Paeds rheum Flashcards
What is JIA?
Juvenile idiopathic arthritis (JIA) refers to a condition affecting children and adolescents where autoimmune inflammation occurs in the joints. It is diagnosed where there is arthritis without any other cause, lasting more than 6 weeks in a patient under the age of 16. It has also been known as juvenile chronic arthritis and juvenile rheumatoid arthritis.
The key features of inflammatory arthritis are joint pain, swelling and stiffness.
PT comes in with a salmon pink rash, fevers and joint pain -> What comes to mind?
Think of Still’s disease (systemic JIA) when a patient presents with a salmon-pink rash, fevers and joint pain. In children that have fevers for more than 5 days, the key non-infective differentials to remember are Kawasaki disease, Still’s disease, rheumatic fever and leukaemia.
What are the 5 types of JIA you need to know?
1.Systemic JIA
2.Polyarticular JIA
3.Oligoarticular JIA
4.Enthesitis related arthritis
5.Juvenile psoriatic arthritis
What can you tell me about systemic JIA?
Also known as stills disease:
Things to look out for: Salmon pink rash thatr is associated with fever or joint pain.
Antinuclear antibodies and rheumatoid factors are typically negative. There will be raised inflammatory markers, with raised CRP, ESR, platelets and serum ferritin.
A key complication is macrophage activation syndrome (MAS)
What do you know about polyarticular JIA?
idiopathic inflammatory arthritis in 5 joints or more. The inflammatory arthritis tends to be symmetrical and can affect the small joints of the hands and feet, as well as the large joints such as the hips and knees. There are minimal systemic symptoms, but there can be mild fever, anaemia and reduced growth. Systemic symptoms are mild, unlike systemic onset JIA.
Can be seropositive or seronegative for RF
This is also knowns as pauciarticular JIA. It involves 4 joints or less. Usually it only affects a single joint, which is described as a monoarthritis. It tends to affect the larger joints, often the knee or ankle. It occurs more frequently in girls under the age of 6 years.
Look out for: ant Uveitis
Antinuclear antibody +
Enthesitis related artheritis?
Patients have inflammatory arthritis in the joints as well as enthesitis.
HLA-B27 related so look out for IBD, psoriasis and anterior uveitis
Patients with enthesitis will be tender to localised palpation of the entheses. Therefore it is worth palpating key areas to elicit tenderness of the entheses
Juvenile psoriatic arthritis?
seronegative inflammatory arthritis associated with psoriasis, the skin condition. The pattern of joint involvement varies. Patients can have a symmetrical polyarthritis affecting the small joints similar to rheumatoid, or an asymmetrical arthritis affecting the large joints in the lower limb.
Plaques of psoriasis on the skin
Pitting of the nails (nail pitting)
Onycholysis, separation of the nail from the nail bed
Dactylitis, inflammation of the full finger
Enthesitis, inflammation of the entheses, which are the points of insertion of tendons into bone
What is the mx of JIA?
1.NSAIDs, such as ibuprofen
2.Steroids, either oral, intramuscular or intra-artricular in oligoarthritis
3.Disease modifying anti-rheumatic drugs (DMARDs), such as methotrexate, sulfasalazine and leflunomide
4.Biologic therapy, such as the tumour necrosis factor inhibitors etanercept, infliximab and adalimumab
What is Ehler Danlos syndrome and which subtype do you need to remember?
Ehler-Danlos syndrome is an autosomal dominant connective tissue disorder that mostly affects type III collagen. This results in the tissue being more elastic than normal leading to joint hypermobility and increased elasticity of the skin.
Hypermobile Ehlers-Danlos syndrome represents most cases of EDS in clinical practice and exams. It is the most common and least severe type of Ehlers-Danlos syndrome. The key feature is joint hypermobility, but patients also have soft and stretchy skin.
How would you differentiate Ehler Danlos from marfans?
Marfan syndrome, which also features hypermobility, by examining for features such as high arch palate, arachnodactyly and arm span.
Mx of Ehler Danlos?
There is no cure for Ehler Danlos with tx focussed around maintaining joint health over much else:
Physiotherapy to strengthen and stabilise the joints
Occupational therapy to maximise function
Maintaining good posture in the joints
Moderating the intensity of activity to minimise flares
Psychology may be required to a help manage the chronic condition and pain
What score can you use to assess Ehler Danlos?
Beighton score - max of 9 points and you basically judge hypermobility in various joints
Px of ehler danlos?
The most common presenting complaint for hypermobile EDS is joint pain and hypermobility
Hypermobility in joints
Joint pain after exercise or inactivity
Joint dislocations, for example the shoulders or hips
Soft stretchy skin
Easy bruising
Poor healing of wounds
Bleeding
Headaches
Autonomic dysfunction causing dizziness and syncope
What is HSP and what is it caused by?
Henoch-Schonlein Purpura (HSP) is an IgA vasculitis that presents with a purpuric rash affecting the lower limbs and buttocks in children. Inflammation occurs in the affected organs due to IgA deposits in the blood vessels.
Also affects the skin and GI tract
Most common in children the age of 10
