post midterm 4: spondylo

Question 1

What causes spondyloarthropathy?

Answer 1

Pathogenesis uncertain; genetic factors play a role

**HLA-B27 association
HLA-B27 is present in up to 88% of patients with uveitis
HLA-B27 is present in 80-90% of patients with ankylosing

Question 2

spondyloarthropathy affects what pops?

Answer 2

male more than female and caucasian

Age < 45

Question 3

spondyloarthropathy shows up as what on lab findings?

Answer 3

No typical serologic abnormalities “seronegative”

*CRP often elevated

Question 4

what does spondyloarthropathy affect?

Answer 4

*Large axial joints (sacroiliac, vertebral) develop endochondral bone formation and joint fusion.
-similar to tendonitis
*Peripheral arthritis = Non-erosive
[Monoarthritis or oligoarthritis (few joints)]

*Associated inflammation
Ocular, aortic, lung, skin
Fatigue, fevers

Question 5

what are some Criteria For Spondyloarthropathy ?

Answer 5

*Age < 45
*Inflammatory back pain
*Sacroiliitis on imaging
HLA-B27
*Enthesopathy=attachment of a tendon or ligament to a bone
Synovitis (predominantly lower extremity)
Dactylitis
Uveitis
Psoriasis
*Inflammatory bowel disease
Recent urethritis, cervicitis, or acute diarrhea
Positive family history

Question 6

describe the back pain assoc with Ankylosing Spondylitis? how long does it last, what improves it?

Answer 6

Inflammatory back pain, insidious onset (months or longer), stiffness
*often improves with movement
Sacroiliitis, usually progressive
Vertebral disc/ligamentous involvement -> ankylosis

Question 7

T/F: Ankylosing Spondylitis can involve heart and lungs

Answer 7

True:
Cardiac involvement (10%)
Aortic root dilatation, aortic insufficiency
Cardiomegaly, conducting system abnormalities
Pulmonary fibrosis (cough, dyspnea)

Question 8

what kind of Enthesitis does Ankylosing Spondylitis

Answer 8

Enthesitis: Fasciitis (heel pain), costochondritis (chest pain), tendinitis

Question 9

Features of inflammatory spinal pain vs mechanical back pain:

Answer 9

Insidious onset
Improved with exercise
Morning stiffness and pain at night: same as mechanical…
At least 3 months duration

Question 10

How do you dx Ankylosing Spondylitis?

Answer 10

*Assess spinal mobility (Schober test) 
Diminished in AS
*X-rays  
Sclerosis
Joint-margin blurring
Ankylosis

Question 11

what is often the first indication of undiagnosed spondyloarthropathy?

Answer 11

ocular symptoms:

Spondyloarthropathy present ~ 50% pts with recurrent, unilateral anterior uveitis

Question 12

describe the common ocular associations with Ankylosing Spondylitis?

Answer 12

Nongranulomatous anterior uveitis

• Acute onset (over several days)
• Initial episode unilateral, recurrences in either eye
• Recovery generally in 2 to 3 months

Other findings of ocular inflammation

• Conjunctivitis
• Scleritis
• Keratitis

Recovery is often complete with overall good prognosis
Recurrence is common; late complications can occur (cataracts, glaucoma)

Question 13

what are some complications with progressive spinal akylosis?

Answer 13

1) Progressive spinal ankylosis
- Limited spinal mobility
- Postural deformities – neck flexion
- Impaired vision
- Impaired lung capacity
- Neurological syndromes / compression
2) Long term ocular complications (cataract, glaucoma)
3) Bone loss / osteoporosis
4) Cardiac
5) Aortic insufficiency
6) Coronary artery disease – increased risk

Question 14

how do you tx Ankylosing Spondylitis?

Answer 14

• PT
• surgery

NSAID’s (high doses)
Often effective alone

Glucocorticoids
Systemic short term, local injection, uveitis

Question 15

what is Reactive Spondyloarthropathy (Reiter’s Syndrome)? what causes it?

Answer 15

-Inflammatory disorder of unknown cause

-Symptoms follow within weeks after infection with enteric or urogenital pathogens
-Association seen with HIV infection
Sometimes no pathogen identified (though history of diarrhea)

Question 16

what are some Clinical Features of Reactive Spondyloarthropathy?

Answer 16

Oligoarticular arthritis
Enthesitis
Ocular inflammation
Mucocutaneous lesions
Genital inflammation: Urethritis, balanitis, cervicitis, prostatitis, cystitis
Pericarditis
Constitutional symptoms: fever, weight loss

Question 17

what kind of arthritis do you see in Reiter’s?

Answer 17

Oligoarticular arthritis

• Large joints of lower extremities most often
• Small joints of hand / dactylitis (sausage digits)
• Ascending spinal arthropathy (less commonly)

Question 18

what kind of Ocular inflammation do you see with Reiter’s?

Answer 18

Most commonly conjunctivitis

Less often: anterior uveitis, episcleritis, corneal ulcers

Question 19

what kind of Enthesitis do you see with Reiter’s

Answer 19

Often Achilles / heel

Question 20

what kind of mucocutaneous lesions do you see with Reiter’s?

Answer 20

-Painless oral ulcers
-Keratoderma blenorrhagica
[Pustular lesions progress to hyperkeratotic, scaling lesions]
-Erythema nodosum
Painful erythematous lesions on legs

Question 21

what is the prognosis for Reactive Spondyloarthropathy/Reiter’s?

Answer 21

Prognosis
> 50% resolve within 6 months
> 6 months “chronic”
Majority resolve by 12 months
Chronic arthritis and longer term residual pain can persist; arthritis can recur

Question 22

what is Juvenile Chronic Spondyloarthropathy? when is the onset? what gender does it affect?

