Paediatrics Rheumatology Flashcards
(93 cards)
1
Q
What is juvenile idiopathic arthritis (JIA)?
A
Condition affecting children and adolescents where autoimmune inflammation occurs in the joints
2
Q
When is JIA diagnosed?
A
Arthritis without any other cause lasting more than 6 weeks in a patient under age of 16
3
Q
What is juvenile idiopathic arthritis also known as?
A
Juvenile chronic arthritis
Juvenile rheumatoid arthritis
4
Q
What are the features of JIA?
A
Inflammatory arthritis:
- Joint pain
- Swelling
- Stiffness
5
Q
What are five key subtypes of JIA (associated with different blood tests)?
A
Systemic JIA
Polyarticular JIA
Oligoarticular JIA
Enthesitis related arthritis
Juvenile psoriatic arthritis
6
Q
What is systemic JIA also known as?
A
Still’s disease - systemic illness that can occur throughout childhood
7
Q
What are the features of systemic JIA?
A
Subtle salmon pink rash
High swinging fever
Enlarged lymph nodes
Weight loss
Joint inflammation and pain
Splenomegaly
Muscle pain
Pleuritis and pericarditis
8
Q
What are the blood test findings in systemic JIA?
A
Raised inflammatory markers (CRP, ESR)
Raised platelets and serum ferritin
ANA and rheumatoid factors are typically negative
9
Q
What is a complication of systemic JIA?
A
Macrophage activation syndrome (MAS) = severe activation of the immune system, causing:
- Disseminated intravascular coagulation (DIC)
- Anaemia
- Thrombocytopenia
- Bleeding
- Non-blanching rash
10
Q
What is a key finding in macrophage activation syndrome?
A
Low ESR
11
Q
What are the key non-infective differentials in children with fevers for more than 5 days?
A
Kawasaki disease
Still’s disease
Rheumatic fever
Leukaemia
12
Q
What is polyarticular JIA?
A
Idiopathic inflammatory arthritis in 5 joints or more
13
Q
Which joints are affected in polyarticular JIA?
A
Symmetrical - small joints of hands and feet and large joints e.g. hips and knees
14
Q
What are the systemic symptoms of polyarticular JIA?
A
Minimal systemic symtoms (unlike systemic JIA), can be:
Mild fever
Anaemia
Reduced growth
15
Q
What is the equivalent of rheumatoid arthritis in adults?
A
Polyarticular JIA
16
Q
Are patients with polyarticular JIA seropositive?
A
Most children are negative
Older children can be positive - disease pattern = more similar to RA in adults
17
Q
How many joints are affected in oligoarthritis? What is it also known as?
A
4 joints or less
Pauciarticular JIA
18
Q
Which joints are typically affected by oligoarticular JIA?
A
Larger joints, knee or ankle
19
Q
Who is typically affected by oligoarticular JIA?
A
Girls under the age of 6
20
Q
What is classically associated with oligoarticular JIA?
A
Anterior uveitis (referred to opthalmologist for management and follow up)
21
Q
What are the blood test findings on oligoarticular JIA?
A
ANA often positive
RF is negative
Inflammatory markers normal or mildly elevated
22
Q
Who is enthesitis-related arthritis most common in?
A
Male children over 6
23
Q
What is enthesitis-related arthritis the paediatric version of?
A
Seronegative spondyloarthropathy
- Ankylosing spondylitis
- Psoriatic arthritis
- Reactive arthritis
- IBD related arthritis
24
Q
What is enthesitis?
A
Inflammation of the insertion point of the tendon into the bone
25
What can cause **enthesitis**?
**Repetitive strain** during sporting activities
## Footnote
**Autoimmune inflammatory process**
26
What **imaging** can be used for **enthesitis**?
**MRI scan** of affected joint (can't distinguish cause)
27
Which **gene** is associated with **enthesitis-related arthritis**?
**HLA B27** **gene**
28
What is **look for** on **examination** of **enthesitis-related arthritis**?
**Psoriasis** (psoriatic plaques and nail pitting)
**Inflammatory bowel disease** (intermittent diarrhoea and rectal bleeding)
29
Why should patients with **enthesitis-related arthritis** see an **opthalmologist**?
As they are **prone to anterior uveitis**
30
What are the **key areas** to **palpate** in patients with **enthesitis**?
