Paediatric rheumatology Flashcards
(37 cards)
1
Q
Juvenile idiopathic arthritis : definition
A
- A condition that affects children and adolescents where autoimmune inflammation occurs in the joints
- Arthritis w/o underlying cause for > 6 weeks in patients < 16 years
2
Q
Juvenile idiopathic arthritis : Different types
A
- Systemic JIA
- Polyarticular JIA
- Oligoarticular JIA
- Enthesis-related arthritis
- Juvenile psoriatic arthritis
3
Q
Systemic JIA : Clinical presentation
A
AKA : Still’s disease
Joint inflammation and swelling with;
* Systemic symptoms
1) High swinging fever
2) Salmon pink rash
3) Enlarged lymph nodes
- Raised ESR + CRP
4
Q
Systemic JIA : Complication
A
- Macrophage activation syndrome
i) Severe activation of the immune system with a massive inflammatory response
ii) Presentation : DIC, Thrombocutopaenia
5
Q
Polyarticular JIA : Clinical features
A
- Idiopathic inflammatory arthritis in >5 joints
- Mild systemic symptoms
- Similar to RA in children
6
Q
Oligoarticular JIA : Clinical features
A
- < 4 joints affected : usually affects only a single large joint e.g. Knee or Ankle
- Incidence : Girls < 6 years of age
- Associated with : Anterior uveitis
- Bloods : **Antinuclear antibody + **
7
Q
Enthesis related JIA : Definition
A
- Inflammation at the site where tendons and ligaments attach to the bone
- Seronegative spondyloarthritis : HLAB27 gene
8
Q
Enthesis related JIA : Clinical features
A
- HLAB27 + association : IBD, Psoriasis, uveitis
- Tenderness at entheses : Achilies tendon, plantar fascia, knee hip
9
Q
Juvenile Psoriatic arthritis : Definition
A
- Seronegative arthritis associated with psoriasis
10
Q
Juvenile Psoriatic arthritis : Clinical features
A
- Polyarthritis + Enthesitis
- Psoriasis plaque, nail pitting
11
Q
Rheumatic fever : Definition
A
- Inflammatory condition that develops as a result of abnormal immune complication of Streptococcus pyogenes infection
12
Q
Rheumatic fever : Clinical presentation
A
- The typical presentation of rheumatic fever occurs
- 2 – 4 weeks following a streptococcal infection, such as tonsillitis.
Symptoms affect multiple systems, causing:
1. Fever
2. Joint pain
3. Rash
4. Shortness of breath
5. Chorea
13
Q
Rheumatic fever : Joint involvement
A
- Migratory arthritis : different joints inflame and improve at different times
- Large joints : hot, swollen, painful joints
14
Q
Rheumatic fever : Cardiac involvement
A
- Carditis : Pericarditis, Myocarditis and Endocarditis
- Auscultation
* Murmur : Mitral stenosis
* Pericardial rub
15
Q
Rheumatic fever : Skin involvement
A
- Subcutaneous nodules : firm, painless nodules over extensor surface joints e.g. elbows
- Erythema marginatum rash : pink rings of varying size - on torso/limbs
16
Q
Rheumatic fever : Nervous system involvement
A
- Sydenham Chorea : irregular uncontrolled rapid movement of limbs
17
Q
Rheumatic fever : Investigation
A
- Throat swab : bacterial culture
- Antistreptococcal antibodies (ASO) - indicates recent strep infection
- ECHO, ECG and CXR
18
Q
Rheumatic fever : Jone’s major criteria
A
- J – Joint arthritis
- O – Organ inflammation, such as carditis
- N – Nodules
- E – Erythema marginatum rash
- S – Sydenham chorea
19
Q
Rheumatic fever : Jone’s minor criteria
A
- Fever
- ECG Changes (prolonged PR interval) without carditis
- Arthralgia without arthritis
- Raised inflammatory markers (CRP and ESR)
20
Q
Rheumatic fever : Jone’s criteria for diagnosis
A
Diagnosis can be made if there is evidence of recent streptococcal infection, plus:
1. Two major criteria
OR
2. One major criteria plus two minor criteria
21
Q
Rheumatic fever : Management
A
- Eradication of Streptococcal infection : Penicillin Vfor 10 days
- NSAID + Steroids : Joint pain and inflammation
22
Q
Rheumatic fever : Complications
A
- Mitral stenosis
- Chronic Heart failure
23
Q
Kawasaki disease : Definition
A
Systemic medium vessel vasculitis
24
Q
Kawasaki disease : Pathophysiology
A
- Activation of immune system : Immune response to infectious agent
- Vasculitis : activation of endothelial cells in the blood vessels } vasculitis of small and medium size vessels (Coronary artery)
- Damage to endothelium : Cytokine release, formation of immune complexes
- Thrombosis and aneurysm formation : due to damage to the endothelium
25
Kawasaki disease : Clinical features
**Persistent high fever > 5 days with** ;
1. ***Strawberry tongue* **(red tongue with large papillae)
2. ***Cracked lips***
3. ***Cervical lymphadenopathy***
4. ***Bilateral conjunctivitis***
26
Kawasaki disease : Skin manifestation
1. *Widespread erythematous maculopapular rash*
AND
2. *Desquamation* (skin peeling) on the palms and soles.
