Rheumatology Flashcards
What is JIA?
Juvenile idiopathic arthritis
What is juvenile idiopathic arthritis?
Condition affecting children and adolescents where autoimmune inflammation occurs in the joints
When is JIA diagnosed?
Where there is arthritis with no other known cause, lasting > 6 weeks in patients <16
What are the key features of JIA?
Joint pain
Swelling
Stiffness
What are the 5 key subtypes of JIA?
Systematic JIA Polyarticular JIA Oligoarticular JIA Enthesitis related arthritis Juvnile psoriatic arthritis
What is the difference between each of the 5 JIA subtypes?
The serology (blood tests)
What is systemic JIA?
Systemic idiopathic inflammatory condition that occurs in childhood
What is systemic JIA also known as?
Still’s disease
What are the typical features of systemic JIA?
Subtle salmon-pink rash High fevers Enlarged lymph nodes Weight loss Joint inflammation/ pain Splenomegaly Muscle pain Pleuritis and pericarditis
What will be raised in systemic JIA and what will stay negative?
Raised inflammatory markers (CRP/ ESR0, platelets, serum ferritin
Antinuclear antibodies and rheumatoid factors stay negative
What is a key complication of systemic JIA?
Macrophage activation syndrome
What is macrophage activation syndrome?
Severe activation of the immune system with massive immune response
How does MAS present?
Acutely unwell child DIC Anaemia Thrombocytopenia Bleeding Non-blanching rash Low ESR
In a child with a fever for > 5 days, what are the key non-infective differentials?
Kawasaki disease
Still’s disease
Rheumatic fever
Leukaemia
What is polyarticular JIA?
Idiopathic inflammatory arthritis in 5 joints or more (equivalent of RA in adults)
Where is usually affected in polyarticular JIA?
Symmetrical in small joints of hands and feet, as well as large joints like hips and knees
Does polyarticular JIA present with systemic symptoms?
May be mild (e.g. mild fever, anaemia, reduced growth)
What is the serology of polyartiular JIA?
seronegative (negative for rheumatoid factor)
What is oligoarticular JIA?
JIA involving 4 joints or less (usually only a single joint)
Where does oligoarticular JIA usually affect?
A larger joint (e.g. ankle or knee)
In which patients is oligoarticular JIA more common?
Girls < 6
What is a common associated feature with oligoarticular JIA?
Anterior uveitis
What is the serology of oligoarticular JIA?
Normal inflammatory markers
Seronegative (no rheumatoid factor)
Positive antinuclear antibodies
What is enthesitis-related arthritis?
Inflammatory arthritis in the joints as well as enthesitis
What is enthesitis?
Inflammation of the entheses
What is an enthesis?
Point where a tendon of a muscle inserts into a bone
In which patients is enthesitis-related arthritis more common?
Male children >6
What is enthesitis-related arthritis comparable to in adults?
Seronegative spondylarthopathies
What are the causes of enthesitis?
Traumatic stress (e.g. repeptitive strain during sport) Autoimmune inflammatory process
How is enthesitis diagnosed?
MRI scan
What is enthesitis-related arthritis associated with?
HLA B27 gene
What other signs and symptoms should be looked for in patients with enthesitis-related arthritis?
Psoriasis
IBD symptoms
Anterior uveitis
Tenderness to entheses
What is juvenile psoriatic arthritis?
Seronegative inflammatory arthritis associated with psoriases
What are the examination signs of juvenile psoriatic arthritis?
Plaques of psoriases Pitting of nails Onycholysis Dactylitis Enthesitis
How is JIA managed?
MDT NSAIDs Steroids (Oral, IM or intra-articular) DMARDs (e.g. Methotrexate) Biologic therapy (e.g. anti-TNF's )
What is EDS?
Ehlers- Danlos syndrome
What is Ehlers- Danlos syndrome?
Group of genetic conditions that cause defects in collagen
What does collagen defect cause?
Hypermobility of joints and abnormalities in connective tissues
What are the different types of Ehlers-Danlos syndrome?
Hypermobile
Classical
Vascular
Kyphoscoliotic
What is the most common EDS?
Hypermobile Ehlers-Danlos syndrome
What are the key features of hypermobile EDS?
Joint pain and hypermobility
Soft, stretchy skin
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
What are patients with classical EDS more at risk of?
Hernias
Prolapses
Mitral regurg
Aortic root dilation
What is the inheritance pattern of classical EDS?
Autosomal dominant
What is the most dangerous form of EDS?
Vascular EDS
What happens in vascular EDS?
The blood vessels are partiularly fragile as a result of defective collagen
What are the characteristic features of vascular EDS?
Thin, transluscent skin
What is the inheritance pattern of vascular and kyphoscoliotic EDS?
Autosomal dominant
What are the key features of kyphoscoliotic EDS?
Hypotonia as neonate and infant
Kyphoscoliosis as they grow
Joint hypermobility
Tall and slim
What is the key complication of kyphoscoliotic EDS?
Rupture of medium sized arteries
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
What score is used to assess hypermobility?
Beighton score
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
How is hypermobile EDS diagnosed?
Using beighton score
What should be excluded when diagnosing IDS and how?
Marfan syndrome by examining for high arch palate, arachnodactyly and arm span
How is EDS managed?
Maintaining healthy joints (OT, PT)
Monitor for complications
Minimise symptoms
What do patients with hypermobility often develop?
Osteoarthritis at a younger age
What is a key complication of hypermobile EDS?
Postural orthostatic tachycardia syndrome (POTS)
What is POTS and why does it occur?
Autonomic dysfunction causes inappropriate tachycardia on sitting or standing, resulting in syncope, headaches, nausea ect
What is HSP?
