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Rheumatology Flashcards

1
Q

What is JIA?

A

Juvenile idiopathic arthritis

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2
Q

What is juvenile idiopathic arthritis?

A

Condition affecting children and adolescents where autoimmune inflammation occurs in the joints

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3
Q

When is JIA diagnosed?

A

Where there is arthritis with no other known cause, lasting > 6 weeks in patients <16

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4
Q

What are the key features of JIA?

A

Joint pain
Swelling
Stiffness

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5
Q

What are the 5 key subtypes of JIA?

A 
Systematic JIA
Polyarticular JIA
Oligoarticular JIA
Enthesitis related arthritis
Juvnile psoriatic arthritis
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6
Q

What is the difference between each of the 5 JIA subtypes?

A

The serology (blood tests)

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7
Q

What is systemic JIA?

A

Systemic idiopathic inflammatory condition that occurs in childhood

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8
Q

What is systemic JIA also known as?

A

Still’s disease

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9
Q

What are the typical features of systemic JIA?

A 
Subtle salmon-pink rash
High fevers
Enlarged lymph nodes
Weight loss
Joint inflammation/ pain
Splenomegaly
Muscle pain
Pleuritis and pericarditis
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10
Q

What will be raised in systemic JIA and what will stay negative?

A

Raised inflammatory markers (CRP/ ESR0, platelets, serum ferritin
Antinuclear antibodies and rheumatoid factors stay negative

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11
Q

What is a key complication of systemic JIA?

A

Macrophage activation syndrome

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12
Q

What is macrophage activation syndrome?

A

Severe activation of the immune system with massive immune response

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13
Q

How does MAS present?

A 
Acutely unwell child
DIC
Anaemia
Thrombocytopenia
Bleeding
Non-blanching rash 
Low ESR
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14
Q

In a child with a fever for > 5 days, what are the key non-infective differentials?

A

Kawasaki disease
Still’s disease
Rheumatic fever
Leukaemia

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15
Q

What is polyarticular JIA?

A

Idiopathic inflammatory arthritis in 5 joints or more (equivalent of RA in adults)

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16
Q

Where is usually affected in polyarticular JIA?

A

Symmetrical in small joints of hands and feet, as well as large joints like hips and knees

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17
Q

Does polyarticular JIA present with systemic symptoms?

A

May be mild (e.g. mild fever, anaemia, reduced growth)

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18
Q

What is the serology of polyartiular JIA?

A

seronegative (negative for rheumatoid factor)

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19
Q

What is oligoarticular JIA?

A

JIA involving 4 joints or less (usually only a single joint)

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20
Q

Where does oligoarticular JIA usually affect?

A

A larger joint (e.g. ankle or knee)

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21
Q

In which patients is oligoarticular JIA more common?

A

Girls < 6

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22
Q

What is a common associated feature with oligoarticular JIA?

A

Anterior uveitis

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23
Q

What is the serology of oligoarticular JIA?

A

Normal inflammatory markers
Seronegative (no rheumatoid factor)
Positive antinuclear antibodies

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24
Q

What is enthesitis-related arthritis?

