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Pediatrics > Rheumatological Disease > Flashcards

Rheumatological Disease Flashcards

1
Q

How to diagnose JIA?

A

Diagnosis of exclusion

Signs of arthritis lasting at least 6weeks with no other diagnosis

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2
Q

Which congenital anomalies is common to develop JIA?

A

Down syndrome

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3
Q

Why is JIA called this instead of RA?

A

Not always present rheumatoid factor

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4
Q

Investigations that could be changed in JIA

A
  • ANA, RA, HLA B27 might have it (doesn’t exclude JIA)

- WBC, ESR

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5
Q

Synovial hypertrophy in MRI in child indicate the presence of:

A

JIA due to inflammation

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6
Q

Clinical presentation of JIA

A
  • irritability, gardening, gelling resembling (morning stiffness, pain)
  • contracture
  • fever, fatigue , anorexia
  • motor developmental delay
  • uveitis
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7
Q

What is gelling phenomena and where do we see it?

A

(refuse to walk after a long car ride)

JIA

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8
Q

What are the types of JIA?

A
  • systemic
  • oligoarthritis
  • polyarthritis (+RA, -RA)
  • Enthesitis
  • psoriatic
  • undifferntiated
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9
Q

What is the triad of systemi JIA

A

Quitidian Fever - evancant rash - arthritis which is not the main feature

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10
Q

What is quitadian fever

A

Spiking every 12 hours in systemic JIA

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11
Q

What is evancent rash

A

A rash that is salmon pink in trunk\extremties and appears during the fever of systemic JIA

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12
Q

What is oligo-JIA, and in which group of children do we commonly see it in?

A

Less than 4 joints affecting girls less than 3 years of age.

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13
Q

If patient with JIA has ANA positive, what will be the outcome?

A

High risk for the presence Uveitis, if diagnosed later, can cause blindness

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14
Q

What determines prognosis of poly-JIA

A

The RA (more distruction)

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15
Q

Enthesitis related arthritis is more commonly associated with (male or female)

A

Male

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16
Q

Enthesitis is

A

Not only the joint inflammation but the tendon inflammation

17
Q

What is key word to look for when diagnosis psoriatic arthritis?

A

The presence of psoriasis in a family

18
Q

What is the mainfestation of psoriatic arthrtis in the fingers

A

Dactylitis (sausage like)

19
Q

What is the least likely type of JIA that has less uveitis?

A

Systemic

20
Q

What are the long term consequences of JIA

A
  • gross and local growth disruption (gross like failure to thrive, local like closure of growth plate)
  • osteopenia (inflammation)
  • functional disability (can’t write and wear clothes)
21
Q

What is the treatment of choice for JIA?

A

DMARDs - Steroids in systemic - Intrarticular injection

22
Q

What are the catageries of vasculitis

A
  • large
  • Medium: kawasaki
  • Small: granulomatous - non granumoatous (henoch purpura)
23
Q

What is the henoch purpora?

A

IgA accumulation in organs

  • skin: rash
  • joint: pain
  • kidney and GI
  • CNS (rarely)
24
Q

What is the most triggering pathogen for henoch purpora

A

Group b-hemolytic streptoccus

25
Q

What are the clinical mainfestations of henoch purpura

A

.

26
Q

What is the rash of henoch purpura.

A

Palpable, non blanchable, rash affecting the buttock and the upper limb

27
Q

Investigations of henoch purpura?

A
  • urinanlysis & BP
  • Normal platelt
  • high serum IgA
28
Q

What is the most dangerous complication of henoch purpura

A

Renal failure (bad prognostic factor)

29
Q

How to manage henoch purpura?

A

IV fluid - analgesia

30
Q

When to give steroid for henoch purpura?

A

If it’s severe deteriorating to reach for renal failure

31
Q

What is SLE, what is the trigger?

A
  • Autoantibodies by the adaptive system

- Apoptosis of cells release trigger SLE causing B-cell intolerance (تنهبل)

32
Q

Why do we recommend people with SLE to wear sunblock even without malar rash

A

Reduce the apoptosis and Prevent further activation of immunity

33
Q

What is the difference between childhood and adult SLE?

A

Childhood is more severe and aggressive involving multi-organ damage.

34
Q

What is SLIK criteria:

A

Diagnose SLE in children.
at least 4 criteria (1 lab - 1 clinical)

  • [oral ulcer, non scarring alopecia, malar rash, arthrtitis, arthralgia, serosistis (pleuritis), renal involvement, neurological (psychosis), hemolytic anemia, leukopenia & thrombocytopenia]
  • ANA, AntiDNA, Antismith, direct coomb test, complement.
35
Q

What are the investigtions to order for SLE?

A
  • immunological lab

- screening for renal, cardiac, optha, pulmonary.

36
Q

How to manage SLE?

A

Hydroxycloroquinine (antimalarial) + Steroids!

“+sunblock and DMARDs”

37
Q

What is the only side effect of hydroxycloroquine?

A

Maculapathy. After 5 years

38
Q

Talangectasia of the hard palate in patients with SLE indicates

A

Active SLE

Pediatrics (41 decks)

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