Bone and Joint Pathodiagnosis Week 4

What is the age range of "Juvenile"?

16 yo and under

How long must synovitis persists in order to diagnose juvenile rheumatoid arthritis?

6 weeks

What % of JRA cases are Sero positive?

10-15%

What % of JRA cases are sero negative?

80-85%

What are the 3 types of sero negative JRA?

Classic systemic form

Polyarticular

Pauciarticualr/monoarticular

What is an AKA for classic systemic JRA?

Still's disease

What % of sero negative JRA does Still's disease make up?

20%

What gender is most affected by still's disease?

It has equal gender distribution

Migratory rash is a defining symptom of which form of JRA?

Still's disease

T or F: Still's disease has affects joints unilaterally and has systemic manifestations

True

What % of sero negative JRA does polyarticular JRA make up?

40%

What gender does polyarticular JRA affect most?

girls (2:1)

How many joints must be affected to diagnose polyarticular JRA?

>4

T or F: polyarticular JRA has systemic manifestations and affects joints unilaterally

False (it has systemic manifestations, but affects joints bilaterally)

What % of sero negative JRA does pauciarticular/monoarticular JRA make up?

40%

What type of JRA is diagnosed when 1 joint is affected?

monoarticular

What type of JRA is diagnosed when 2-4 joints are affected?

pauciarticular

T or F: Pauciarticular/monoarticular JRA commonly has systemic manifestations

False (it rarely has systemic manifestations)

What % of Pauciarticular/monoarticular JRA cases are complicated by inflammation of the eye?

5-10%

What form of JRA often affects the knee joint?

Pauciarticular/monoarticular JRA

T or F: Sero positive JRA patients tend to have a worse prognosis

True

T or F: JRA generally has a good prognosis

True

What is the total population incidence of Systemic Lupus erythmatosus?

1:2000

What demographic is most affected by SLE?

women in childbearing years (90%)

What is the hallmark lab finding of SLE?

Antinuclear antibody (ANA)

What is the most common cause of death in SLE patients?

kidney failure

What are the signs of glomerulonephritis in SLE patients?

hematuria and proteinuria

What causes the rash of SLE?

Degeneration of the basal layer of the skin

What % of SLE patients have non-erosive synovitis?

90%

What are the complications of CNS Vasculitis in SLE?

acute vasculitis, leading to infarction and hemorrhage

What are the complications of heart vasculitis in SLE?

non bacterial endocarditis

What are the complications of lung vasculitis in SLE?

Pleuritis and pleural effusion

What % of SLE patients have joint pain?

40%

What are the initial symptoms of SLE?

Constitutional (fever, malaise, anorexia)

What part of the body does the erythematous rash of SLE affect?

face, neck, elbows, and dorsum of the hand

T or F: the rash of SLE is bilaterally symmetrical

True

What is the AKA for the butterfly rash of SLE?

Macular rash

What % of SLE patients have a macular rash?

90%

When does generalized alopecia occur in SLE patients?

during the active phase

What characterizes the chronic phase of SLE?

exacerbations and remissions

What is the most common manifestation of SLE in the spine?

C1/C2 instability (5% of the time)

T or F: the deformities caused by SLE are non-rigid

True

What % of SLE patients are positive for FANA (fluorescent antinuclear antibody)

99%

What % of SLE patients are positive for LE (lupus erythmatosus) cells?

70%

What treatment is used in severe cases of SLE?

corticosteroids

What type of tissue is primarily affected by scleroderma?

connective tissue

What gender is most affected by scleroderma?

Females (3:1)

What is the most common age range affected by scleroderma?

30-50 yo

T or F: the incidence of scleroderma declines with age

False (the incidence increases with age)

What complications arise from tissue inflammation in scleroderma patients?

small vessel vasospasms and fibrosis

What complications arise from deposition of fibrous tissue in scleroderma patients?

decreased size of lumen

What form of scleroderma has early widespread skin involvement, early visceral involvement, and rapid progression?

