Rheumatology Flashcards
What is the triad for Systemic JIA?
Fever, Salmon rash & arthritis
- > 39, daily, evening or late afternoon then hypothermia
What complications can you get with JIA?
Macrophage Activation Syndrome (MAS)
Eye:
- Inflammatory eye disease/visual damage
GI:
- Hepatosplenomegaly
Lymphadenopathy
Serositis (inflammation of serous membrane)
Micrognathia
MSK related
- Growth retardation
- Joint erosion
- Fixed deformities
- Limb length discrepancy
- Osteoporosis
Complications due to pain:
- fitness
- diability
- psychosocial
What is MAS? What is it associated with? What are the signs? What is the biochemical profile?
Syndrome associated with the use of NSAIDs, gold or abrupt changes in medication.
Macrophages get activated and devour everything.
Signs:
- Hepatic failure: ascites, jaundice, N & V
- DIC: Disseminated intravascular coagulopathy
Biochemical profile:
- Pancytopenia
- Elevated D Dimer
- HUGE increase in ferritin
- Low fibrinogen
Treatment for macrophage activation syndrome
Fresh Frozen Plasma Pulse Steroids, 1g (high dose) methyl prednisolone Cyclosporine Methotrexate IL-1 blockage - anakinra
What is spondyloarthropathy?
Enthesitis-related arthritis
Group of diseases affecting peripheral joints & axial skeleton
What is enthesitis?
Inflammation of the tendon, ligament, fascia or capsule
What is the epidemiology of spondyloarthropathy?
Late childhood/adolescence
F : M = 7 : 1
What genes are involved with spondyloarthropathy?
HLA B27.
+ve in 50% of patients.
What combination of risk factors increases the risk of develpoing spondyloarthropathy?
+ve HLA B27 & fam hx = 10x greater risk.
Must have fam hx then the risk increases
What tendons & joints are commonly involved in enthesitis
Patella Achilles Lower back SI joints Feet
On examination, where is the pain usually for enthesitis?
2, 6 and 10 o clock positions.
- where the quadriceps insert onto the patella
- where the patella ligament joins the patella to the tibial tuberosity
Which parts of the feet are affected in enthesitis
The heads of the 1st - 5th metatarsals
Base of the 5th metatarsal
Calcaneus
How is synovitis different from enthesitis?
Synovitis is sore along the joint line.
Enthesitis is sore at specific parts where the tendon inserts.
What clinical profile would have the highest risk of developing juvenile ankylosing spondylitis?
+ve B27 gene
Boy
High ESR
How to diagnose JAS & adult Ankylosing spondylitis?
JAS: Radiographic profo of sacroilitis
AS: use the new york criteria
Treatment for Spondyloarthropathy? (not juvenile ankylosing spondylitis)
Tailor to joint involvement & risk of disease progression
PT, OT for school, splint, heel cups
Monitor for eye disease & GI complications. ALL subtyeps of spondyloarthropathy can develop eye complications
What are the extra-articular complications of Spondyloarthropathies
Acute anterior uveitis (20%)
- Painful, erythematous photophobia
- Iris & ciliary body
Aortitis: rare, late in adults with severe AS
Amyloidosis
Describe the clinical presentation of JAS
- Enthesitis
- Axial Arthritis
- Peripheral arthritis
HLA B27 +Ve
ANA -ve
RF -ve
May have eye involvement. No GI or skin complications
Definition of Juvenile Psoriatic Arthritis
Inflammatory arthritis beginning < 16 y.o & associated with psoriasis
What is the diagnostic criteria for JPA?
- Arthritis & psoriasis (or)
- Arthritis + 2 of the following:
- Dactylitis - sausage finger
- Nail pitting
- fam hx of psoriasis in 1st degree relative
If the ptx is RF +ve can it be JPA?
NO. Juvenile Psoriatic Arthritis is RF -ve
Epidemiology of Juvenile Psoriatic Arthritis
15% of all childhood arthritis are JPA
2-3 per 100,000 every year
Bimodal peak. Pre-school & 10
Caucasian predominace
Describe Psoriatic Rashes
Well-demarcated, scaly, erythematous lesions
On the extensor surfaces. Can be found on neck, ears & hairline.
Koebner’s phenomenon
What is koebner’s phenomenon
When a tiny injury or irritation triggers plaque formation
