20 - Rheumatology Flashcards
If a child has a fever for more than 5 days, what key non-infectious differentials do you need to consider?
- Still’s Disease
- Kawasaki Disease
- Leukaemia
- Rheumatic fever
What examinations do you need to do for a child with a limp?
- Abdomen
- Axillae/Groin
- Testicular
- Hips
- Spine
Do not want to miss things like appendicitis, leukaemia and testicular torsion
What differentials do you need to consider for different aged children with an acute limp?
Apart from MSK issues, what are some other causes of a limp in children?
What is juvenile idiopathic arthritis and the different subtypes of this?
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
How does Systemic JIA (Still’s disease) present?
- Subtle salmon-pink rash
- High swinging fevers
- Joint inflammation and pain
- Enlarged lymph nodes
- Weight loss
- Splenomegaly
- Muscle pain
- Pleuritis and pericarditis
What bloods will be deranged in Still’s disease?
- Raised inflammatory markers: CRP, ESR, Platelets, Ferritin
- ANA and RhF: negative
What is a key complication of Still’s disease?
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
What is polyarticular/pauciarticular JIA?
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
What is oligoarticular JIA?
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
What is Enthesitis-related arthritis in children?
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
What is juvenile psoriatic arthritis?
Seronegative inflammatory arthritis associated with psoriasis
Signs on examination:
- Plaques of psoriasis
- Pitting
- Onycholysis
- Dactylitis
- Enthesitis
What are the complications of JIA?
- Growth failure (from steroids and chronic disease)
- Visual impairment (from chronic anterior uveitis)
- Joint destruction (requiring early prosthesis)
- Flexion contractures
- Joint pain into adulthood
How is JIA managed?
Reduce inflammation within the joints, minimise symptoms and maximise function
Refer to Paeds rheumatologist
NSAIDs, Steroids and Immunotherapy
What immunotherapy is used in JIA?
- Steroids: PO, Intrarticular IM
- Methotrexate: first line
- Biologics: TNFa-inhibitors e.g adalimumab, etanercept
What are some causes of reactive arthritis in children?
Urethritis, Arthritis, Conjunctivitis after recent infection
- Rheumatic fever: Streptococcal
- Viral: Gastroenteritis , Rubella, Parvovirus
- STI: Chlamydia trachomatis
What is Ehlers Danlos Syndrome and the different subtypes?
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.
How does EDS present?
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
How is hyper mobility scored?
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
How is EDS managed?
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
What is Henoch-Schonlein Purpura and the four classical features of this?
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
HSP is commonly triggered after a URTI. How may kidney and GI involvement present?
- 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
What is the pattern of purpura and arthritis in children with HSP?
Purpura: usually buttocks and legs, can turn to ulceration and necrosis
Arthritis: typical knees and ankles
What are some differentials for HSP?
Other causes of non-blanching rash need to be ruled out:
- Meningococcal sepsis
- Leukaemia
- ITP
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)
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
How is HSP managed?
Supportive
- Simple analgesia
- Rest
- Hydration
Close Monitoring whilst active
- Urine dipstick monitoring for renal involvement
- Blood pressure monitoring for hypertension
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
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
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
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
What is a key complication of Kawasaki disease?
Coronary Artery Aneurysm
Need to perform echocardiogram ASAP as can be fatal
inflammation can weaken the vessel walls of the coronary arteries leading to an aneurysm
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
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)
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
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
How is Kawasaki disease managed?
- High dose aspirin to reduce the risk of thrombosis
- IV immunoglobulins to reduce the risk of coronary artery aneurysms
- Close follow up with echocardiograms to monitor for coronary artery aneurysms
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
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.
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
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
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
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 – Joint arthritis
- O – Organ inflammation, such as carditis
- N – Nodules
- E – Erythema marginatum rash
- S – Sydenham chorea
Minor Criteria:
- Fever
- ECG Changes (prolonged PR interval) without carditis
- Arthralgia without arthritis
- Raised inflammatory markers (CRP and ESR)
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
What are the complications of rheumatic fever?
