Rheumatology Flashcards
3 key features of inflammatory arthritis
Joint pain, swelling and stiffness
Key feature of systemic juvenile idiopathic arthritis
Salmon pink rash
Systemic juvenile idiopathic arthritis common name
Stills disease
Typical features of systemic juvenile idiopathic arthritis
Salmon pink rash
Fevers, lymph nodes, weight loss, join pain and inflammation, splenomegaly, muscle pain, pleuritis
Blood tests for systemic juvenile idiopathic arthritis
AMA and RF normally negative
Raised inflammatory markers - CRP, ESR, platelets, ferritin
ESR low if macrophage activation syndrome
Key complication of systemic juvenile idiopathic arthritis
Macrophage activation syndrome
Overactivation of immune system -> DIC -> non blanching rash/ anaemia, thrombocytopenia
LOW ESR
Key differentials for children with fevers for more than 5 days (non infective)? (4)
Kawasaki disease, stills disease, rheumatic fever, leukaemia
5 types of juvenile idiopathic arthritis
Systemic, polyarticular, oligoarticular, enthesitis related, juvenile psoriatic
How many joints does polyarticular and oligoarticular juvenile idiopathic arthritis effect
Polyarticular 5 or more
Oligoarticular 4 or less
Signs and symptoms in polyarticular juvenile idopathic arthritis
5 or more joints with symmetrical arthritis.
Mild fever, anaemia, reduced growth
RA in children name
Polyarticular juvenile idiopathic arthritis
Condition associated with oligoarticular juvenile idiopathic arthritis
Anterior uveitis
Oligoarticular JIA presentation
Commonly only effects 1 joint
Girls under 6 years
Anterior uveitis association
Enthesis definition
Point at which tendon inserts onto bone
What is the name of the group of conditions: ankylosing spondylitis, psoriatic arthritis, reactive arthritis, IBD.
Seronegative spondyloarthropathy
Gene mutation in majority of enthesitis related juvenile idiopathic arthritis
HLA B27
How will patients with enthesitis related arthritis present
Tender palpation of entheses
Psoriasis, IBD, anterior uveitis
Seronegative meaning
RF test negative
4 types of management of juvenile idiopathic arthritis
NSAIDS
Steroids
DMARDS
Biologicals (tumour necrosis factor inhibitors)
Ehlers-danlos syndrome cause
Genetic defect in collagen
4 types of EDS
Hypermobile (most common) - soft stretchy skin
Classical - remarkably stretchy skin, very smooth. Hernias, prolapses, MR, aortic root dilatation
Vascular - thin translucent skin. Vessels prone to rupture
Kyphoscoliotic - hypotonia and kyphoscoliosis
Inheritance type of EDL
Autosomal dominant
Hypermobile (most common) is genetic but unknown
Scoring system for hyper mobility
Beighton score
Max of 9
Hypermobile EDS symptoms other than joint pain and hypermobility
Soft skin, easy brushing, GORD, IBS, PROM, incontinence, bleeding, autonomic dysfunction
POTS
Other common genetic condition that causes hypermobility
Marfan syndrome - high arch palate, arachnodactyly and long arm span
Management of EDS
No cure
Physio, good posture, moderate exercise
Rheumatology condition associated with POTS
EDS
Henoch Schonlein Purpura cause
IgA vasculitis
Inflammation of skin, kidneys, and GIT from IgA deposits in blood vessels
Henoch Schonlein Purpura 4 features
Purpura
Joint pain
Abdominal pain
Renal involvement
Child presents with purpura rash, joint pain, abdominal pain and deranged U&Es - DX?
Henoch Schonelin Purpura
5 differentials of non blanching rash
Menigococcal sepsis Henoch Schonlein Pupura Idiopathic thrombocytopenic purpura Haemolytic uraemia syndrome (E Coli 0157) Leukaemia
Important test to do in children with hence schonlein purpura
Dipstick urine - 50% have renal involvement (IgA nephritis)
2+ protein -> nephrotic syndrome
Criteria for hence schonlein purpura diagnosis
EULAR/PRINTO/PRES criteria
Pupura and one of:
Abdominal pain, arthritis, IgA deposits, proteinuria
Kawasaki type of disease
Mucocutaneous lymph node syndrome
Vasculitis
Kawasaki epidemiology
Boys under 5
Asian, Japanese and Korean children
Kawasaki disease key complication
Coronary artery aneurysm
Key features of Kawasaki disease
High fever for more than 5 days
Widespread maculopapular rash and desquamation (skin peeling)
Strawberry tongue, cracked lips, cervical lymphadenopathy, bilateral conjunctivitis
Key features of Kawasaki disease
High fever for more than 5 days
Widespread maculopapular rash and desquamation (skin peeling)
Strawberry tongue, cracked lips, cervical lymphadenopathy, bilateral conjunctivitis
What is desquamation and what Peads disease is it associated with
Peeling skin
Kawasakis disease
Investigations in Kawasaki disease
FBC - anaemia, leukocytosis and thrombocytosis
LFT - hypoalbuminemia and elevated LFTs
Inflammatory markers (ESR) - raised
Urinalysis - white cells without infection
Echo - check for coronary artery pathology
3 phases to Kawasaki disease
Acute - 1 to 2 weeks child most unwell
Subacute - 2 to 4 weeks desquamation and arthralgia. Risk of coronary artery pathology
Convalescent - 2 to 4 weeks symptoms settle
Management of Kawasakis disease
High dose aspirin - reduce risk of thrombosis
IV immunoglobulins - reduce risk of coronary artery aneurysms
Why is aspirin not normally given to children
Risk of Reyes syndrome
Rheumatic fever type of disease
Autoimmune condition triggered by streptococcus bacteria.
Antibodies against strep also target body tissues
Why is rheumatic fever uncommon in UK
Early treatment of streptococcus (group A beta-haemolytic - commonly pyogenes) that causes tonsillitis with antibiotics.
Most common bacteria to cause RF
Group A beta-haemolytic streptococcus
Streptococcus pyogenes
Type of reaction in RF
Type 2 hypersensitivity
Timing between streptococcus infection and RF
2- 4 weeks
Most common infection 2-4 weeks before RF
Tonsilitis (strep pyogenes)
5 groups of symptoms in RF
Joint involvement - migratory arthritis (hot swollen large joints that move from one joint to another)
Heart involvement - carditis (pericarditis, myocarditis, endocarditis), mitral valve disease, pericardial rub
Skin involvement - subcutaneous nodules, erythema marginatum rash
Firm painless nodules on extensor surfaces
Nervous system - chorea (irregular, uncontrolled rapid movements on limbs)
Rash in RF
Erythema marginatum
Type of nervous system symptom in RF
Chorea - rapid, uncontrolled random movements on limbs
Type of joint problems in RF
Migratory arthritis - swelling in large joints moves from one to another
Are subcutaneous nodules painful in RF
NO
3 key investigations for RF
Throat swap for group A streptococcus
ASO antibody
ECG/ echo/ CXR for carditis
Criteria to diagnose RF
Jones criteria
Treatment of tonsillitis caused by streptococcus bacteria
Phenoxymethylpenicillin for 10 days
Management of RF
NSAIDS - arthritis
Aspirin and steroids - carditis
Prophylactic abx - prevent further strep infections
Why do RF patients need abx prophylaxis
Further exposure -> ab production -> reoccurrence of RF
Complication of RF
Reoccurrence
Mitral stenosis
Chronic heart failure