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Y4 Paediatrics > 20 - Rheumatology > Flashcards

20 - Rheumatology Flashcards

1
Q

If a child has a fever for more than 5 days, what key non-infectious differentials do you need to consider?

A
  • Still’s Disease
  • Kawasaki Disease
  • Leukaemia
  • Rheumatic fever
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2
Q

What examinations do you need to do for a child with a limp?

A
  • Abdomen
  • Axillae/Groin
  • Testicular
  • Hips
  • Spine

Do not want to miss things like appendicitis, leukaemia and testicular torsion

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3
Q

What differentials do you need to consider for different aged children with an acute limp?

A
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4
Q

Apart from MSK issues, what are some other causes of a limp in children?

A
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5
Q

What is juvenile idiopathic arthritis and the different subtypes of this?

A

Autoimmune inflammation in the joints. Diagnosed when there is arthritis without any other cause lasting more than 6 weeks and patient aged under 16

Inflammatory arthritis so joint pain, swelling and stiffness

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6
Q

How does Systemic JIA (Still’s disease) present?

A
  • Subtle salmon-pink rash
  • High swinging fevers
  • Joint inflammation and pain
  • Enlarged lymph nodes
  • Weight loss
  • Splenomegaly
  • Muscle pain
  • Pleuritis and pericarditis
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7
Q

What bloods will be deranged in Still’s disease?

A
  • Raised inflammatory markers: CRP, ESR, Platelets, Ferritin
  • ANA and RhF: negative
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8
Q

What is a key complication of Still’s disease?

A

Macrophage activation syndrome (MAS)

Severe activation of the immune system with a massive inflammatory response

Acutely unwell child with DIC, anaemia, thrombocytopenia, bleeding and a non-blanching rash. It is life threatening. A key investigation finding is a low ESR

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9
Q

What is polyarticular/pauciarticular JIA?

A

Idiopathic inflammatory arthritis in 5 joints or more

Usually symmetrical with minimal systemic symptoms

Polyarticular JIA is the equivalent of rheumatoid arthritis in adults but are seronegative

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10
Q

What is oligoarticular JIA?

A

Involves 4 joints or less, usually only single joint

Tends to affect the larger joints, often the knee or ankle. It occurs more frequently in girls under the age of 6 years

Associated with anterior uveitis. No systemic symptoms

ANA positive but RhF negative

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11
Q

What is Enthesitis-related arthritis in children?

A

Paediatric version of the seronegative spondyloarthropathy group of conditions that affect adults (AS, IBD, etc)

HLA B27 gene so consider symptoms of psoriasis and IBD e.g nail pitting

Tender to local palpation of enethesis

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12
Q

What is juvenile psoriatic arthritis?

A

Seronegative inflammatory arthritis associated with psoriasis

Signs on examination:

  • Plaques of psoriasis
  • Pitting
  • Onycholysis
  • Dactylitis
  • Enthesitis
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13
Q

What are the complications of JIA?

A
  • Growth failure (from steroids and chronic disease)
  • Visual impairment (from chronic anterior uveitis)
  • Joint destruction (requiring early prosthesis)
  • Flexion contractures
  • Joint pain into adulthood
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14
Q

How is JIA managed?

A

Reduce inflammation within the joints, minimise symptoms and maximise function

Refer to Paeds rheumatologist

NSAIDs, Steroids and Immunotherapy

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15
Q

What immunotherapy is used in JIA?

A
  • Steroids: PO, Intrarticular IM
  • Methotrexate: first line
  • Biologics: TNFa-inhibitors e.g adalimumab, etanercept
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16
Q

What are some causes of reactive arthritis in children?

A

Urethritis, Arthritis, Conjunctivitis after recent infection

  • Rheumatic fever: Streptococcal
  • Viral: Gastroenteritis , Rubella, Parvovirus
  • STI: Chlamydia trachomatis
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17
Q

What is Ehlers Danlos Syndrome and the different subtypes?

A

Defects in collagen resulting in abnormal connective tissue in skin, eyes, blood vessels. Leads to hypermobility

Hypermobile EDS: most common and least severe type. They have joint hypermobility, but patients also have soft and stretchy skin

Classical EDS: stretchy skin, severe joint hypermobility, joint pain and abnormal wound healing. Prone to hernias, prolapses, mitral regurgitation and aortic root dilatation. Inheritance is autosomal dominant.

Vascular EDS: most dangerous form of EDS as skin, internal organs and arteries are fragile and prone to rupturing.