**Interphalangeal** joints in hand
**Wrist**
**Greater trochanter** on lateral aspect of hip
**ASIS** (quadriceps insertion)
**Patella** (quadriceps and patella tendon indertion)
**Base of achilles** at the calcaneus
**Metatarsal heads** on the base of the foot
31
What is **juvenile psoriatic arthritis**?
**Seronegative inflammatory arthritis** associated with **psoriasis**
32
Which **joints** are **affected** in **juvenile psoriatic arthritis?**
Varies: can be **symmetrical polyarthritis** affecting **small joints** similar to RA or can be **symmetrical** affecting **large joints** in the lower limb
33
What are the **signs** of **juvenile psoriatic arthritis**?
**Plaques** of **psoriasis** on the skin
**Nail pitting**
**Onycholysis -** separation of the nail from the nail bed
**Dactylitis -** inflammation of the full finger
**Enthesitis**
34
What is the **medical management** of **juvenile idiopathic arthritis**?
Coordinated by MDT:
* **NSAIDs** e.g. **ibuprofen**
* **Steroids** (oral / IM / intra-articular) in oligoarthritis
* **DMARDs** e.g. methtrexate, sulfasalazine, leflunomide
* **Biologic therapy** e.g. TNF inhibitors (etanercept, infliximab and adalimumab)
35
What is **Ehlers-Danlos syndrome**?
Umbrella term for **group of genetic conditions** causing **defective collagen** resulting in **hypermobility** of the patient's joints and abnormal **connective tissue** e.g. skin, bones, blood vessels and organs
36
What are the four main **types** of **Ehlers-Danlos**?
**Hypermobile Ehlers-Danlos syndrome**
**Classical Ehlers-Danlos syndrome**
**Vascular Ehlers-Danlos syndrome**
**Kyphoscoliotic Ehlers-Danlos syndrome**
37
What are the **features** of **hypermobild Ehlers-Danlos syndrome**?
Least severe type of Ehlers-Danlos
**Joint hypermobility**
**Soft and stetchy skin**
Gene has not yet been found
38
What are the **features** of **classical Ehlers-Danlos**?
**Stretchy skin**
**Smooth and velvety**
**Severe joint hypermobility**, joint pain and abnormal wound healing
**Lumps** over **pressure points** e.g. elbows
Prone to **hernias**, **prolapses**, **mitral regurgitation** and **aortic root dilatation**
39
What is the **mode of inheritance** for **classical Ehlers-Danlos**?
**Autosomal dominant**
40
What are the **features** of **vascular Ehlers-Danlos**?
**Thin, translucent skin**
Most dangerous form of EDS
Blood vessels are fragile
Skin, internal organs and arteries are **prone to rupturing**
41
What is the **management** of **vascular Ehlers-Danlos**?
Monitored for **vascular abnormalities** and told to **seek urgent medical attention** for sudden unexplained pain / bleeding
42
What is the **mode of inheritance** of **vascular Ehlers-Danlos?**
**Autosomal dominant**
43
What are the **features** of **Kyphoscoliotic Ehlers-Danlos**?
**Hypotonia** as a neonate / infant
**Kyphoscoliosis** as they grow
Joint hypermobility
Patients are **tall** and **slim**
Risk of **rupture** of **medium sized arteries**
44
What is the **mode of inheritance** of **kyphoscoliotic Ehlers-Danlos**?
**Autosomal dominant**
45
What are the **two main symptoms** in **hypermobile EDS**?
**Joint pain**
**Hypermobility**
46
What are the **other presenting features** of **hypermobile EDS**?
Joint dislocation e.g. shoulders or hips
Soft stretchy skin
Bruising
Poor wound healing
Bleeding
Headaches
GORD
Abdo pain
IBS
Menorrhagia and dysmenorrhoea
PROM in pregnancy
Urinary incontinence
Pelvic organ prolapse
Temporomandibular joint dysfunction
Myopia
47
What **score** is used for **hypermobility**? How is it calculated?
**Beighton Score**
Palms flat on floor with straight legs (score 1)
Elbows hyperextend
Knees hyperextend
Thumb can bend to touch the forearm
Little finger hyperextends past 90 degrees
48
What is it **important** to **exclude** in **EDS**?
