugs that can cause drug-induced lupus-like syndrome?
Procainamide
Hydralazine
Isoniazid
What are 4 ways that drug-induced lupus-like syndrome can be differentiated from SLE?
-Sex ratio is nearly equal
-Not usually CNS features or nephritis
-No antibodies to native DNA
-Clinical features and lab abnormalities usually revert to normal when drug is withdrawn
What is the diagnostic criteria for SLE? (4 of 11)
SOAP BRAIN MD

Serositis
Oral ulcers
Arthritis
Photosensitivity
Blood changes (hematologic)
Renal involvement
ANA (positive 100%)
Immunological changes
Neurological signs
Malar rash
Discoid lupus/rash
What are the diagnostic criteria associated with renal disease with SLE?
> 0.5 g/day proteinuria, OR
3+ dipstick proteinuria, OR
Cellular casts
What are the immunologic abnormalties that are included in the diagnostic criteria for SLE? (4)
Positive LE cell preparation, OR
Antibody to native DNA, OR
Antibody to Sm, OR
False-positive serologic test for syphilis
What are the systemic S/S of SLE?
fever
anorexia
malaise
weight loss
What is the classic rash that is seen in 50% of SLE patients?
butterfly rash
What are the skin manifestations that may occur with SLE?
butterfly rash
discoid lupus
periungual erythema
splinter hemorrhages
alopecia
What type of "phenomenon" may occur with SLE?
Raynaud's
-often occurs before other features of the disease
What are the ocular manifestations that may occur with SLE? (4)
Conjunctivitis
Photophobia
Transient or permanent monocular blindness
Cotton wool spots
What may occur in the lungs with SLE? (4)
pleurisy
pleural effusion
bronchopneumonia
pneumonitis
What are the cardiac symptoms that may occur with SLE?
*involvement of pericardium
cardiac failure (myocarditis/HTN)
cardiac arrhythmias
What may occur with the kidneys in patients with SLE?
Glomerulonephritis or interstitial nephritis
What may occur in the joints of SLE?
*joint pain +/- active synovitis
Is mesenteric vasculitis and neurologic symptoms part of the S/S of SLE?
yes
What do the labs with SLE show?
-ANA (sensitive, not specific)
-antibodies to double-stranded DNA & Sm (specific not sensitive)
-depressed serum complement
-3 types of antiphospholipid antibodies
-Anemia
-Leukopenia
-Thrombocytopenia
-Direct Coombs positive
-Proteinuria
-Hematuria
-Hypocomplementemia
What may the 3 types of antiphospholipid antibodies that may show up with SLE cause?
-One causes false-positives for syphilis
-Lupus anticoagulant --> risk factor for venous and arterial thrombosis and miscarriage
-anticardiolipin antibodies --> affect a serum cofactor, not phospholipids directly
Does SLE show a positive or negative direct Coombs test?
positive
Is SLE curable?
no, but it is treatable
What is the #1 goal of treatment with SLE patients?
patient education and emotional support
What should photosensitive patients with SLE do?
limit sun exposure and use sunscreen
What can be used for a rash with SLE?
topical corticosteriods
What is used for joint pain with SLE?
rest
NSAIDs
What type of medication with SLE is used for rashes and joint symptoms that do not respond to NSAIDs?
Hydroxychloroquine
What type of medication might be effective for thrombocytopenia with SLE?
Danazol
What type of medication is used for more serious SLE cases?
corticosteroids
-CNS disease may require higher doses
-taper to lowest effective dose
What is used for lupus nephritis associated with SLE?
immunosuppressant mycophenolate mofetil (CellCept)
What are all the treatment options for SLE?
-patient education & emotional support
-limit sun exposure
-topical corticosteroids
-Hydroxychloroquine
-Danazol
-DHEA (side effect may limit use)
-corticosteroids
-CellCept
What is the course/prognosis of SLE?
-Usually a remitting and relapsing course
-10 year survival rates > 85%
-5 times increased risk of MI
-Outlook is becoming increasingly favorable due to recent advances in therapy
What is scleroderma?
autoimmune widespread thickening/hardening of the skin
Where does scleroderma commonly occur on the body?
Areas of increased pigmentation and depigmentation
What are 2 rheumatology conditions that Raynaud's phenomenon may occur in?
