Bone & Joint Midterm 2

What is the MC seronegative inflammatory arthritis?

JRA

What are the different arthritides of Juvenile Chronic Arthritis? Which is most common?

-Juvenille Onset Rheumatoid Arthritis

-Seronegative Chronic Arthritis (MC)

-Juvenile Onset Ankylosing Spondylitis

-Juvenile Onset Psoriatic Arthritis

-Juvenile Onset Enteropathic Arthritis

What are the Juvenile seronegative chronic arthritis from causing the most severe systemic issues to the most rare?

1.Classic Systemic Form (Still's Disease)

2. Polyarticular

3. Pauciarticular

What is the definition of JRA?

-childhood (<16 yrs) onset of adult RA

What % of JRA is seropositive, what % is seronegative?

10-15% Seropositive

85-90% Seronegative

What does the diagnosis of JRA require?

6+ weeks of objective synovitis (primer)

What % of JRA patients have Still's Disease?

20

Is Still's Disease seen more commonly in males of females?

equal distribution

What are the systemic manifestation of Still's Disease?

-high intermittent fever, lymphadenopathy, hematosplenomegaly (enlarged liver and spleen)

-Carditis, leujocytosis, anemia

-fleeting, migratory rash-trunk face, extremities

What are the symptoms in the joints with Still's Disease?

-pain and swelling w/in weeks, months

What do you see on x-ray with stills disease?

no changes

What % of stills disease becomes severe? When does this happen?

25%, becomes chronic arthritis after systemic manifestations are gone

How man joints are affected with polyarticular JRA?

4 or more

What % of JRA patients are Polyarticular?

40%

Is polyarticular JRA more common in boys or girls?

girls 2x more

Will polyarticular JRA have severe, mild to moderate or rare systemic signs (fever, lymphadenopathy, rash)?

mild to moderate

How does polyarticular JRA present in the joints?

-bilateral

-symmetric pain and swelling

Where are the common sites of polyarticular JRA?

-MCP jts and wrists

-feet

-ankles and knees

How many joints are affected with pauciarticular JRA?

2-4 joints (less than 4)

What % of JRA will be pauciarticular/monoarticualr JRA?

40%

Is Pauciarticular/monoarticular JRA more commmon in girls or boys?

girls 3x more

Is Pauciarticular/monoarticular JRA severe, mild to moderate or rare systemic signs (fever, lymphadenopathy, rash)?

rare

Pauciarticular/monoarticular JRA may be complicated by inflammation of the ___

eye 10-50% of pts

Where does Pauciarticular/monoarticular JRA most commonly affect?

knee

-insidious onset of pn, swelling, stiffness

What are the lab findings with JRA?

-non diagnostic (-RF)

-active acute stages - elevated ESR & C-reactive protein

With JRA, what lab finding leads to a worse prognosis?

+RF 10-15%

What is the prognosis with JRA?

good, complete remission is 75% patients

What is the treatment for JRA?

maintain jt position, muscle function & strength

Are systemic manifestations more common in JRA or ARA?

JRA

Are subcutaneous nodules more common in JRA or ARA?

ARA

Are growth abnormalities more common in JRA or ARA?

JRA

With lab findings, is the rheumatoid factor more commonly seen with JRA or ARA?

ARA

What form of arthritis can SLE cause?

inflammatory

What does SLE stand for?

systemic lupus erythematosus

What is the definition of SLE?

autoimmune, generalized inflammation of connective tissue

SLE involves multiple ___, ie skin, joints, kidneys

organ systems

SLE injury is principally a result of ___

vasculitis

Is SLE acute or chronic? What is it characterized by?

Chronic

characterized by remissions and exacerbations

What is the epidemiology of SLE?

women of childbearing years (90% of pts, 20-30s)

1:2000 in pop

What is the etiology of SLE?

unknown

What are the theories of etiology for SLE?

genetic/environmental factors

-drugs

-viruses

-sex hormones

What is the pathophysiology of SLE?

-defect in the regulatory mechanisms that sustain self-tolerance and prevent autoimmune attacks on body's tissues

-Antinuclear antibodies (ANA)

-lesions

What are the lesions with SLE caused by?

deposition of immune complexed and fibrinoid material in tissue and by direct attack of autoantibodies

Vasculitis with SLE can lead to ___

-glomerulonephritis

problems with:

-skin

-joints

-CNS

-heart

-lungs

Glomerulonephritis causes what symptoms?

-hematuria

-proteinuria

-hypertension

What is the MOST common cause of death with SLE?

kidney failure

What happens with skin with SLE?

degeneration of basal layer of skin -> rash

What happens with the joints with SLE?

non-erosive synovitis

What is the chief complaint in 40% of patients with SLE?

joint pain, polyarthralgias

What happens in the CNS with SLE?

acute vasculitis -> infarcts, hemorrhages

What happens to the heart with SLE?

non bacterial endocarditis

What happens to the lungs with SLE?

pleuritis, pleural effusion

What are the initial signs and symptoms with SLE?

fever, malaise, anorexia, wt loss, rash (worse w/ sunlight)

T/F SLE patients can have generalized alopecia during active phase

true

What is the name sign for the rash with SLE?

Butterfly/Macular Rash

Arthritis affects up to __% of pts with SLE

90%

How does the arthritis present in patients with SLE?

bilateral and symmetric pain, swelling and stiffness

-laxity in joints

Where is the arthritis with SLE commonly seen in the body?

-hands

-knee

-wrist

-shoulder

What are the x-ray findings with SLE?