A

Inflammatory arthritis in the joints as well as enthesitis

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25
What is enthesitis?
Inflammation of the entheses
26
What is an enthesis?
Point where a tendon of a muscle inserts into a bone
27
In which patients is enthesitis-related arthritis more common?
Male children >6
28
What is enthesitis-related arthritis comparable to in adults?
Seronegative spondylarthopathies
29
What are the causes of enthesitis?
``` Traumatic stress (e.g. repeptitive strain during sport) Autoimmune inflammatory process ```
30
How is enthesitis diagnosed?
MRI scan
31
What is enthesitis-related arthritis associated with?
HLA B27 gene
32
What other signs and symptoms should be looked for in patients with enthesitis-related arthritis?
Psoriasis IBD symptoms Anterior uveitis Tenderness to entheses
33
What is juvenile psoriatic arthritis?
Seronegative inflammatory arthritis associated with psoriases
34
What are the examination signs of juvenile psoriatic arthritis?
``` Plaques of psoriases Pitting of nails Onycholysis Dactylitis Enthesitis ```
35
How is JIA managed?
``` MDT NSAIDs Steroids (Oral, IM or intra-articular) DMARDs (e.g. Methotrexate) Biologic therapy (e.g. anti-TNF's ) ```
36
What is EDS?
Ehlers- Danlos syndrome
37
What is Ehlers- Danlos syndrome?
Group of genetic conditions that cause defects in collagen
38
What does collagen defect cause?
Hypermobility of joints and abnormalities in connective tissues
39
What are the different types of Ehlers-Danlos syndrome?
Hypermobile Classical Vascular Kyphoscoliotic
40
What is the most common EDS?
Hypermobile Ehlers-Danlos syndrome
41
What are the key features of hypermobile EDS?
Joint pain and hypermobility | Soft, stretchy skin
42
What are the key features of classical EDS?
Remarkably stretchy skin (smooth and velvety) Severe joint hypermobility, joint pain and abnormal wound healing Lumps over pressure points
43
What are patients with classical EDS more at risk of?
Hernias Prolapses Mitral regurg Aortic root dilation
44
What is the inheritance pattern of classical EDS?
Autosomal dominant
45
What is the most dangerous form of EDS?
Vascular EDS
46
What happens in vascular EDS?
The blood vessels are partiularly fragile as a result of defective collagen
47
What are the characteristic features of vascular EDS?
Thin, transluscent skin
48
What is the inheritance pattern of vascular and kyphoscoliotic EDS?
Autosomal dominant
49
What are the key features of kyphoscoliotic EDS?
Hypotonia as neonate and infant Kyphoscoliosis as they grow Joint hypermobility Tall and slim
50
What is the key complication of kyphoscoliotic EDS?
Rupture of medium sized arteries
51
What other symptoms may you get in hypermobile EDS?
``` Hypermobility Joint pain and dislocations Soft stretchy skin Easy bruising/ poor healing Bleeding Headaches Syncope GORD Abdo pain/ IBS Menorrhagia/ dysmenorrhea Urinary prolapse ect ```
52
What score is used to assess hypermobility?
Beighton score
53
What things are assessed in Beighton score?
Palms flat on floor with straight legs Elbows/ knees hyperextension Thumb bend to touch forearm Little finger hyperextends past 90 degrees
54
How is hypermobile EDS diagnosed?
Using beighton score
55
What should be excluded when diagnosing IDS and how?
Marfan syndrome by examining for high arch palate, arachnodactyly and arm span
56
How is EDS managed?
Maintaining healthy joints (OT, PT) Monitor for complications Minimise symptoms
57
What do patients with hypermobility often develop?
Osteoarthritis at a younger age
58
What is a key complication of hypermobile EDS?
Postural orthostatic tachycardia syndrome (POTS)
59
What is POTS and why does it occur?
Autonomic dysfunction causes inappropriate tachycardia on sitting or standing, resulting in syncope, headaches, nausea ect
60
What is HSP?
Henoch-Schonlein Purpura
61
What kind of condition is HSP?
IgA vasculitis
62
What happens in HSP?
Inflammation occurs in affected organs due to IgA deposits in blood vessels
63
What organs are particularly affected by HSP?
Skin Kidneys GI tract
64
What usually triggers HSP?
URTI | Gastroenteritis
65
In which age range is HSP most common?
Children under 10
66
What are the 4 classic features of HSP?
``` Purpura (100%) Joint pain (75%) Abdominal pain (50%) IgA nephritis (50%) ```
67
What causes the rash in HSP?
Inflammation and leaking of blood from small blood vessels under the skin
68
What are purpura?
Red-purple lumps under the skin containing blood
69
Where are the common sites for purpura in HSP?
Start on legs and spread to buttocks
70
What joints are most affected by arthralgia/ arthritis as a result of HSP?
Knees and ankles
71
What can associated abdominal features with HSP lead to in severe cases?
GI haemorrhage Intussusception Bowel infarction
72
What does IgA nephritis lead to?
Haematuria and proteinuria
73
What differentials should be excluded when diagnosing HSP?
Meningococcal septicaemia Leukaemia ITP Haemolytic uraemic syndrome
74
What investigations are done into HSP?
``` FBC and Blood film U&E's Serum albumin (nephrotic syndrome) CRP (Sepsis) Blood cultures (sepsis) Urine dipstick + Protein:creatinine ration Blood pressure ```
75
What is the criteria for diagnosing HSP?