SLE
scleroderma
Does scleroderma have a positive or negative ANA?
positive
What is the cause of scleroderma?
unknown
Where does diffuse fibrosis of scleroderma occur?
skin
internal organs
*Who does scleroderma more commonly affect?
women > men
20s to 40s
What are the 2 forms of scleroderma?
Limited (CREST syndrome) – skin fibrosis limited to face and hands

Diffuse – may have tendon friction rubs
What does CREST (which is associated with SLE) stand for?
Calcinosis
Raynaud's phenomenon
Esophageal dysfunction
Sclerodactyly
Telangiectasias
Is the kidneys and GI tract involved with limited scleroderma?
no involvement of kidneys, but does have involvement of GI tract
What are the S/S of scleroderma?
*dysphagia
*GI hypomotility
*pulmonary fibrosis
*cardiac involement
*scleroderma renal crisis
skin changes
polyarthralgia
Raynaud's phenomenon
fever and malaise
telangiectasia
pigmentation changes
fingertip ulceration
How does the skin change with scleroderma?
subcutaneous edema, then thickening and loss of folds
What are the cardiac abnormalities that may occur with scleroderma?
pericarditis
heart block
myocardial fibrosis
right heart failure
What is calcinosis?
calcium deposits in the skin
What is Raynaud's phenomenon?
spasm of blood vessels in response to cold or stress
What is the esophageal dysfunction that may occur with CREST syndrome associated with limited scleroderma?
acid reflux and decrease in motility of esophagus
What is sclerodactyly?
thickening and tightening of the skin on the fingers and hands
What is telangiectasias?
dilation of capillaries causing red marks on surface of skin (like broken spider veins)
What do the labs for scleroderma show?
mild anemia
positive ANA
scleroderma antibody (SCL-70) in 33%
How is scleroderma treated?
symtomatic/supportive

no known med. will alter disease progress
How is Raynaud's treated with scleroderma?
calcium channel blockers, or losartan
How are the digital ulcers with scleroderma treated?
IV iloprost
How is esophageal reflux with scleroderma treated?
PPI
How is malabsorption associated with scleroderma treated?
tetracycline
How is a renal crisis associated with scleroderma treated?
ACE-I
How is pulmonary fibrosis for scleroderma treated?
cyclophosphamide
What is the percentage of a 9 year survival rate with scleroderma?
40%
What are 2 types of cancer that scleroderma may have an increased risk for?
breast and lung cancer
What are the sicca components of Sjogren's syndrome?
*dryness of eyes and dry mouth
Sjogren's syndrome has an increased incidence of ___.
lymphoma
What is Sjogren's syndrome?
-Chronic autoimmune dysfunction of exocrine glands in many areas of the body
-Alone or in association with rheumatoid arthritis or other connective tissue diseases
What is keratoconjunctivitis sicca?
associated with Sjogren's syndrome
-Decreased tear production
-Lymph and plasma cell infiltration of lacrimal glands
What are the associated conditions that may occur with Sjogren's syndrome?
*RA
Lupus
Biliary cirrhosis
Scleroderma
Polymyositis
Hashimoto’s thyroiditis
Polyarteritis nodosa
Idiopathic pulmonary fibrosis
Who does Sjogren's syndrome commonly occur in?
women > men (9:1)

greatest incidence from 40-60 years
What are the S/S of Sjogren's syndrome?
eye problems
parotid gland enlargement (1/3 of patients)
*xerostomia
*loss of taste/smell
pancreatitis
pleuritis
obstructive lung disease
vasculitis
renal tubular acidosis 20%
chronic interstitial nephritis
What S/S may occur with the eyes in Sjogren's syndrome patients?
ocular burning/itching
*ropy secretions
"grain of sand" sensation
What are 3 complications that may occur due to xerostomia associated with Sjogren's syndrome?
Difficulty swallowing dry foods
Increased thirst
*Increased dental caries
What do the labs for Sjogren's show?
RF positive (60-70%)
ANA positive (70%)
SS-A and SS-B antibodies
What may SS-A antibodies with Sjogren's syndrome be associated with?
more extra-glandular manifestations
What diagnostic procedures can be done for Sjogren's syndrome?
lip biopsy (salivary glands)
parotid gland biopsy
*Schirmer test
What are 2 ways that the Schirmer's test for Sjogren's syndrome can be performed?
-measure amount of moisture in eye with thin paper strip
-put flourescein drops in eyes and see if when blow nose yellow comes out (shows nasolacrimal duct is working if it does)
What type of medication can be used for Sjogren's syndrome?