27
Kawasaki disease : Diagnosis
1. Clinical diagnosis
* ***Full blood count*** can show anaemia, leukocytosis and thrombocytosis } high risk of clotting
* ***Liver function tests*** can show hypoalbuminemia and elevated liver enzymes
* ***Inflammatory markers*** (particularly ESR) are raised
* ***Echocardiogram*** can demonstrate coronary artery pathology
28
Kawasaki disease : Management
1. ***High dose aspirin* **: to reduce the risk of *thrombosis*
2. ***IV immunoglobulins*** : to reduce the risk of *coronary artery aneurysms*
3. ***ECHO*** : f/u for monitoring for coronary aneurysm
29
Kawasaki disease : Complication
**Coronary Aneurysm**
30
Henoch-Schonlein Purpura : Definition
***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*.**
31
Henoch-Schonlein Purpura : Pathophysiology
1. **Abnormal immune response** due to infectious trigger (unknown)
2. **Excess IgA antibodies releases** } bind to antigens
3. **IgA immune complexes form**
4. **Inflammation of blood vessels** : complexes deposit In blood vessels particularly skin, joint, GI tract and kidneys
* Swelling and thickening of vessels : ischaemia
* Increased permeability : contents leak out of blood vessels
32
Henoch-Schonlein Purpura : Clinical features
1. **Skin : Purpura - ‘purple spots on the skin’**
* Palpable and start in legs works up to buttocks } may develop skin ulceration and necrosis
* Leakage of blood from inflamed blood vessel into tissues
2 . **Arthritis : Pain + swelling of joints**
3 . **Abdominal pain**
* Inflammation of GI vessels } may lead to GI haemorrhage or bowel obstruction
4 . **Nephritis : inflammation of glomeruli**
* Lead to haematuria and proteinuria } may lead to Nephrotic syndrome
* Oedema
33
Henoch-Schonlein Purpura : Investigations
1. *Full blood count* and *blood film* for thrombocytopenia, sepsis and leukaemia
2. *CRP* for sepsis
3. *Blood cultures* for sepsis
4. *Renal profile* for kidney involvement
5. *Serum albumin* for nephrotic syndrome
6. *Urine dipstick* for proteinuria
7. *Urine protein:creatinine ratio* to quantify the proteinuria
8. *Blood pressure* for hypertension (Nephrotic syndrome)
34
Henoch-Schonlein Purpura : Diagnosis
* **Palpable purpura** (not petichiae) + at least **one** of:
1. Diffuse abdominal pain
2. Arthritis or arthralgia
3. IgA deposits on histology (biopsy)
4. Proteinuria or haematuria
35
Henoch-Schonlein Purpura : Management
Management is *supportive*, with simple analgesia, rest and proper hydration.
36
Henoch-Schonlein Purpura : Prognosis
* usually excellent, HSP is a self-limiting condition, especially in children without renal involvement
* Blood pressure and urinanalysis should be monitored to detect progressive renal involvement
* 1/3rd of patients have a relapse
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