**Marfan syndrom****e** (examine for high arch palate, arachnodactyly and arm span)
49
What is the **management** of **EDS**?
No cure - only **maintain healthy joints**, monitor for complications
* **Physio** - to strengthen joints
* **OT** to maximise function
* Maintain good posture
* **Psychology** to manage chronic condition and pain
50
What can EDS often lead to?
Generalised **osteoarthritis**
51
What can be the result of the **autonomic dysfunction** in **hypermobile** **EDS**?
**Postural** orthostatic **tachycardia** syndrome:
* Inappropriate **tachycardia** on sitting or **standing up**
* **Presyncope**, **syncope**, **headaches**, **nausea** and **tremor**
52
What is **Henoch-Schonlein Purpura** (HSP)?
**IgA vasculitis** presenting with **purpuric rash** affecting the **lower limbs** and **buttocks** in children
**Inflammation** also occurs in **affected organs** due to **IgA deposits** in the **blood vessels**
53
Where does **Henoch-Schonlein Purpura** affect?
Skin
Kidneys
GI tract
54
What is HSP triggered by?
Upper airway infection
Gastroenteritis
55
What are the **four classic features** of HSP?
**Purpura**
**Joint pain**
**Abdo pain**
**Renal involvement**
56
What is the **purpura** caused by?
**Inflammation** and **leaking of blood** from **small blood vessels** causing **purpura** - red-purple lumps under skin containing blood
57
How can **purpura** develop in **severe cases**?
Skin **ulceration** and **necrosis** develops
58
What is the difference between **arthritis** and **arthralgia**?
**Arthritis** is **pain** with **swelling** - arthralgia is just pain
59
What can severe cases of GI involvement with HSP lead to?
**Gastrointestinal haemorrhage**
**Intussusception**
**Bowel infarction**
60
What does HSP cause in the **kidneys**? What are the **features**?
**IgA nephritis** in the kidneys
**Microscopic/macroscopic haematuria**
**Proteinuria** (if 2+ on dip = nephrotic syndrome - oedema)
61
What are **differentials** for a **non-blanching rash**?
**Meningococcal septicaemia**
**Leukaemia**
**Idiopathic thrombocytopenic purpura**
**Haemolytic uraemic syndrome**
62
What **investigations** are there for **HSP**?
**FBC** and blood film for thrombocytopenia, sepsis and leukaemia
**Renal profile** for kidney damahe
**Serum albumin** for nephrotic syndrome
**CRP** for sepsis
**Urine dip** for proteinuria
**Urine protein:creatinine ratio** to quantify proteinuria
**Blood pressure** for hypertension
63
What **set of criteria** is used to diagnose **HSP**?
**EULAR/PRINTO/PRES criteria**
64
What is the EULAR/PRINTO/PRES criteria for diagnosing HSP?
Have **palpable purpura** (not petichiae) + at least one:
* Diffuse **abdo pain**
* **Arthritis** or **arthralgia**
* IgA deposits on histology (biopsy)
* Proteinuria or haematuria
65
What is the **management** of **HSP**?
Analgesia
Rest
Hydration
**Steroid** use is debatable - may shorten duration of illness but won't affect long term outcomes (may be used in severe GI pain / renal involvement)
66
How are patients with **HSP** monitored?
**Urine dip** for renal involvement
**Blood pressure** monitoring for HTN
67
What is the **prognosis** of **HSP**?
Abdo pain settles in a few days
Full **recovery** in **4** to **6** **weeks** (if no kidney involvement)
**1/3** have **recurrence** in **6 months**
**Small proportion** develop **end stage renal failure**
68
What is **Kawasaki disease**? What is it also known as?
Systemic, **medium-sized vessel vasculitis** affecting young children (under 5)
Also known as **mucocutaneous lymph node syndrome**
69
Who does **Kawasaki disease** typically affect?
Asian children, japanese and korean (boys)
## Footnote
**No clear cause or trigger**
70
What are the **key features** of **Kawasaki disease**?
**Persistent high fever** (above 39) for more than 5 days
**Widespread erythmatous maculopapular rash**
**Desquamation** (skin peeling) on palms and soles
71
What are the **other features** of **Kawasaki disease**?