Pilocarpine (severe xerostomia) OR Cevimiline is alternate
What type of drugs should be avoided with Sjogren's syndrome?
*decongestants and atropinic drugs
-b/c they dry you out even more :(
What type of "therapies" can be used to treat Sjogren's syndrome?
Supportive treatment
Artificial tears
Hard candies or gums
Good oral hygiene
What are the complications of Sjogren's syndrome?
-Benign or malignant lymphoproliferation
-Malignant lymphomas (3 – 10%)
-Waldenström’s macroglobulinemia
What is Waldenström’s macroglobulinemia?
Rare disease featuring a bone marrow tumor of B cells that generates large quantities of a monoclonal antibody
What S/S increase the risk of lymphoma in Sjogren's syndrome patients?
marked parotid enlargement, splenomegaly, vasculitis, anemia, peripheral neuropathy
What is the prognosis for Sjogren's syndrome?
-Usually good prognosis with normal life span
-Prognosis related to types of associated disease
-Risk of lymphoma
Who is fibromyalgia most common in?
women ages 20 – 50
What is fibromyalgia?
a common rheumatic syndrome
-Chronic widespread musculoskeletal pain syndrome with multiple tender points
What syndrome does fibromyalgia share many features with?
chronic fatigue syndrome (CFS)
What are 3 proposed causes of fibromyalgia?
Sleep disorders
Depression
Viral infections

however, cause is unknown
What are 5 conditions that fibromyalgia is a complication of (has an increased incidence for)?
Hypothyroidism
RA
Sleep apnea (in men)
osteoarthritis
lyme disease
What are the 3 components of the immune system overload ("the cup runneth over")?
stressful episode
injury
lingering illness
What are the associated symptoms with fibromyalgia? (10)
Chronic Fatigue Syndrome
Irritable Bowel Syndrome
Tension Headache
Migraine Headache
Primary Dysmenorrhea
Restless legs syndrome
Periodic limb Movement Disorder
Temporomandibular joint pain
Myofascial Pain Syndrome
What is the most common symptoms of fibromyalgia?
widespread pain throughout the body. It may start in the neck and shoulders and spread over time
-It may vary according to time of day, activity level, weather, sleep patterns and stress
What are the clinical findings with fibromyalgia?
-Chronic aching pain and stiffness, often involving the entire body but with prominence of pain around neck, shoulders, low back, and hips
-Fatigue, sleep disorders, subjective numbness, chronic headaches, and irritable bowel symptoms are common
According to the American College of Rheumatology, what does a person have to have in order to be diagnosed with fibromyalgia?
condition if there is a history of pain for at least 3 months in at least 11 of 18 specific tender point sites on the body
What are the "trigger points" of pain produced by palpation with fibromyalgia (PE is otherwise normal)?
trapezius
medial fat pad of knee
lateral epicondyle of elbow
What may a patient come in and say if the have fibromyalgia?
"I hurt all over"
-Widespread musculoskeletal pain
What quadrants are involved with fibromyalgia?
Each of 4 body quadrants involved (regional)
-Pain on left and right side of body
-Pain above and below waist
Is axial skeleton pain present with fibromyalgia?
yes
-Cervical spine
-Anterior chest
-Thoracic spine
-Low back
When is the pain the worse with fibromyalgia?
in the morning and at the end of the day
What labs should be done if fibromyalgia is suspected?
TSH
CBC
ESR
CK
sleep study
ANA
RF
How is fibromyalgia treated?
Patient education
Exercise daily
Stress reduction
Medications
Alternative/complimentary medicine
What meds. can be used to treat fibromyalgia? (5)
TCA

Pregabalin (Lyrica)
Amitriptyline (Elavil)
Fluoxetine (Prozac)
Chlorpromazine (Thorazine)
Cyclobenzaprine (Flexeril)q
What types of alternative/complimentary medicine can be done for fibromyalgia? (5)
Chiropractor
Massage
Osteopathic
Acupressure/acupuncture
biofeedback
Is fibromyalgia a progressive condition?
no
What is the prognosis of fibromyalgia?
Chronic symptoms
Can resume activities
Is cancer risk greater with polymyositis or dermatomyositis?
Dermatomyositis
What is polymyositis?
Inflammation of muscle fibers
Etiology unknown
WBCs invade muscles
Most commonly trunk/torso
Severe weakness
Flares and relapses
What are the associated diseases with polymyositis?
