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Paediatrics > Paediatric rheumatology > Flashcards

Paediatric rheumatology Flashcards

1
Q

Juvenile idiopathic arthritis : definition

A
  1. A condition that affects children and adolescents where autoimmune inflammation occurs in the joints
  2. Arthritis w/o underlying cause for > 6 weeks in patients < 16 years
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2
Q

Juvenile idiopathic arthritis : Different types

A
  1. Systemic JIA
  2. Polyarticular JIA
  3. Oligoarticular JIA
  4. Enthesis-related arthritis
  5. Juvenile psoriatic arthritis
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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
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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

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5
Q

Polyarticular JIA : Clinical features

A
  1. Idiopathic inflammatory arthritis in >5 joints
  2. Mild systemic symptoms
  3. Similar to RA in children
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6
Q

Oligoarticular JIA : Clinical features

A
  1. < 4 joints affected : usually affects only a single large joint e.g. Knee or Ankle
  2. Incidence : Girls < 6 years of age
  3. Associated with : Anterior uveitis
  4. Bloods : **Antinuclear antibody + **
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7
Q

Enthesis related JIA : Definition

A
  1. Inflammation at the site where tendons and ligaments attach to the bone
  2. Seronegative spondyloarthritis : HLAB27 gene
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8
Q

Enthesis related JIA : Clinical features

A
  1. HLAB27 + association : IBD, Psoriasis, uveitis
  2. Tenderness at entheses : Achilies tendon, plantar fascia, knee hip
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9
Q

Juvenile Psoriatic arthritis : Definition

A
  1. Seronegative arthritis associated with psoriasis
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10
Q

Juvenile Psoriatic arthritis : Clinical features

A
  1. Polyarthritis + Enthesitis
  2. Psoriasis plaque, nail pitting
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11
Q

Rheumatic fever : Definition

A
  1. Inflammatory condition that develops as a result of abnormal immune complication of Streptococcus pyogenes infection
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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

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13
Q

Rheumatic fever : Joint involvement

A
  1. Migratory arthritis : different joints inflame and improve at different times
  2. Large joints : hot, swollen, painful joints
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14
Q

Rheumatic fever : Cardiac involvement

A
  1. Carditis : Pericarditis, Myocarditis and Endocarditis
  2. Auscultation
    * Murmur : Mitral stenosis
    * Pericardial rub
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15
Q

Rheumatic fever : Skin involvement

A
  1. Subcutaneous nodules : firm, painless nodules over extensor surface joints e.g. elbows
  2. Erythema marginatum rash : pink rings of varying size - on torso/limbs
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16
Q

Rheumatic fever : Nervous system involvement

A
  1. Sydenham Chorea : irregular uncontrolled rapid movement of limbs
17
Q

Rheumatic fever : Investigation

A
  1. Throat swab : bacterial culture
  2. Antistreptococcal antibodies (ASO) - indicates recent strep infection
  3. ECHO, ECG and CXR
18
Q

Rheumatic fever : Jone’s major criteria

A
  1. J – Joint arthritis
  2. O – Organ inflammation, such as carditis
  3. N – Nodules
  4. E – Erythema marginatum rash
  5. S – Sydenham chorea
19
Q

Rheumatic fever : Jone’s minor criteria

A
  1. Fever
  2. ECG Changes (prolonged PR interval) without carditis
  3. Arthralgia without arthritis
  4. 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
  1. Eradication of Streptococcal infection : Penicillin Vfor 10 days
  2. NSAID + Steroids : Joint pain and inflammation
22
Q

Rheumatic fever : Complications

A
  1. Mitral stenosis
  2. Chronic Heart failure
23
Q

Kawasaki disease : Definition

A

Systemic medium vessel vasculitis

24
Q

Kawasaki disease : Pathophysiology

A
  1. Activation of immune system : Immune response to infectious agent
  2. Vasculitis : activation of endothelial cells in the blood vessels } vasculitis of small and medium size vessels (Coronary artery)
  3. Damage to endothelium : Cytokine release, formation of immune complexes
  4. Thrombosis and aneurysm formation : due to damage to the endothelium
25
Q

Kawasaki disease : Clinical features

A

Persistent high fever > 5 days with ;
1. **Strawberry tongue (red tongue with large papillae)
2. *Cracked lips
3. *Cervical lymphadenopathy
4. Bilateral conjunctivitis

26
Q

Kawasaki disease : Skin manifestation

A
  1. Widespread erythematous maculopapular rash

AND

  1. Desquamation (skin peeling) on the palms and soles.
27
Q

Kawasaki disease : Diagnosis

A
  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
Q

Kawasaki disease : Management

A
  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
Q

Kawasaki disease : Complication

A

Coronary Aneurysm

30
Q

Henoch-Schonlein Purpura : Definition

A

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.

31
Q

Henoch-Schonlein Purpura : Pathophysiology

A
  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
Q

Henoch-Schonlein Purpura : Clinical features

A
  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
Q

Henoch-Schonlein Purpura : Investigations

A
  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
Q

Henoch-Schonlein Purpura : Diagnosis

A
  • 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
Q

Henoch-Schonlein Purpura : Management

A

Management is supportive, with simple analgesia, rest and proper hydration.

36
Q

Henoch-Schonlein Purpura : Prognosis

A
  • 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
37
Q
A

Paediatrics (13 decks)

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