-normal jt space

-reversible deformities (non-rigid)

-osteoporosis

-ST atrophy, Ca+ deposits

Although spinal manifestations are rare with SLE what can occur in <5% of pts at the C1/C2 joint?

instability

What are the lab findings with SLE? What is the main one?

ANA-antinuclear antibodies (main) 99% of pts

LE cells 70%

What is the treatment for severe cases of SLE?

corticosteroids

What can chronic use of corticosteroids lead to?

-osteoporosis and spinal compression fracture

-gastric/duodenal ulcers

What is an AKA for scleroderma?

progressive systemic sclerosis

What is the definition of scleroderma?

generalized inflammation of CT characterized by excessive fibrosis throughout the body

What parts of the body can scleroderma affect?

-skin

-lungs

-heart

-GI tract

-MS system

-kidneys

What is the epidemiology of scleroderma?

-females 3x more

-30-50 yrs

-increases with age

-worldwide

What is the etiology of scleroderma?

unknown

autoimmune

What is the pathophysiology of scleroderma?

-tissue inflammation -> small vessel vasospasm and fibrosis

-deposition of fibrous tissue=decreased lumen size

-loss of vascularity

-induration (hardening)

What are the 2 different clinical presentation of scleroderma? Which is most common?

-diffuse scleroderma

-CREST phenomenon (MC)

What are the characteristics of diffuse scleroderma?

-early widespread skin involvement

-early visceral involvement

-rapid progression

What are the characteristics of CREST phenomenon?

-limited skin involvement

-later visceral involvement

-slow progression

What happens with skin in patients with scleroderma?

-edema

-progressive fibrosis dermis

-thinning of epidermis

-atrophy of dermal appendages

-autoamputation

-increase in melanin

-subcutaneous calcium depositis

Where does scleroderma begin and progress in the upper extremities, neck and face?

begins distally and progresses proximally

Is scleroderma unilateral/bilateral, symmetrical/asymmetrical?

bilateral symmertrical

What is the most common cause of death in scleroderma?

lungs

70% of patients with scleroderma have lung involvement what is the affect in the lungs?

impaired gas exchange due to fibrosing alveoli

What happens in the GI tract with scleroderma?

-atrophy and replacement of muscularis layer w/fibrotic tissue

-lower 2/3 esophagus become rigid and narrow->dysphagia

-small intestine and colon->malabsorption

What happens in the MS system with scleroderma?

-inflammatory synovitis and fibrosis

-fingers and knees

T/F joint destruction is common with scleroderma

false

What happens in the kidneys with scleroderma?

-intimal thickening of vessel walls->cortical ischemia->acute renal failure

-30% of pts develop hypertension

What is the first clinical finding with scleroderma?

skin changes

-may begin with Raynaud's phenomenon

What are the clinical findings with scleroderma?

-skin changes

-rheumatoid like pain and stiffness in knees and finger jts

-dysphagia in 50%

What do the letters stand for in CREST phenomenon?

Calcinosis (calcific deposits in ST)

Raynauds

Esophageal dysmotility

Sclerodactyly (tight flushing)

Telangiectasia (spider veins)

What are the x-ray findings with scleroderma?

-resorption of the fingers w/ retraction of tips

-resporption of distal tufts of the distal phalanges-> sharp, tapered

-usually normal jt space

-Calcinosis of soft tissue in 20%

50% of patients with scleroderma have lab findings with what elevated immunoglobulin?

IgG

What are the lab findings with scleroderma?

-elevated IgG

-+RF

-ANA

-Scl-70

-Anti-centromere Ab

What are the treatments with scleroderma?

-take care of skin-avoid cold, dry, tobacco

-chew careful

-protect from infection

-NSAIDS

65% 10 year survival

What are the 4 common seronegative arthopathies?

Psoriatic Arthritis

Enteropathic Arthropathy

Ankylosing Spondylitis

Reiter's Syndrome (reactive arthritis)

What is the definition of psoriatic arthritis?

chronic skin disorder characterized by proliferation of the epidermis

5-8% have associated arthritis

What is the epidemiology of psoriatic arthritis?

-.2-.3% of population have psoriasis.

-Mean age of onset is 27 years but can affect anyone.

-Onset of psoriatic arthritis can be up to 20 years later.

What is the etiology of psoriatic arthritis?

unknown

-possible genetic factor, trauma, infection, stress

What is the clinal signs of psoriatic arthritis on the skin and nails?

-erythematous patches covered with silvery scales on extensor surfaces (elbow, knee, scalp, back)

-thimble pitting, thickening, discoloration of nails (40% psoriasis pts, 80-90% PSA pts)

What is the pathognomic sign of psoriatic arthritis on the skin?

Auspitz sign

-remove scales=pinpoint bleeding

What is the pathognomic sign of psoriatic arthritis on the nails?

thimble pitting

What is the classic presentation of psoriatic arthritis?

1 large jt and 1-2 interphalangeal joints with dactylitis "sausage digit"

What are the x-ray findings with psoriatic arthritis?

-st swelling, erosions and bone proliferation

-marginal bone erosions tapering to a pencil in cup deformity

-fluffy new bone adjacent to marginal erosions due to periostitis

What is the frequent sequale of psoriatic arthritis?

ankylosis, especially IP jts of hands and feet

What is the name sign of psoriatic arthritis from fluffy periosteal new bone formation?

Mouse Ear Erosions

What are the SI joint x-ray findings with psoriatic arthritis?

-30-50%

-erosions, reactive sclerosis

-BL and asymmetrical sacrolitis