Rheumatology

Question 1

Juvenille Idiopathic Arthritis: Definition and 3 subtypes

Answer 1

Arthritis that persists for 6 weeks and in a child <16 years of age.

Oligoarticular: more common in females if ANA positive than increased risk for asymptomatic uveitis.
<4 joints affected. Knees, ankles and wrists
Treatment: NSAIDS and Intra articular injections

Polyarticular: RF + or -; acts more like rheumatoid arthirtis and affects the smaller joints.>5 joints are affected

Systemic: 2 weeks of Fever + 1 of the following:
Salmon patch rash (evanescent type rash)
Lymphnodes
HSM
Serositis 

Rash Fever and Arthritis

Question 2

Enthesitis related Arthritis

Answer 2

More common in males than in females

If HLA-B27 + then more likely to have symptomatic uveits. (anterior)

Question 3

Psoriatic Arthritis

Answer 3

The arthritis or the psoriasis can present first.
Need to be careful about using systemic steroids as it can lead to a pustular psoriasis.
Can present as dactylytis in younger children.
High ANA +

Question 4

Acute Rheumatic Fever - Diagnosis

Answer 4

Jones Criteria
2 major OR 1 major and 2 minor.

Joints - migratory arthritis
o - cardiac - valvulopathy, carditis, prolonged pr interval
nodules
erythema marginatum 
syndenham chorea 

Minor Criteria: 
Fever 
Prolonged PR Interval 
Elevated ESR 
Arthralgia

Question 5

Rheumatic Fever Prophylaxis

Answer 5

Carditis with Valvulopathy: for life (or until about 40 years of age); minimum 10 years
Carditis no valvulopathy: 10 years or until 21 years of age
No Carditis: 5 years or until 21 years of age

Question 6

SLE Criteria for Diagnosis

Answer 6

MD SOAP BRAIN

Malar Rash, Discoid Rash, Serositis, Oral ulcers, Antibodies (ANA, Anti DSDNA) Photosensitivity, Blood abnormalities, Renal issues, Arthritis, Immune disorder (low C3 and C4), Neurological manifestations

Question 7

Treatment of SLE

Answer 7

Hydroxychloroquine
Steroids
Ritixumab
MMF
Azathiropine

Question 8

What are the 2 types of Scleroderma

Answer 8

Morphea (localized) is the most common in children
10% CREST Syndrome:
Calcinosis, Raynauds (do not use nifedipine), esophageal dysmotility, Syndactyly and Telangectasia

Question 9

What are the following recurrent Fever Syndromes:

FMF

Answer 9

Familial Mediteranian Fever syndrome: Does not have a distinct timeline, fevers last 1-3 days, and during each of the episodes there is a distinct type of serositis (usually presting with abdominal pain) and rashes. If not treated, will lead to amyloidosis

Question 10

TRAPS

Answer 10

TNF Receptor Associated Periodic syndrome: Famililal hibernian fever. AD. 3-4 weeks of attacks, also at irregular intervals Migrating rash, arthralgias and eye manifestations.

Question 11

HyperImmunoglobulin D (HIDS)

Answer 11

Rare. AR. 90% will occur in less than 1 year of age.
Dutch and French. Usually a vaccine trigger
Fever: 3-7 days every 3-4 weeks.

Question 12

Cryoprin

Answer 12

Cold Associated urticaria

Question 13

PFAPA

Answer 13

Periodic Fever, Apthous Ulcers, Pharyngitis and Adenitis
Most common in children who are less than 5 years of age.
High fever for 3-5 days every 3-8 weeks and well in between.

Question 14

Main Features of MAS

Answer 14

High Ferritin
DIC (Decreased Fibrinogen, Increased Fibrin breakdown products, changes in INR and PTT)
Fever and Rash - worsening, MSK pain
Liver changes and Pancytopenia
Increased Trig and Pseudohyponatremia

Question 15

What are other signs of Kawasaki Disease

Answer 15

Asceptic meningits
Anterior uveitis 
Hydrops of Gallbladder
Sterile Pyuria
GGT elevation