Palpable purpura + at least on of: - Diffuse abdominal pain - Arthritis/ arthralgia - IgA deposits on histology - Protein/ haematuria
76
What is the management of HSP?
Supportive | Close monitoring with repeat urine dipstick and blood pressure for renal involvement
77
What is the prognosis for HSP?
Abdominal pain settles in a few days If no kidney involvement, fully recover within 4-6 weeks 1/3 patients have recurrence within 6 months
78
What kind of condition is Kawasaki disease?
Systemic medium-sized vessel vasculitis
79
What is kawasaki disease also known as?
Mucocutaneous lymph node syndrome
80
What age range is typically affected by kawasaki?
Young children (<5)
81
In which children is Kawasaki disease more common?
Asian boys
82
What is the key complication of kawasaki?
Coronary artery aneurysm
83
What is the key feautre that would make you consider kawasaki disease?
Persisent high fever for > 5 days
84
What are the key skin findings in kawasaki?
Widespread erythematous maculopapular rash | Desquamation (skin peeling) on palms and soles
85
What are the key features of kawasaki?
``` Prolonged fever Rash Skin peeling Strawberry tongue Cracked lips Cervical lymphadenopathy Bilateral conjunctivitis ```
86
What investigations can help diagnose kawasaki disease?
``` FBC LFTs Inflammatory markers Urinalysis Echocardiogram ```
87
What will FBC show in kawasaki?
Anaemia Leukocytosis Thrombocytosis
88
What might urinalysis show in kawasaki disease?
Raised WCC without infection
89
What are the 3 phases of kawasaki disease?
Acute Subacute Convalescent
90
How long is the acute phase of kawasaki and what happens in it?
Child is unwell with fever, rash and lymphadenopathy | Lasts 1-2 weeks
91
What happens in the subacute phase and how long does it last?
Acute symptoms settle, desquamation and arthralgia occur. | Lasts 2-4 weeks
92
What is the convalescent stage and how long does it last?
Remaining symptoms settle blood tests slowly return to normal Lasts 2-4 weeks
93
What are the two first line medications used to treat Kawasaki disease?
High dose aspirin | IV immunoglobulins
94
Why are patients with kawasaki given high dose aspirin?
To reduce the risk of thrombosis
95
Why are patients with Kawasaki given Iv immunoglobulins?
To reduce the risk of coronary artery aneurysms
96
Is aspirin usually given to children?
No-Kawasaki is one of the rare cases
97
What kind of condition is acute rheumatic fever?
Autoimmune condition
98
What triggers rheumatic fever?
Streptococcus bacteria
99
What is the most common cause of rheumatic fever?
Strep pygones causing tonsillits
100
What happens in rheumatic fever?
Antibodies are created against strep bacteria which also target tissues in the body.
101
What kind of reaction does rheumatic fever result in?
Type 2 hypersensitivity reaction
102
How long after initial infection does they hypersensitivity reaction usually occur?
2-4 weeks later
103
How does rheumatic fever present?
``` Following strep infection Fever Joint pain Rash SOB Chorea Nodules ```
104
How does rheumatic fever affect the joints?
Causes migratory arthritis, making large joints hot, swollen and painful
105
How does rheumatic fever affect the heart?
``` Causes carditis throughout (pericarditis, myocarditis & endocarditis), leading to: Tachy/bradycardia Murmurs Pericardial rub Heart failure ```
106
How does rheumaic fever affect the skin?
Subcutaeneous nodules & | Erythema marginatum rash
107
Where do nodules form in rheumatic fever?
Over extensor surfaces of joints
108
What is the key nervous system symptom in rheumatic fever?
Chorea
109
What is chorea?
Irregular, uncontrolled and rapid movements of the limbs
110
What investigations help support diagnosis of rheumatic fever?
Throat swab for bacterial culture ASO antibody titres Echo, ECG and CXR for heart involvement
111
What are ASO antibodies?
Anti-streptococcal antibodies
112
What do ASO antibodies indicate?
A recent streptococcus infection
113
What happens to ASO antibody levels after an acute strep infection?
Rise over 2-4 weeks Peak around 3-6 weeks Gradually fall over 3-12 months
114
What is the criteria for diagnosis of rheumatic fever?
Jones criteria
115
What is the Jones criteria for diagnosing rheumatic fever?
Evidence of recent strep infection + Two major citeria OR one major and two minor criteria
116
What is the mnemonic for the Jones criteria?
JONES-FEAR
117
What are the major criteria used for diagnosing rheumatic fever?
``` Joint arthritis Organ inflammation Nodules Erythema marginatum rash Sydenham chorea ```
118
What is the minor criteria used for diagnosing rheumatic fever?
Fever ECG changes Arthralgia (without arthritis) Raised inflammatory markers
119
What can prevent the development of rheumatic fever?
Treatment of strep infections with antibiotics (e.g. penicillin V)
120
How are patients with rheumatic fever managed?
Refer to specialist NSAIDS Aspirin and steroids Prophylactic antibiotics
121
What are the key complications of rheumatic fever?
Recurrene Valcular heart disease Chronic heart failure
122
What is the most common valvular heart disease associated with rheumatic fever?
Mitral stenosis

Paeds (18 decks)

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