ראומטולוגיה

Question 1

Dgx of JIA?

Answer 1

Arthritis < 16 yrs of age
symptoms > 6 wks

Question 2

Lines of Tx for JIA?

Answer 2

1st line- NSAIDS + steroids to joint
2nd line- MTX

in Systemic JIA- 1st line MTX

Question 3

most common subtype of JIA?

and what is the peak of age

Answer 3

oligoarticular < 5 joints involved, a-symmetrical

age 2-4. girls > boys

Question 4

Clinical and Ab of oligo articular

Answer 4

ANA ab- 60%
anterior uveitis - 30%

Question 5

oligo JIA

how many will have full remission?

which things are indicated for risk for chronic ant. uveitis?

Answer 5

90% full remmison

risk for chronic ant. uveitis?
pt with
* ANA+
* Onset < 6 age
* disease prolong > 3years

Question 6

in polyarthritis JIA, Reumatic nodules are a/w which type of Ab?

Answer 6

RF

seen more at adolesence and not young kids

Question 7

Which Ab is involve in bad prognosis of polyarthritis JIA?

Answer 7

RF +
anti-CCP

Question 8

which type of JIA will be seen with:
leukocytosis
high ferritin, CRP, ESR
Thrombocytosis

and solomon rash?

Answer 8

Systemic JIA- present with fever
*salomon rash can accompnied the fever

Question 9

Which extra-joints menefistation can be seen in systemic JIA?

Answer 9

Serositis 50% (pleuritis, pericarditis)
Hepatosplenomegaly- 70%

Question 10

Which life therathing complication can be seen in systemic JIA?

and which lab valuse with right clinical presenation will raise our suspicion

Answer 10

Macrophage activation syndrome (MAS)
encephalopathy,multi organ damage Liver issues, prolong PT+PTT

Low ESR

Question 11

How to dgx macrophage activation syndrome (MAS)?

and how can we confirm our Dgx?

Answer 11

Criteria- extremly eleveted ferritin + 2 of the following:
1. Thrombocytopenia
2. eleveted Liver enzym
3. Hypofibrinogemia
4. Hyper TG

confirm Dgx
Bone merrow present with hemophagocytosis

Question 12

Tx for MAS (macrophage activation syndrome)

Answer 12

high dose IV methylprednisone
+
cyclosporine or anakirna

Question 13

What is the duration and joint involvment of post Sterp arthritis?

Answer 13

Could last for months.
mainly oligo ( < 5 joints)

Question 14

Which pathogens are a/w reactive arthritis?

Answer 14

Salmonella
Shigella
Yarsinea
Compylobacter
Clamydia
jiardia

Question 15

How many of pt with Reactive arthritis are positive for HLA-B27

Answer 15

75%

Question 16

Which disease is a/w
אפיזודות של חום כאבי בטן ותפליט פלאורלי פריחה דמוית אריסיפלס ברגל התחתונה
, מונוארתריטיס ודלקת באשכים

Answer 16

FMF

Question 17

Which mutation and regio is a/w FMF?

Answer 17

M694V

טורקים, עירקים, ארמנים, ערביפ ואיטלקים

AR

Question 18

What are the 2 most common menefistation of FMF?

Answer 18

Fever- most common
Abdominal pain 90%

also:
arthritis
orchditis
erysipelas
serositis- mainly pleuralitis

Question 19

Tx for FMF?

1st and 2nd line + benefit of 1st line tx

Answer 19

1st line- Colcihicine- reduce frequency , duration and severity of attecks + prevent amyloidosis development

2nd lnine- anti- IL1

Question 20

Most common cause of death and complication of FMF?

Answer 20

Amyloidosis

Dgx by- kidney / rectal biopsy

Question 21

What is the clinical presentation of PFAPA

Which ages and duration of episodes?

Answer 21

like its name:
PF- Periodic Fever
A- aphtous somatitis
P- pahyngitis
A- adenitis (cervial LAP)

age 2-5
duration of episodes- 4-6 days
episodes in a year- 8-12

Question 22

Tx for PFAPA episode?

Answer 22

one dose Prednisone 1-2 mg/kg in the start of an episodes.

most clinical symptoms pass within 24hrs

Tonsillectomy can heal in some caseses

Question 23

What is the prognosis of PFAPA?