Answer 22

Group of inflammatory joint disorders in children
-Onset age range often 2nd decade / adolescence
-More common in boys
HLA-B27 association
-Arthritis and enthesitis - usually lower extremity joints
-May initially be diagnosed as isolated injury (tendinitis, etc)
-Sacroiliitis and spinal arthritis may develop later
-Uveitis may occur
-Systemic symptoms
-Fever, weight loss, weakness, anemia, leukocytosis
Symptoms/findings of colitis or psoriasis should be sought

Question 23

what is psoriasis?

Answer 23

Dermatological disease with:
Hyperproliferation of epidermis
Plaque form most common
Red, inflamed lesions covered with silvery white scales

Question 24

T/F: psoriasis is assoc with uveitis and arthritis

Answer 24

True.

Can be triggered by Infections (strep, HIV), stress, skin injury

Question 25

what is Psoriatic Arthritis? describe the arthritis and uveitis that accopanies it

Answer 25

Ascending spinal arthropathy, sacroiliitis
*Associated with uveitis (20%)
Large and small joints, sausage-shaped digits
Characteristic nail changes associated with arthropathy
Arthritis may be destructive, with erosions, ankylosis
Acute or chronic nongranulomatous uveitis

Question 26

compare uveitis you get with psoriasis vs IBD

Answer 26

Psoriasis: Rash and joint Sx usually precede ocular disease
Bowel disease (Crohn’s / UC): Uveitis may precede bowel Dx in 50% or more

*bilateral in both

Question 27

what is sarcoidosis?

Answer 27

A multisystem granulomatous disease of unknown etiology that may affect any tissue but most prominently involves the lungs

Question 28

sarcoidosis affects what pops?

Answer 28

In US higher prevalence in African Americans
Worldwide, 80% of people with sarcoidosis are Caucasian

Women : men = 2 : 1
Onset usually age 20-40

Question 29

what are some Clinical Features of Sarcoidosis?

Answer 29

**involves many organs
Constitutional symptoms
Lymphadenopathy (75%), splenomegaly (25%)
**Pulmonary inflammation (>90%)
Skin lesions
Liver inflammation (90%), *hepatomegaly (20%)
Cardiac infiltration (rare)
*Salivary and lacrimal enlargement (6%)
Neurologic syndromes (15%)
*Musculoskeletal and rheumatic manifestations (myositis, arthritis)
Hypercalcemia
Ocular inflammation (20%, presenting complaint 5%)

Question 30

what kind of skin changes can you see in sarcoidosis?

Answer 30

Erythema nodosum= favorable prognosis
Maculopapular eruption=Central face and neck
-Sites of trauma/tattoos
Violaceous discoloration of nose, cheeks, chin, ears (lupus pernio)
Hyper-/hypo-pigmentation
Focal alopecia

Question 31

what is Lofgren’s syndrome?

Answer 31

Female with:

- Erythema Nodosum
- Hilar Adenopathy
- Migratory Polyarthritis
- (HLA-DQB1*0201) 

Good prognosis, likely to spontaneously remit
*subset of sarcoidosis

Question 32

***what is Heerfordt’s Syndrome (“Uveoparotid Fever”)?

Answer 32

Fever
Painless salivary, lacrimal enlargement
Uveitis
**VIIth, other cranial nerve palsies
**Keratoconjunctivitis sicca

*subset of sarcoidosis

Question 33

**what are some Neurologic Complications

assoc with sarcoidosis?

Answer 33

Cranial nerve lesions (VII)

EOM palsies, optic neuritis, chiasmal syndromes

Internuclear ophthalmoplegia

Meningitis, seizures, hydrocephalus

Hypothalamic/pituitary dysfunction

Peripheral motor/sensory deficits

Question 34

list some assoc ocular comp of sarcoidosis

Answer 34

Usually begins unilaterally; often becomes bilateral
-Anterior granulomatous uveitis
(acute less frequent but more often symptomatic)
-“Mutton-fat” keratic deposits
-May be benign, but cataracts, glaucoma can occur (especially if chronic / recurrent)
-Cystoid macular edema – generally resolves; longer course of inflammation may lead to visual impairment
-Scleritis, episcleritis
-Conjunctival granulomas, conjunctivitis, band keratopathy

Posterior uveitis, vitritis, retinal vasculitis
“candle wax” lesions
Retinitis; can cause neovascularization (sea fan appearance)
Vitritis
Orbital infiltration, exophthalomos, papilledema
Lid involvement by skin lesions

Question 35

how do you dx sarcoidosis?

Answer 35

clinical features:

• Asymptomatic hilar adenopathy
• Unexplained hypercalcemia, kidney stones
• Uveitis

CXR maybe

Pulmonary function testing

Biopsy demonstrating noncaseating granulomas

Exclusion of mycobacterial, fungal infection, lymphoma, etc.
*can be hard to diff bw this and TB early on

Question 36

can you dx sarcoidosis with lab testing?

Answer 36

there is no single test to dx it, some findings include:

• Elevated ACE level (75%) – nonspecific and of questionable value
• Hypercalcemia with elevated 1,25 dihydroxy-vitamin D level
• Elevated ESR
• Leukopenia (5-10%)
• Eosinophilia (25%)
• Elevated alkaline phosphatase
• Elevated transaminases
• Positive rheumatoid factor

Question 37

how do you tx sarcoidosis?

Answer 37

Treatment not always required

• NSAIDs helpful for constitutional symptoms
• *Glucocorticoids are mainstay of therapy

Question 38

what is prognosis of sarcoidosis?

Answer 38

Generally good
2/3 of patients disease remits
Usually within 2 years