Connective Tissue and Joint Disease

Question 1

____ is the most common CT disease in kids

Answer 1

JRA

Question 2

Which of the JRAs is the most common?

Answer 2

Pauciarticular Type I - early onset

Question 3

Name the 5 types of JRA

Answer 3

1. Polyarticular RF neg
2. Polyarticular RF pos
3. Pauciarticular Type I early onset
4. Pauciarticular Type II late onset
5. Systemic onset (Still’s disease)

Question 4

Which of the JRAs have sacroillitis

Answer 4

Pauciarticular Type II late onset

Question 5

which of the JRAs have iridocyclitis? (2)

Answer 5

Pauciarticular Type 1 (early onset) - 50% chronic

Pauciarticular Type 2 (late onset) 10-20% acute

Question 6

Disease?

acute onset fever, rash, hepatosplenomegaly, lymphadenopathy, arthritis, fatigue, myalgia, irritability, pericarditis

Answer 6

systemic onset JRA (Still’s disease)

systemic symptoms may preced arthritis by several months

Question 7

How does cyclosporine work?

Answer 7

blocks production of IL2 and proliferation of synovial T-cells

Question 8

Proper splinting of upper extremity in JRA

Answer 8

functional position with wrist in 15-20degrees extension, fingers in some flexion, 25% at MCP joint and a few degrees at the PIP joints; ulnar deviation controlled, and thumb in oppostion

Question 9

What are the four classifications in amarican college of rheumatology revised criteria for classification of functional status in RA?

Answer 9

class I - completely able to perform usual ADLs
Class II - able to perform usual self-care and vocational activities but limited in avocational
Class III - able to perform usual self-care activities but limited in vocational and avocational
Class IV - limited in ability to perform usual self-care, vocational, and avocational activities.

Question 10

Name the 4 juvenile onset seronegative spondyloarthropathies

Answer 10

1. ankylosing spondylitis (AS)
   2,. reactive arthritis (formerly Reiters syndrome)
2. arthritis with irritable bowel disease
3. psoriatic arthritis

Question 11

Name the organisms associated with reiters syndrome (reactive arthritis) a seronegative arthritis of juvenile assumption

Answer 11

Chlamydia trachomatis, chlamydia pneumoniae, salmonella, shigella flexneri, yersinia enterocolitica

Question 12

Name the 6 things in SLE that are associated with poor outcomes

Answer 12

1. hematuria
2. proteinuria
3. persistent hypertension
4. Pulm HTN
5. chronic active disease
6. biopsy-proven diffuse proliferative glomerulonephritis

Question 13

Name the diagnostic criteria for SLE
must have 4 of 11 of these things
(90% sensitive, 98% specific)

Answer 13

1. malar rash
2. discoid lupus rash
3. photosensitivity
4. oral or nasal mucocutaneous ulceration
5. non-erosive arthritis
6. nephritis
7. encephalopathy
8. pleuritis or pericarditis
9. cytopenia
10. positive immunoserology: LE cells, antinative DNA antibodies, anti-SM antibodies, falst _ test for syphilis.
11. positive ANA titer

Question 14

Name the 4 types of juvenile scleroderma

Answer 14

1. Morphea 0 skin, and small lesions occur with minimal sclerosis (guttate morphea) - self limited after 2-3 years
2. systemic sclerosis
3. overlap syndromes - includes mixed CT disease whichhas features of SLE, RA, dermatomyositis, and scleroderma
4. OTHER - CREST

C calcinosis
R raynauds
E esophageal abnormalities
S sclerodactyly
T telangiectasia

Question 15

what criteria is used to diagnose rheumatic fever?

Answer 15

Jones criteria

1. MAJOR: carditis, polyarthritis, chorea, erythema marginatum, subcut nodules
2. MINOR: fever, arthralgia, elevated ESR/CRP, Prolonged PR interval
3. Preceding group A strep infection: throat culture, rapid strep antigen, elevated strep ab.

Question 16

Name the hallmark of hemophilia

Answer 16

hemarthrosis

Question 17

Name the three most common hemophilias

Answer 17

A - VIII deficiency (classic)
B - IX deficiency (Christmas hemophillia)
C XI deficiency

Question 18

Describe severity classification for hemophilia

Answer 18

Mild disease - presents with > 5% factor activity
Mod disease - >1% factor activity
Severe disease - <1% factor activity