Systemic sclerosis

What form of scleroderma has limited skin involvement, later visceral involvement, and slower progression?

CREST phenomenon

What does CREST stand for?

Calcinosis, raynaud's phenomenon, esophageal dysmotility, scleroadctyly, and telangiectasis

What is the name of calcium deposits under the skin?

calcinosis

What is the defining characteristic of Raynaud's phenomenon?

Skin that turns, white, then blue, then red due to excessive cold response

What is an AKA for thinning skin?

Sclerodactyly

What is telangiectasis?

spider veins

What % of scleroderma patients experience calcium deposits under the skin?

20%

T or F: scleroderma begins distally in the upper extremity and progresses proximally

True

T or F: scleroderma is typically unilateral

False (bilaterally symmetrical)

What is the most common cause of death in scleroderma patients?

impaired gas exchange due to fibrosis of the alveoli (70%)

What are the complications of scleroderma affecting the GI tract?

dysphagia (due to narrowing of the esophagus) (50%)

malabsorption (due to atrophy and fibrosis of the small intestine)

T or F: joint destruction is a common occurrence in scleroderma

False (it is uncommon)

When joint destruction occurs in scleroderma, which joints are most affected?

Fingers and knees

What are the complications of scleroderma affecting the kidneys?

Cortical ischemia and acute renal failure

What % of scleroderma patients have hypertension due to complications of the kidney?

30%

What was formerly the most common cause of death of scleroderma?

Kidney failure

What is the first organ to change in scleroderma?

The skin

What is a common x-ray finding in the hands of a scleroderma patient?

Resorption of distal tufts of the distal phalanges

T or F: Scleroderma patients typically have normal joint spaces

True

What antibody is often elevated in scleroderma patients?

IgG (50%)

What portion of scleroderma patients are positive for rheumatoid factor?

20-30%

What % of scleroderma patients are positive for antinuclear antibody?

40-50%

Which antibodies are unique to scleroderma?

Scl-70 and anticentromere Ab

T or F: treatment for scleroderma is palliative, not curative

True

What is the survival duration for most scleroderma patients?

10 years (65%)

What are the 4 seronegative arthropathies (PEAR)?

Psoriatic Arthritis

Enteropathic arthropathy

Ankylosing Spondylitis

Reiter's syndrome

What are the AKAs for Ankylosing spondylitis?

Bechterew disease

Marie strumpell disease

What are the effects of ankylosing spondylitis on the spine and SI joints?

ossification, fibrosis, and fusion

What is an insertion of a ligament or capsule into bones?

Enthesis

What race is most affected by ankylosing spondylitis?

caucasians

What gender is most affected by ankylosing spondylitis?

Even gender distribution

What age group is most affected by ankylosing spondylitis?

young adults 10-30 yo

What is the strongest associated genetic factor in the development of AS?

The HLA B27 gene

What % of Northern Europeans have the HLA B27 gene?

8-9%

What % of people with the HLA B27 gene develop AS?

5%

T or F: AS affects the upper extremity more than the lower extremity

False (it affects the lower extremity more)

What is the name of inflammation of the SI joint?

Sacroiliitis

What segmental subluxation is caused by AS?

Atlanto-axial

What % of people with both reiter's syndrome and AS suffer from prostatitis?

80%

How long must someone suffer from low back pain and stiffness to be diagnosed with AS?

3 months

Where does AS start?

SI joint

What is the direction of progression of AS?

Up the spine

What % of people with AS have the HLA B27 gene?

90-95%

What are the potential locations of AS?

SI joints

Spine - discs, apophyseal joints

Femoroacetabular joints

Pelvis

Knees

Hands

Shoulders

Chest

Heart

Which side of the SI joint is more affected by AS?

The lower third on the iliac side

What is the term for loss of joint definition as seen on X-ray in AS patients?

pseudowidening

T or F: AS appears at the T/L junction before the lumbar spine most of the time

True

What is the name of vertical bridging found in AS (spinal ligament ossification)?

syndesmophytes