Paediatric rheumatology Flashcards
Juvenile idiopathic arthritis : definition
- 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
Juvenile idiopathic arthritis : Different types
- Systemic JIA
- Polyarticular JIA
- Oligoarticular JIA
- Enthesis-related arthritis
- Juvenile psoriatic arthritis
Systemic JIA : Clinical presentation
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
Systemic JIA : Complication
- Macrophage activation syndrome
i) Severe activation of the immune system with a massive inflammatory response
ii) Presentation : DIC, Thrombocutopaenia
Polyarticular JIA : Clinical features
- Idiopathic inflammatory arthritis in >5 joints
- Mild systemic symptoms
- Similar to RA in children
Oligoarticular JIA : Clinical features
- < 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 + **
Enthesis related JIA : Definition
- Inflammation at the site where tendons and ligaments attach to the bone
- Seronegative spondyloarthritis : HLAB27 gene
Enthesis related JIA : Clinical features
- HLAB27 + association : IBD, Psoriasis, uveitis
- Tenderness at entheses : Achilies tendon, plantar fascia, knee hip
Juvenile Psoriatic arthritis : Definition
- Seronegative arthritis associated with psoriasis
Juvenile Psoriatic arthritis : Clinical features
- Polyarthritis + Enthesitis
- Psoriasis plaque, nail pitting
Rheumatic fever : Definition
- Inflammatory condition that develops as a result of abnormal immune complication of Streptococcus pyogenes infection
Rheumatic fever : Clinical presentation
- 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
Rheumatic fever : Joint involvement
- Migratory arthritis : different joints inflame and improve at different times
- Large joints : hot, swollen, painful joints
Rheumatic fever : Cardiac involvement
- Carditis : Pericarditis, Myocarditis and Endocarditis
- Auscultation
* Murmur : Mitral stenosis
* Pericardial rub
Rheumatic fever : Skin involvement
- Subcutaneous nodules : firm, painless nodules over extensor surface joints e.g. elbows
- Erythema marginatum rash : pink rings of varying size - on torso/limbs
Rheumatic fever : Nervous system involvement
- Sydenham Chorea : irregular uncontrolled rapid movement of limbs
Rheumatic fever : Investigation
- Throat swab : bacterial culture
- Antistreptococcal antibodies (ASO) - indicates recent strep infection
- ECHO, ECG and CXR
Rheumatic fever : Jone’s major criteria
- J – Joint arthritis
- O – Organ inflammation, such as carditis
- N – Nodules
- E – Erythema marginatum rash
- S – Sydenham chorea
Rheumatic fever : Jone’s minor criteria
- Fever
- ECG Changes (prolonged PR interval) without carditis
- Arthralgia without arthritis
- Raised inflammatory markers (CRP and ESR)
Rheumatic fever : Jone’s criteria for diagnosis
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
Rheumatic fever : Management
- Eradication of Streptococcal infection : Penicillin Vfor 10 days
- NSAID + Steroids : Joint pain and inflammation
Rheumatic fever : Complications
- Mitral stenosis
- Chronic Heart failure
Kawasaki disease : Definition
Systemic medium vessel vasculitis
Kawasaki disease : Pathophysiology
- 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
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
Kawasaki disease : Skin manifestation
- Widespread erythematous maculopapular rash
AND
- Desquamation (skin peeling) on the palms and soles.
Kawasaki disease : Diagnosis
- 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
Kawasaki disease : Management
- **High dose aspirin **: to reduce the risk of thrombosis
- IV immunoglobulins : to reduce the risk of coronary artery aneurysms
- ECHO : f/u for monitoring for coronary aneurysm
Kawasaki disease : Complication
Coronary Aneurysm
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 toIgA deposits in the blood vessels.
Henoch-Schonlein Purpura : Pathophysiology
- Abnormal immune response due to infectious trigger (unknown)
- Excess IgA antibodies releases } bind to antigens
- IgA immune complexes form
-
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
Henoch-Schonlein Purpura : Clinical features
-
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
Henoch-Schonlein Purpura : Investigations
- Full blood count and blood film for thrombocytopenia, sepsis and leukaemia
- CRP for sepsis
- Blood cultures for sepsis
- Renal profile for kidney involvement
- Serum albumin for nephrotic syndrome
- Urine dipstick for proteinuria
- Urine protein:creatinine ratio to quantify the proteinuria
- Blood pressure for hypertension (Nephrotic syndrome)
Henoch-Schonlein Purpura : Diagnosis
- Palpable purpura (not petichiae) + at least one of:
- Diffuse abdominal pain
- Arthritis or arthralgia
- IgA deposits on histology (biopsy)
- Proteinuria or haematuria
Henoch-Schonlein Purpura : Management
Management is supportive, with simple analgesia, rest and proper hydration.
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