Lymphoma
Breast Ca
Ovarian Ca
Colon Ca
SLE
Scleroderma
RA
What is the most common symptom of polymyositis?
muscle weakness
What muscles are generally involved with polymyositis?
those that are closest to the trunk of the body
What are the S/S of polymyositis?
Muscle weakness
Onset – gradual/rapid
Muscle atrophy
Difficulty getting up from chair, stairs, raising arms over head
Muscle ache
Muscle pain with palpation
Fatigue
Weight loss
Low-grade fever
Skin involvement(dermatomyositis)
What is Gottron's sign, which is associated with polymyositis?
scaly reddish discoloration over the knuckles, elbows and knees
In childhood dermatomyositis, what can develop in the fatty layer of the skin?
hard lumps of calcium deposits
What can heart and lung involvement with polymyositis lead to?
irregular heart rhythm, heart failure, and shortness of breath
Who is polymyositis more common in?
females
middle childhood and 20s and elderly (bimodal distribution)
Is polymyositis a systemic disease?
it can be
-it is if it affects other areas of the body
What is the typical rash of dermatomyositis?
dusky, erythematous eruption over the face, perioral regions, neck, shoulders, chest, and arms
What is pathognomonic of dermatomyositis?
heliotrope suffusion
around the eyes
What is the diagnostic criteria for polymyositis?
four or more of the following:
-Proximal muscle weakness (trunk, thighs, shoulders)
-Increased serum CPK
-Muscle pain on grasping or spontaneously
-EMG myogenic changes
-Positive anti-Jo1 antibody
-Nondestructive arthritis or arthralgia
-Pathologic inflammatory signs
-Systemic inflammatory signs
(Temperature over 37 C at axilla, C-Reactive Protein increased, ESR >20 mm/hour)
How is polymyositis treated?
Prevent atrophy/contractures
treat dermatomyositis
Prednisone
Methotrexate
Azathioprine (Imuran)
IV immunoglobulin
Rituximab (B cell depletion therapy)
How is dermatomyositis treated?
pruritus management
topical corticosteroid
avoid sun exposure
What are vasculitis syndromes?
Heterogeneous group of disorders characterized by inflammation within the walls of affected blood vessels
How are vasculitis syndromes classified?
by size of vessel
What 2 conditions can occur with small vessel vasculitis syndrome?
Henoch-Schönlein purpura and Wegener’s granulomatosis
What condition can occur with medium vessel vasculitis syndrome?
polyarteritis nodosa
What 2 conditions can occur with large vessel vasculitis syndrome?
temporal arteritis
Behçet’s syndrome
What is the most common vasculitis seen in children?
Henoch-Schönlein Purpura
(also occurs in adults)
What are the 4 typical features of Henoch-Schönlein Purpura?
Palpable purpura
Abdominal pain
Arthritis
Hematuria
What happens with Henoch-Schönlein Purpura?
Leukocytoclastic vasculitis with IgA deposition
Where is the purpura usually located with Henoch-Schönlein Purpura?
typically on the lower extremities
How long does Henoch-Schönlein Purpura usually last?
Usually self-limited disease lasting 1-6 weeks
Who is Henoch-Schönlein Purpura more likely to become chronic in?
in adults --> persistent or intermittent purpura
How is Henoch-Schönlein Purpura treated?
Corticosteroids (some pts)
Azathioprinen (renal disease)
What is the (original) triad for Wegener’s granulomatosis?
upper respiratory disease
lower respiratory disease
GN
When should Wegener’s granulomatosis be suspected?
if nasal congestion or sinusitis are refractory to usual treatment
What is the pathology triad for Wegener’s granulomatosis?
small vessel vasculitis
granulomatous inflammation
necrosis
Wegener’s granulomatosis is a prototype of ___ associated disease.
ANCA (90%)
Wegener’s granulomatosis is generally fatal within ___ year(s) of diagnosis if not treated.
1
Who is Wegener’s granulomatosis most common in?
Men = women
Most common in 30s and 40s
What percentage of patients with Wegener’s granulomatosis present with upper or lower respiratory symptoms or both?
90%
How many months does Wegener’s granulomatosis develop over?
4-12 months
What are the upper respiratory symptoms that may show up with Wegener’s granulomatosis?
nasal congestion
sinusitis
otitis media
mastoiditis
gum inflammation
stridor
What are the lower respiratory symptoms that may show up with Wegener’s granulomatosis?
cough
dyspnea
hemoptysis
What are the S/S of Wegener’s granulomatosis?
