Rheumatological Disease Flashcards
How to diagnose JIA?
Diagnosis of exclusion
Signs of arthritis lasting at least 6weeks with no other diagnosis
Which congenital anomalies is common to develop JIA?
Down syndrome
Why is JIA called this instead of RA?
Not always present rheumatoid factor
Investigations that could be changed in JIA
- ANA, RA, HLA B27 might have it (doesn’t exclude JIA)
- WBC, ESR
Synovial hypertrophy in MRI in child indicate the presence of:
JIA due to inflammation
Clinical presentation of JIA
- irritability, gardening, gelling resembling (morning stiffness, pain)
- contracture
- fever, fatigue , anorexia
- motor developmental delay
- uveitis
What is gelling phenomena and where do we see it?
(refuse to walk after a long car ride)
JIA
What are the types of JIA?
- systemic
- oligoarthritis
- polyarthritis (+RA, -RA)
- Enthesitis
- psoriatic
- undifferntiated
What is the triad of systemi JIA
Quitidian Fever - evancant rash - arthritis which is not the main feature
What is quitadian fever
Spiking every 12 hours in systemic JIA
What is evancent rash
A rash that is salmon pink in trunk\extremties and appears during the fever of systemic JIA
What is oligo-JIA, and in which group of children do we commonly see it in?
Less than 4 joints affecting girls less than 3 years of age.
If patient with JIA has ANA positive, what will be the outcome?
High risk for the presence Uveitis, if diagnosed later, can cause blindness
What determines prognosis of poly-JIA
The RA (more distruction)
Enthesitis related arthritis is more commonly associated with (male or female)
Male
Enthesitis is
Not only the joint inflammation but the tendon inflammation
What is key word to look for when diagnosis psoriatic arthritis?
The presence of psoriasis in a family
What is the mainfestation of psoriatic arthrtis in the fingers
Dactylitis (sausage like)
What is the least likely type of JIA that has less uveitis?
Systemic
What are the long term consequences of JIA
- 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)
What is the treatment of choice for JIA?
DMARDs - Steroids in systemic - Intrarticular injection
What are the catageries of vasculitis
- large
- Medium: kawasaki
- Small: granulomatous - non granumoatous (henoch purpura)
What is the henoch purpora?
IgA accumulation in organs
- skin: rash
- joint: pain
- kidney and GI
- CNS (rarely)
What is the most triggering pathogen for henoch purpora
Group b-hemolytic streptoccus
What are the clinical mainfestations of henoch purpura
.
What is the rash of henoch purpura.
Palpable, non blanchable, rash affecting the buttock and the upper limb
Investigations of henoch purpura?
- urinanlysis & BP
- Normal platelt
- high serum IgA
What is the most dangerous complication of henoch purpura
Renal failure (bad prognostic factor)
How to manage henoch purpura?
IV fluid - analgesia
When to give steroid for henoch purpura?
If it’s severe deteriorating to reach for renal failure
What is SLE, what is the trigger?
- Autoantibodies by the adaptive system
- Apoptosis of cells release trigger SLE causing B-cell intolerance (تنهبل)
Why do we recommend people with SLE to wear sunblock even without malar rash
Reduce the apoptosis and Prevent further activation of immunity
What is the difference between childhood and adult SLE?
Childhood is more severe and aggressive involving multi-organ damage.
What is SLIK criteria:
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.
What are the investigtions to order for SLE?
- immunological lab
- screening for renal, cardiac, optha, pulmonary.
How to manage SLE?
Hydroxycloroquinine (antimalarial) + Steroids!
“+sunblock and DMARDs”
What is the only side effect of hydroxycloroquine?
Maculapathy. After 5 years
Talangectasia of the hard palate in patients with SLE indicates
Active SLE