**Strawberry tongue** (red tongue with large papillae)
## Footnote
**Cracked lips**
**Cervical lymphadenopathy**
**Bilateral conjunctivitis**
72
What are the **investigations** for **Kawasaki disease**?
**FBC** for anaemia, leukocytosis, thrombocytosis
**LFTs** for hypoalbuminaemia, and elevated liver enzymes
**Inflammatory markers** (particularly raised ESR)
**Urinalysis** raised WBCs without infection
**Echo** for coronary artery pathology
73
What are the **three phases** for **Kawasaki**?
**Acute phase**: 1-2 weeks - most unwell with fever, rash and lymphadenopathy
**Subacute phase**: 2-4 weeks - acute symptoms settle - desquamation and arthralgia (risk of coronary artery aneurysm)
**Convalesent stage**: 2-4 weeks - remaining symptoms settle - blood tests normalise and coronary aneurysms may regress
74
What is the **management** of **Kawasaki disease**?
**High dose aspirin** - reduce risk of **thrombosis**
**IV immunoglobulins** - reduce risk of **coronary artery aneurysm**
Close following with echo for coronary artery aneurysm
75
Why is **aspirin** usually avoided in children?
Risk of **Reye's syndrome**
76
What is **acute rheumatic fever**?
**Autoimmune condition** triggered by **streptococcus bacteria**
77
What is **acute rheumatic fever** caused by?
**Antibodies** created against **streptococcus** bacteria which also **targets tissues in the body**
78
What is **rheumatic fever** usually caused by?
**Group A beta-haemolytic streptococcal** typically **strep pyogenes** causing **tonsillitis**
79
What **type of hypersensitivity** reaction is **rheumatic fever**?
**Type 2 hypersensitivity reaction** where immune system begins attacking cells throughout body
80
How does **rheumatic fever** present?
**2-4** weeks following a streptococcal infection e.g. **tonsillitis**
* **Fever**
* **Joint pain**
* **Rash**
* **RoB**
* **Chorea**
* **Nodules**
81
Why does **rheumatic fever** cause **migratory arthritis**?
Affecting **large joints** which become **hot, swollen and painful** - become inlammed and improve at different times
82
What does the **carditis** in **rheumatic fever** cause?
**Pericarditis**
**Myocarditis**
**Endocarditis**
83
How does the **carditis** in rheumatic fever manifest?
**Tachycardia** or **bradycardia**
**Murmurs** from valvular heart disease, typically mitral valve disease
**Pericardial rub** on auscultation
**Heart failure**
84
What are the **two key skin findings** in **rheumatic fever**?
**Subcutaneous nodules** (firm, painless, extensor surfaces)
**Erythema marginatum** rash (pink rings, torso, proximal limbs)
85
What is the **nervous system involvement** in **rheumatic fever**?
**Chorea** - irregular, uncontrolled and rapid movements of the limbs
86
What is **chorea** also known as?
**Sydenham chorea**
**St Vitus' Dance**
87
What **investigations** are there for **rheumatic fever**?
**Throat swab** for bacterial culture
**ASO antibody titres**
**Echo**, **ECG** and **CXR**
88
How do the **anti-streptococcal antibodies** (ASO) vary with **rheumatic fever**?
89
When are **ASO** levels repeated?
**Confirm** negative test
Assess if levels rising / falling
90
What diagnostic **criteria** is used for **rheumatic fever**?
**Jones Criteria**
91
What does the **Jones criteria for diagnosis** involve?
**Evidence of** recent **streptococcal infection**
Plus: two major criteria or one major plus two minor
**Major**:
**J** - **Joint arthritis**
**O** - **Organ inflammation**
**N** - **Nodules**
**E** - **Erythema marginatum rash**
**S** - **Sydenham chorea**
**Minor**:
**F**ever
**E**CG changes (prolonged PR interval) without carditis
**A**rthralgia without arthritis
**R**aised inflammatory markers (CRP and ESR)
92
What is the **management** of **rheumatic fever**?
Treat strep infections to **prevent**
**NSAIDs** for **joint pain**
**Aspirin and steroids** for **carditis**
**Prophylactic abx** (oral / IM penicillin to **prevent further** strep infections)
**Monitor** for **complications**
93
What are the **complications** of **rheumatic fever**?
Recurrence
**Valvular heart disease** e.g. **mitral** stenosis
Chronic **heart failure**