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18
Q

How does EDS present?

A

Joint pain and Hypermobility

  • Joint dislocations
  • Soft stretchy skin
  • Easy bruising
  • Poor healing of wounds
  • Bleeding
  • Headaches
  • Autonomic dysfunction causing dizziness and syncope
  • GORD
  • IBS
  • Menorrhagia and dysmenorrhea
  • Premature rupture of membranes in pregnancy
  • Urinary incontinence
  • Pelvic organ prolapse
  • TMJ dysfunction
  • Myopia and other
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19
Q

How is hyper mobility scored?

A

Beighton Score

1 point is scored for each side of the body, with a maximum score of 9:

  • 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
20
Q

How is EDS managed?

A

Diagnosis with Beighton Score but need to rule out Marfan’s

Maintaining health joints, monitoring for complications and minimising symptoms

  • Follow up with relevant specialties e.g POTS
  • Physiotherapy to strengthen and stabilise the joints
  • Occupational therapy to maximise function
  • Maintaining good posture in the joints
  • Moderating the intensity of activity to minimise flares
  • Psychology may be required to a help manage the chronic condition and pain
21
Q

What is Henoch-Schonlein Purpura and the four classical features of this?

A

Childhood IgA vasculitis

Usually triggered by a viral infection, leading to an immune-mediated reaction and IgA deposits in blood vessels

Purpura on buttocks and legs, Joint Pain, Abdominal Pain, Kidney Involvement

22
Q

HSP is commonly triggered after a URTI. How may kidney and GI involvement present?

A
  • GI upset: nausea, vomiting and pain. Usually 8 days following rash
  • Severe GI features: GI bleeding, intussusception, severe pain from perforation
  • Haematuria with mild/absent proteinuria
  • Nephritic syndrome: oliguria, haematuria and hypertension
23
Q

What is the pattern of purpura and arthritis in children with HSP?

A

Purpura: usually buttocks and legs, can turn to ulceration and necrosis

Arthritis: typical knees and ankles

24
Q

What are some differentials for HSP?

A

Other causes of non-blanching rash need to be ruled out:

  • Meningococcal sepsis
  • Leukaemia
  • ITP
25
What investigations are done for HSP diagnosis?
Lots of investigations are done to rule out serious differentials **_Bedside_** * **Urine dipstick**: blood/protein * **Protein/creatinine ratio** (PCR) * **Blood pressure** **_Bloods_** * **FBC**: check haemoglobin and platelet count * **Blood film**: look for other causes of purpura (i.e. malignancy) * **Coagulation** * **If infection concern**: CRP and blood cultures * **Routine**: U&Es, Bone profile, LFTs * **ASOT** * **Autoimmune screen** (if diagnosis in doubt)
26
What is the diagnostic criteria for HSP?
***EULAR/PRINTO/PRES criteria*** Needs palpable purpura (not petichae) and one of: * Diffuse abdominal pain * Arthritis or arthralgia * IgA deposits on histology (biopsy) * Proteinuria or haematuria
27
How is HSP managed?
**_Supportive_** * Simple analgesia * Rest * Hydration **_Close Monitoring whilst active_** * **Urine dipstick** monitoring for renal involvement * **Blood pressure** monitoring for hypertension
28
Are steroids used in HSP treatment?
* May shorten duration of illness but not affect long term outcomes or rate of recurrence * May be considered if severe GI or Kidney involvement
29
Most children with HSP can be managed in the community. What are some criteria for a hospital admission?
* **Hydration**: unable to maintain adequate hydration with oral intake * **Pain**: severe abdominal pain or joint pain limiting mobility/self-care * **GI complications**: bleeding * **Altered mental status** * **Renal disease**: AKI, hypertension, and/or nephritis/nephrotic syndrome
30
What is the prognosis with HSP?
* Usually **self limiting over around 4-6 weeks** if no kidney involvement. Abdo pain will settle in days * **⅓ of patients will have recurrence** within 6 months * **1% will develop end stage renal disease**
31
What is Kawasaki disease and who does this disease affect more?
**Systemic medium sized vessel vasculitis** Affects young children **under 5,** usually **boys** and **asian children** Unknown trigger
32
What is a key complication of Kawasaki disease?
**Coronary Artery Aneurysm** Need to perform echocardiogram ASAP as can be fatal
33
What are the clinical features of Kawasaki disease?
**Persistent high fever** (above 39ºC) for **more than 5 days** Other features include: * ***Widespread erythematous maculopapular rash*** * ***Strawberry tongue*** (red tongue with large papillae) * ***Cracked lips*** * ***Cervical lymphadenopathy*** * ***Bilateral conjunctivitis*** * ***Desquamation on hands and feet***
34
What is the diagnostic criteria for Kawasaki disease?
**Fever for \>5 days, and 4/5 of the 'CREAM' features:** * Bilateral non-purulent conjunctivitis * Rash * Edema/Erythema of hands and feet * Lymphadenopathy (cervical, commonly unilateral) * Mucosal involvement (strawberry tongue, oral fissures etc)
35
What investigations might be done once a diagnosis of Kawasaki's disease is considered and what will they show?
* **FBC:** anaemia, leukocytosis and thrombocytosis * **LFTs:** hypoalbuminemia and elevated liver enzymes * **Inflammatory markers** (particularly ESR): raised * **Urinalysis:** raised white blood cells without infection * **Echocardiogram:** coronary artery pathology ECHOCARDIOGRAM MOST IMPORTANT TO LOOK FOR COMPLICATION OF CORONARY ARTERY ANEURYSM
36
What are the three phases of Kawasaki disease?
* **Acute:** child is most unwell with fever, rash and lymphadenopathy. Lasts 1-2 weeks * **Subacute phase**: Acute symptoms settle, desquamation and arthralgia occur and risk of coronary artery aneurysms forming. Lasts 2 – 4 weeks * **Convalescent stage**: The remaining symptoms settle, the blood tests slowly return to normal and the coronary aneurysms may regress. Last 2 – 4 weeks
37
How is Kawasaki disease managed?
* **High dose aspirin** to reduce the risk of **thrombosis** (note: risk of Reye’s syndrome) * **IV immunoglobulins** to reduce the risk of **coronary artery aneurysms** * Close follow up with echocardiograms to monitor for coronary artery aneurysms
38
Kawasaki disease is one of the only times aspirin can be used in children. Why can aspirin not be used otherwise?
**Reye's Syndrome** **Severe, progressive encephalopathy** with **fatty infiltration of the liver, kidneys and pancreas** needing ICU input. Due to treating viral infections with aspirin in children
39
What is Rheumatic Fever and the pathophysiology of this?
***Autoimmune condition*** triggered by Strep pyogenes infection, presenting 2-4 weeks after infection ***Multi-system disorder*** that affects the joints, heart, skin and nervous system. It is rare in the UK due to early treatment of streptococcus with antibiotics.
40
How may rheumatic fever present?
2 – 4 weeks following a streptococcal infection, such as ***tonsillitis:*** * Fever * Joint pain * Rash * Shortness of breath * Chorea * Nodules Joints, heart, skin, nervous system
41
What are some heart and skin manifestations of rheumatic fever?
**Heart:** * Pericarditis * Endocarditis * Murmurs from mitral valve disease * Pericardial rub on auscultation * Heart failure **Skin involvement:** * Subcutaneous nodules * Erythema marginatum rash
42
What investigations are done if rheumatic fever is suspected?
* **Throat swab** for bacterial culture * **ASO antibody titres** repeated after 2 weeks * **Echocardiogram**, **ECG** and **chest xray** can assess the heart involvement
43
What is the criteria for diagnosis of rheumatic fever?
**Jone's Criteria (JONES – FEAR)** * Two major criteria ***OR*** * One major criteria plus two minor criteria **_Major Criteria:_** * **J – J**oint arthritis * **O – O**rgan inflammation, such as carditis * **N – N**odules * **E – E**rythema marginatum rash * **S – S**ydenham chorea **_Minor Criteria:_** * **F**ever * **E**CG Changes (prolonged PR interval) without carditis * **A**rthralgia without arthritis * **R**aised inflammatory markers (CRP and ESR)
44
How is rheumatic fever managed?
* **Prevention:** treat tonsillitis caused by S.Pyogenes phenoxymethylpenicillin (penicillin V) for 10 days * **NSAIDs:** joint pain, avoid aspirin in under 16 * **Aspirin** and **steroids** are used to treat carditis * **Prophylactic antibiotics** (oral or intramuscular penicillin) * **Monitoring and management of complications**
45
What are the complications of rheumatic fever?
- Sydenham chorea - Endocarditis - Heart failure - Arrhythmias

Y4 Paediatrics (32 decks)

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