Rheumatology

Question 1

What is juvenile idiopathic arthritis

Answer 1

Autoimmune inflammation of the joints

Lasts > 6 weeks

Key features: joint pain, swelling, and stiffness

Question 2

What is systemic juvenile idiopathic arthritis

Answer 2

Aka Still’s disease

Presentation: subtle salmon-pink rash, high swinging fevers, enlarged lymph nodes, weight loss, joint inflammation and pain, splenomegaly, muscle pain, pleuritis, pericarditis

Investigations: raised inflammatory markers, raised platelets, high serum ferritin

Complications: macrophage activation syndrome

Question 3

What is polyarthritic idiopathic arthritis

Answer 3

Inflammatory arthritis of 5+ joints

Symmetrical

Presentation: mild fever, anaemia, reduced growth

Equivalent to rheumatoid arthritis in adults

Question 4

What is oligoarticular juvenile idiopathic arthritis

Answer 4

> 5 joints affected

Tends to affect large joints

Usually in girls < 6

Associated with anterior uveitis

ANA positive

Question 5

What is enthesis-related arthritis

Answer 5

A type of juvenile idiopathic arthritis

Males > 6

Paediatric version of spondyloarthropathy

Investigate with MRI

Key areas of tenderness: interphalangeal joints, wrist, over greater trochanter, tendon insertions around patella, base of achilles tendon

Question 6

What is the management of juvenile idiopathic arthritis

Answer 6

MDT care

NSAIDs

Steroids

DMARDs (methotrexate, sulfasalazine)

Biological therapies (TNF inhibitors - infliximab))

Question 7

What is Ehler-Danlos syndrome

Answer 7

Group of genetic conditions that cause defects in collagen

Question 8

What are the types of Ehler-Danlos syndrome

Answer 8

Hypermobility EDS (hypermobility, soft and stretchy skin)

Classic EDS (stretchy skin, severe hypermobility, joint pain, abnormal wound healing)

Vascular EDS (blood vessels very fragile)

Kyphoscoliotic EDS

Question 9

How might a patient with Ehler-Danlos syndrome present

Answer 9

Joint hypermobility

Joint pain

Joint dislocation

Soft, stretchy skin

Easily bruised

Poor wound healing

Bleeding headaches

Dizziness, syncope

GORD

Abdominal pain

IBS

Menorrhagia

PROM

Urinary incontinence

Pelvic organ prolapse

TMJ dysfunction

Myopenia

Question 10

What is the Beighton score for hypermobility in Ehler-Danlos syndrome

Answer 10

1 point for each side of the body (max 9)

Palms flat on floor with straight legs

Elbows hyperextended

Knees hyperextended

Thumb bent to touch forearm

Little finger hyperextended past 90 degrees

Question 11

What is the management for Ehler-Danlos syndrome

Answer 11

Physio

Occupational therapy

Psychology

Question 12

What is Henoch-Schonlein purpura

Answer 12

IgA vasculitis

IgA deposited in blood vessels

Get purpuric rash on lower limbs and buttocks

Triggered by URTI/gastroenteritis

Mostly < 10s

Question 13

How might a patient with Henoch-Schonlein purpura present

Answer 13

Purpura

Joint pain

Abdominal pain (GI haemorrhage, intussusception, bowel infarction)

Renal involvement (haematuria and proteinuria)

Question 14

What are the investigations for Henoch-Schonlein purpura

Answer 14

Bloods

Blood cultures

Urine dip

Blood pressure

Question 15

What is the management for Henoch-Schonlein purpura

Answer 15

Supportive care (simple analgesia, rest, proper hydration)

Consider steroids

Question 16

What is the prognosis for Henoch-Schonlein purpura

Answer 16

Without renal involvement, fully recover in 4-6 weeks

1/3 have recurrence in 6 months

Some develop end stage renal failure

Question 17

What is Kawasaki disease

Answer 17

Vasculitis of medium sized vessels

Usually in under 5s

More common in east asians

Question 18

How might a patient with Kawasaki disease present

Answer 18

Persistent high fever

Widespread erythematous maculopapular rash

Desquamation (skin peeling, on palms and soles)

Strawberry tongue

Cervical lymphadenopathy

Bilateral conjunctivitis

Question 19

What are the investigations for Kawasaki disease

Answer 19

Bloods

Urine dip

ECHO

Question 20

What is the management for Kawasaki disease

Answer 20

High dose aspirin (reduce risk of thrombosis)

IV immunoglobulins (reduce risk of coronary artery aneurysms)

Question 21

What is rheumatic fever

Answer 21

Autoimmune condition triggered by streptococcus bacteria

Multisystem disorder

Type 2 hypersensitivity (2-4 weeks after initial infection)

Question 22

How might a patient with rheumatic fever present

Answer 22

Fever

Joint pain

Rash

Shortness of breath

Chorea (irregular, uncontrolled, rapid movements of limbs)

Nodules

Heart involvement (pericarditis, myocarditis, endocarditis)

Question 23

What are the investigations for rheumatic fever

Answer 23

Throat swab

ASO antibody titre

ECHO

ECG

Chest X-ray

Question 24

What is the Jones criteria for diagnosing rheumatic fever

Answer 24

2 major, or 1 major and 2 minor

Major (jones): joint arthritis, organ inflammation, nodules, erythema marginatum rash, sydenham chorea

Minor (fear): fever, ECG changes, arthralgia without arthritis, raised inflammatory markers

Question 25

What is the management for rheumatic fever

Answer 25

Prevention: 10 days penicillin V

NSAIDs (joint pain)

Aspirin and steroids (carditis)

Prophylactic antibiotics

Question 26

What are the complications of rheumatic fever

Answer 26

Recurrence

Valvular heart disease

Chronic heart failure