Henoch-Schonlein Purpura
What kind of condition is HSP?
IgA vasculitis
What happens in HSP?
Inflammation occurs in affected organs due to IgA deposits in blood vessels
What organs are particularly affected by HSP?
Skin
Kidneys
GI tract
What usually triggers HSP?
URTI
Gastroenteritis
In which age range is HSP most common?
Children under 10
What are the 4 classic features of HSP?
Purpura (100%) Joint pain (75%) Abdominal pain (50%) IgA nephritis (50%)
What causes the rash in HSP?
Inflammation and leaking of blood from small blood vessels under the skin
What are purpura?
Red-purple lumps under the skin containing blood
Where are the common sites for purpura in HSP?
Start on legs and spread to buttocks
What joints are most affected by arthralgia/ arthritis as a result of HSP?
Knees and ankles
What can associated abdominal features with HSP lead to in severe cases?
GI haemorrhage
Intussusception
Bowel infarction
What does IgA nephritis lead to?
Haematuria and proteinuria
What differentials should be excluded when diagnosing HSP?
Meningococcal septicaemia
Leukaemia
ITP
Haemolytic uraemic syndrome
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
What is the criteria for diagnosing HSP?
Palpable purpura + at least on of:
- Diffuse abdominal pain
- Arthritis/ arthralgia
- IgA deposits on histology
- Protein/ haematuria
What is the management of HSP?
Supportive
Close monitoring with repeat urine dipstick and blood pressure for renal involvement
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
What kind of condition is Kawasaki disease?
Systemic medium-sized vessel vasculitis
What is kawasaki disease also known as?
Mucocutaneous lymph node syndrome
What age range is typically affected by kawasaki?
Young children (<5)
In which children is Kawasaki disease more common?
Asian boys
What is the key complication of kawasaki?
Coronary artery aneurysm
What is the key feautre that would make you consider kawasaki disease?
Persisent high fever for > 5 days
What are the key skin findings in kawasaki?
Widespread erythematous maculopapular rash
Desquamation (skin peeling) on palms and soles
What are the key features of kawasaki?
Prolonged fever Rash Skin peeling Strawberry tongue Cracked lips Cervical lymphadenopathy Bilateral conjunctivitis
What investigations can help diagnose kawasaki disease?
FBC LFTs Inflammatory markers Urinalysis Echocardiogram
What will FBC show in kawasaki?
Anaemia
Leukocytosis
Thrombocytosis
What might urinalysis show in kawasaki disease?
Raised WCC without infection
What are the 3 phases of kawasaki disease?
Acute
Subacute
Convalescent
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
What happens in the subacute phase and how long does it last?
Acute symptoms settle, desquamation and arthralgia occur.
Lasts 2-4 weeks
What is the convalescent stage and how long does it last?
Remaining symptoms settle blood tests slowly return to normal
Lasts 2-4 weeks
What are the two first line medications used to treat Kawasaki disease?
High dose aspirin
IV immunoglobulins
Why are patients with kawasaki given high dose aspirin?
To reduce the risk of thrombosis
Why are patients with Kawasaki given Iv immunoglobulins?
To reduce the risk of coronary artery aneurysms
Is aspirin usually given to children?
No-Kawasaki is one of the rare cases
What kind of condition is acute rheumatic fever?
Autoimmune condition
What triggers rheumatic fever?
Streptococcus bacteria
What is the most common cause of rheumatic fever?
Strep pygones causing tonsillits
What happens in rheumatic fever?
Antibodies are created against strep bacteria which also target tissues in the body.
What kind of reaction does rheumatic fever result in?
Type 2 hypersensitivity reaction
How long after initial infection does they hypersensitivity reaction usually occur?
2-4 weeks later
How does rheumatic fever present?
Following strep infection Fever Joint pain Rash SOB Chorea Nodules
How does rheumatic fever affect the joints?
Causes migratory arthritis, making large joints hot, swollen and painful
How does rheumatic fever affect the heart?
Causes carditis throughout (pericarditis, myocarditis & endocarditis), leading to: Tachy/bradycardia Murmurs Pericardial rub Heart failure
How does rheumaic fever affect the skin?
Subcutaeneous nodules &
Erythema marginatum rash
Where do nodules form in rheumatic fever?
Over extensor surfaces of joints
What is the key nervous system symptom in rheumatic fever?
Chorea
What is chorea?
Irregular, uncontrolled and rapid movements of the limbs
What investigations help support diagnosis of rheumatic fever?
Throat swab for bacterial culture
ASO antibody titres
Echo, ECG and CXR for heart involvement
What are ASO antibodies?
Anti-streptococcal antibodies
What do ASO antibodies indicate?
A recent streptococcus infection
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
What is the criteria for diagnosis of rheumatic fever?
Jones criteria
What is the Jones criteria for diagnosing rheumatic fever?
Evidence of recent strep infection + Two major citeria OR one major and two minor criteria
What is the mnemonic for the Jones criteria?
JONES-FEAR
What are the major criteria used for diagnosing rheumatic fever?
Joint arthritis Organ inflammation Nodules Erythema marginatum rash Sydenham chorea
What is the minor criteria used for diagnosing rheumatic fever?
Fever
ECG changes
Arthralgia (without arthritis)
Raised inflammatory markers
What can prevent the development of rheumatic fever?
Treatment of strep infections with antibiotics (e.g. penicillin V)
How are patients with rheumatic fever managed?
Refer to specialist
NSAIDS
Aspirin and steroids
Prophylactic antibiotics
What are the key complications of rheumatic fever?
Recurrene
Valcular heart disease
Chronic heart failure
What is the most common valvular heart disease associated with rheumatic fever?
Mitral stenosis