-Migratory oligoarthritis
-Ocular disease
-Purpura or other skin lesions
-Renal disease
-Fever, malaise, weight loss
-upper/lower respiratory S/S
-Congestion/crusting/ulceration/perforation of nasal septum
-“Saddle nose” deformity
-Proptosis/ptosis/episcleritis
-DVT/PE are common
-Microscopic hematuria
-Segmental necrotizing GN with crescent formation
What do the labs for Wegener’s granulomatosis show?
Slight anemia
mild leukocytosis
ANCA (90%)
Hematuria with RBC casts
Bony sinus erosions
Lung granulomas

Histology shows vasculitis, granulomatous inflammation, necrosis, acute and chronic inflammation
What does a CXR or CT for Wegener’s granulomatosis show?
infiltrates, nodules, masses, and cavities
How is Wegener’s granulomatosis treated?
-Cyclophosphamide (3-6 months)
-Azathioprine or Methotrexate (maintain remission)

*Early treatment is crucial
Are TNF inhibitors effective with Wegener’s granulomatosis?
no
What type of HTN is involved with Polyarteritis Nodosa?
Renin-mediated
What is Polyarteritis Nodosa?
Necrotizing arteritis of medium sized vessels
What are 10% of Polyarteritis Nodosa cases caused by?
hepatitis B
What type of onset does Polyarteritis Nodosa have?
insidious with fever, malaise and weight loss
What occurs in the extremities with Polyarteritis Nodosa? (3)
arthralgia
myalgia
neuropathy
What are the typical skin findings with Polyarteritis Nodosa? (3)
livedo reticularis
subcutanous nodules
skin ulcers
What can Mesenteric vasculitis associated with Polyarteritis Nodosa cause?
acute abdomen
-abdominal pain w/ N/V
What do the labs for Polyarteritis Nodosa show?
Slight anemia and leukocytosis
Sed rate usually elevated
Assess for hepatitis B
Is there a disease specific serologic test for Polyarteritis Nodosa?
no
What is used to confirm diagnosis for Polyarteritis Nodosa?
biopsy and angiography
-Biopsy of involved organ is essential
-Angiography may show microaneurysms
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
What is the prognosis for Polyarteritis Nodosa?
-5 year survival rate is 20% without treatment
-With treatment, 5 year survival rate is 60 – 90%
Who is Behçet’s Syndrome most common in?
Asian, Turkish or Middle Eastern people
Does Behçet’s Syndrome involve arteries or veins?
both
What is “pathergy” phenomenon (Behçet’s Syndrome)?
sterile pustule at site of needle stick
What is the hallmark of Behçet’s Syndrome?
painful aphthous ulceration in the mouth
What are the S/S of Behçet’s Syndrome?
-Recurrent, painful aphthous ulcers of mouth and genitals
-Tender papular lesions that mimic erythema nodosum that tend to ulcerate
-Pathergy phenomenon
-Nonerosive arthritis of knees and ankles
-Uveitis may be severe
-Increased thrombotic events
-CNS involvement
How may the CNS be involved with Behçet’s Syndrome? (4)
Aseptic meningitis
Cranial nerve palsies
Seizures
Spinal cord lesions
How is Behçet’s Syndrome treated?
Corticosteroids
azathioprine
Cyclophosphamide (severe ocular/CNS disease)
Are Giant Cell Arteritis & Polymyalgia Rheumatica
a spectrum of the same disease?
yes
What is the clinical diagnosis of Polymyalgia Rheumatica based upon?
pain and stiffness of the shoulder and pelvic girdle
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
Is the sed. rate increased or decreased with Polymyalgia Rheumatica?
elevated > 50
How is Polymyalgia Rheumatica treated?
Prednisone
Methotrexate (some patients)
Are disease flares common with Polymyalgia Rheumatica?
yes
What is one big difference b/t fibromyalgia and chronic fatigue syndrome?
-pain is major component with fibromyalgia
-fatigue is major component with chronic fatigue syndrome
With deep palpation of trigger points in fibromyalgia, is the pain localized or does it radiate?
stays localized
Is fibromyaliga unilateral or bilateral?
bilateral
Does fibromyalgia affect the upper or lower body?
both
Is fibromyalgia symmetrical?
yes
Is Henoch-Schonlein purpura blanching or non-blanching?
non-blanching (will still be red when pushed on)