ראומטולוגיה Flashcards
Dgx of JIA?
Arthritis < 16 yrs of age
symptoms > 6 wks
Lines of Tx for JIA?
1st line- NSAIDS + steroids to joint
2nd line- MTX
in Systemic JIA- 1st line MTX
most common subtype of JIA?
and what is the peak of age
oligoarticular < 5 joints involved, a-symmetrical
age 2-4. girls > boys
Clinical and Ab of oligo articular
ANA ab- 60%
anterior uveitis - 30%
oligo JIA
how many will have full remission?
which things are indicated for risk for chronic ant. uveitis?
90% full remmison
risk for chronic ant. uveitis?
pt with
* ANA+
* Onset < 6 age
* disease prolong > 3years
in polyarthritis JIA, Reumatic nodules are a/w which type of Ab?
RF
seen more at adolesence and not young kids
Which Ab is involve in bad prognosis of polyarthritis JIA?
RF +
anti-CCP
which type of JIA will be seen with:
leukocytosis
high ferritin, CRP, ESR
Thrombocytosis
and solomon rash?
Systemic JIA- present with fever
*salomon rash can accompnied the fever
Which extra-joints menefistation can be seen in systemic JIA?
Serositis 50% (pleuritis, pericarditis)
Hepatosplenomegaly- 70%
Which life therathing complication can be seen in systemic JIA?
and which lab valuse with right clinical presenation will raise our suspicion
Macrophage activation syndrome (MAS)
encephalopathy,multi organ damage Liver issues, prolong PT+PTT
Low ESR
How to dgx macrophage activation syndrome (MAS)?
and how can we confirm our Dgx?
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
Tx for MAS (macrophage activation syndrome)
high dose IV methylprednisone
+
cyclosporine or anakirna
What is the duration and joint involvment of post Sterp arthritis?
Could last for months.
mainly oligo ( < 5 joints)
Which pathogens are a/w reactive arthritis?
Salmonella
Shigella
Yarsinea
Compylobacter
Clamydia
jiardia
How many of pt with Reactive arthritis are positive for HLA-B27
75%
Which disease is a/w
אפיזודות של חום כאבי בטן ותפליט פלאורלי פריחה דמוית אריסיפלס ברגל התחתונה
, מונוארתריטיס ודלקת באשכים
FMF
Which mutation and regio is a/w FMF?
M694V
טורקים, עירקים, ארמנים, ערביפ ואיטלקים
AR
What are the 2 most common menefistation of FMF?
Fever- most common
Abdominal pain 90%
also:
arthritis
orchditis
erysipelas
serositis- mainly pleuralitis
Tx for FMF?
1st and 2nd line + benefit of 1st line tx
1st line- Colcihicine- reduce frequency , duration and severity of attecks + prevent amyloidosis development
2nd lnine- anti- IL1
Most common cause of death and complication of FMF?
Amyloidosis
Dgx by- kidney / rectal biopsy
What is the clinical presentation of PFAPA
Which ages and duration of episodes?
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
Tx for PFAPA episode?
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
What is the prognosis of PFAPA?
Spontenous remission in age 4-8 without consequences
Which is the first most common vasculitis in kids and which is the second?
1st- HSP
2nd- Kawasaki
what are the criteria for Dgx of kawasaki disease?
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
What is a-typical kawasaki?
Fever > 5 days + 2-3 kawasaki criteria
How we Diagnose A-typical kawasaki?
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)
Tx for Kwasaki disease?
IVIG high dose
Aspirin- high dose until fever resolve for 48h »_space; then low dose apsirin for 6 wks
if no respond:
another course of IVIG , steorids and consider infliximab
severly ill pt- Cyclosporine or cyclophosphamide
After IVIG there is a contraindication for which vaccination and for how ling?
11 month
no MMR or Varicella
no live vaccine
In which time frame IVIG treatment for kawasaki is recommended?
up to 10 days since onset of disease
in severe / non response disease- 2nd dose IVIG, Sterodis and or infliximab
Which type of vasculitis is HSP?
what is the age prevelance?
IgA vasculitis - most common type in childrens
small veseel vasculitis
What we expect the PLT count to be in HSP?
normal or eleveted
How to Dgx HSP?
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
Tx for HSP and complications?
Supportive
Complications- steorids (severe pain + renal invovlment)
arthritis - NSAIDS, but use with caution
Prognosis of HSP:
* reslove?
* reccurence?
* CKD?
* ESRD?
- reslove- 4 wks 80%
- reccurence- 4-6 months 60%
- CKD- 2%
- ESRD- out of 2% , 5% will end with ESKD
What is the HSP tetrad?
- joint pain - w/o effusion
- Palbable purpura
- abdominal pain- mainly colicky
- Renal involment - microscopic hematuria (in severe cases protenuria)
Which disease is a/w symmetrical proximal muscle weakness, with diffrent types of rash and gottorn paples on DIP + PIP, Haliothropic rash
Juvenile Dermatomyositis
like adult but in childrens more GI involvment and calcifications
how many of pt with Juvenile dermatomyositis will present with calcinosis?
40%
complication
Helitrope rash + Gottorns nodules are two pathognemonic features of?
dermatomyositis
Helitrope- פריחה סגלגלה מעל העיניים
Gottorn nodules- פריחה ורודה אדמדמה במפרקים בעיקר של הידיים
what are the criteria to Dgx of Juvenile dermatomyositis?
tx?
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
Which Abs and complement are in correlation of SLE severity?
C3, C$
CH50
anti- dsDNA
Tx of choice for SLE?
Steroids pulse + hydroxycholoquine
במקרה של פגיעה כלייתית או מוחית- תוספת ציקולופוספמיד /מיקופנולט / מתוטרקסט /אזתיופורין
