Rheumatological Disease

Question 1

How to diagnose JIA?

Answer 1

Diagnosis of exclusion

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

Question 2

Which congenital anomalies is common to develop JIA?

Answer 2

Down syndrome

Question 3

Why is JIA called this instead of RA?

Answer 3

Not always present rheumatoid factor

Question 4

Investigations that could be changed in JIA

Answer 4

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

- WBC, ESR

Question 5

Synovial hypertrophy in MRI in child indicate the presence of:

Answer 5

JIA due to inflammation

Question 6

Clinical presentation of JIA

Answer 6

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

Question 7

What is gelling phenomena and where do we see it?

Answer 7

(refuse to walk after a long car ride)

JIA

Question 8

What are the types of JIA?

Answer 8

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

Question 9

What is the triad of systemi JIA

Answer 9

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

Question 10

What is quitadian fever

Answer 10

Spiking every 12 hours in systemic JIA

Question 11

What is evancent rash

Answer 11

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

Question 12

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

Answer 12

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

Question 13

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

Answer 13

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

Question 14

What determines prognosis of poly-JIA

Answer 14

The RA (more distruction)

Question 15

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

Answer 15

Male

Question 16

Enthesitis is

Answer 16

Not only the joint inflammation but the tendon inflammation

Question 17

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

Answer 17

The presence of psoriasis in a family

Question 18

What is the mainfestation of psoriatic arthrtis in the fingers

Answer 18

Dactylitis (sausage like)

Question 19

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

Answer 19

Systemic

Question 20

What are the long term consequences of JIA

Answer 20

• 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)

Question 21

What is the treatment of choice for JIA?

Answer 21

DMARDs - Steroids in systemic - Intrarticular injection

Question 22

What are the catageries of vasculitis

Answer 22

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

Question 23

What is the henoch purpora?

Answer 23

IgA accumulation in organs

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

Question 24

What is the most triggering pathogen for henoch purpora

Answer 24

Group b-hemolytic streptoccus

Question 25

What are the clinical mainfestations of henoch purpura

Answer 25

.

Question 26

What is the rash of henoch purpura.

Answer 26

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

Question 27

Investigations of henoch purpura?

Answer 27

- urinanlysis & BP - Normal platelt - high serum IgA

Question 28

What is the most dangerous complication of henoch purpura

Answer 28

Renal failure (bad prognostic factor)

Question 29

How to manage henoch purpura?

Answer 29

IV fluid - analgesia

Question 30

When to give steroid for henoch purpura?

Answer 30

If it’s severe deteriorating to reach for renal failure

Question 31

What is SLE, what is the trigger?

Answer 31

- Autoantibodies by the adaptive system | - Apoptosis of cells release trigger SLE causing B-cell intolerance (تنهبل)

Question 32

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

Answer 32

Reduce the apoptosis and Prevent further activation of immunity

Question 33

What is the difference between childhood and adult SLE?

Answer 33

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

Question 34

What is SLIK criteria:

Answer 34

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.

Question 35

What are the investigtions to order for SLE?

Answer 35

- immunological lab | - screening for renal, cardiac, optha, pulmonary.

Question 36

How to manage SLE?

Answer 36

Hydroxycloroquinine (antimalarial) + Steroids! “+sunblock and DMARDs”

Question 37

What is the only side effect of hydroxycloroquine?

Answer 37

Maculapathy. After 5 years

Question 38

Talangectasia of the hard palate in patients with SLE indicates

Answer 38

Active SLE