Answer 23

Spontenous remission in age 4-8 without consequences

Question 24

Which is the first most common vasculitis in kids and which is the second?

Answer 24

1st- HSP
2nd- Kawasaki

Question 25

what are the criteria for Dgx of kawasaki disease?

Answer 25

at least 4/5 rememebr- **CRASH and BURN** C- conjuctuvitis bi-lateral R- Rash (maculopappular of scalded) A- adenitis (cervical unilaterl LN, at least one is > 1.5 cm) S- somatitis- Struabrry toungh with red and crackled lips H- Hands & feet- swollow red hands and feets BURN- Fever > 5 days

Question 26

What is a-typical kawasaki?

Answer 26

Fever > 5 days + 2-3 kawasaki criteria

Question 27

How we Diagnose A-typical kawasaki?

Answer 27

labs reasults after 7 days showing the following: **eleveation of ESR + CRP + 3/6 of the following: ** 1. WBC > 15K 2. anemia 3. PLT > 450 (thrombocytosis) 4. Hypoalbuminemia (albumin < 3) 5. eleveted ALT 6. urine > 10 WBC/HPF (Sterile Pyurea)

Question 28

Tx for Kwasaki disease?

Answer 28

IVIG high dose Aspirin- high dose until fever resolve for 48h >> then low dose apsirin for 6 wks ## Footnote if no respond: another course of IVIG , steorids and consider infliximab severly ill pt- Cyclosporine or cyclophosphamide

Question 29

After IVIG there is a contraindication for which vaccination and for how ling?

Answer 29

11 month no MMR or Varicella no live vaccine

Question 30

In which time frame IVIG treatment for kawasaki is recommended?

Answer 30

up to 10 days since onset of disease in severe / non response disease- 2nd dose IVIG, Sterodis and or infliximab

Question 31

Which type of vasculitis is HSP? what is the age prevelance?

Answer 31

**IgA vasculitis - most common type in childrens** small veseel vasculitis

Question 32

What we expect the PLT count to be in HSP?

Answer 32

normal or eleveted

Question 33

How to Dgx HSP?

Answer 33

palpable purpura + 1 of the following: 1. abdominal pain 2. Arthritis / arthralgia 3. Biopsy with IgA 4. Kidney involvment- protenuria > 3 gr, hematuria or RBC casts

Question 34

Tx for HSP and complications?

Answer 34

Supportive Complications- steorids (severe pain + renal invovlment) arthritis - NSAIDS, but use with caution

Question 35

Prognosis of HSP: * reslove? * reccurence? * CKD? * ESRD?

Answer 35

* reslove- 4 wks 80% * reccurence- 4-6 months 60% * CKD- 2% * ESRD- out of 2% , 5% will end with ESKD

Question 36

What is the HSP tetrad?

Answer 36

1. joint pain - w/o effusion 2. Palbable purpura 3. abdominal pain- mainly colicky 4. Renal involment - microscopic hematuria (in severe cases protenuria)

Question 37

Which disease is a/w symmetrical proximal muscle weakness, with diffrent types of rash and gottorn paples on DIP + PIP, Haliothropic rash

Answer 37

Juvenile Dermatomyositis | like adult but in childrens more GI involvment and calcifications

Question 38

how many of pt with Juvenile dermatomyositis will present with calcinosis?

Answer 38

40% | complication

Question 39

Helitrope rash + Gottorns nodules are two pathognemonic features of?

Answer 39

dermatomyositis Helitrope- פריחה סגלגלה מעל העיניים Gottorn nodules- פריחה ורודה אדמדמה במפרקים בעיקר של הידיים

Question 40

what are the criteria to Dgx of Juvenile dermatomyositis? | tx?

Answer 40

Typical rash (Helitrhope or gottorn papules) + 3 /4: 1. proximal symmeric muscle weakness 2. Eleveted liver enzyme- CPK, ALT, AST, LDH, aldolase 3. typical EMG 4. muscle biopsy | Tpoical Steroids + calcinurin inhibitors

Question 41

Which Abs and complement are in correlation of SLE severity?

Answer 41

C3, C$ CH50 anti- dsDNA

Question 42

Tx of choice for SLE?

Answer 42

Steroids pulse + hydroxycholoquine ## Footnote במקרה של פגיעה כלייתית או מוחית- תוספת ציקולופוספמיד /מיקופנולט / מתוטרקסט /אזתיופורין