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
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
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 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)
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
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
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?
- Sydenham chorea
- Endocarditis
- Heart failure
- Arrhythmias
