Rheumatology Flashcards
- What is Kawasaki’s Disease? What is the trigger?
- What groups of patients is Kawasaki’s Disease prevalent in?
- What is the presentation of Kawasaki’s Disease?
- What are the investigations / diagnosis of Kawasaki’s Disease?
- What is the management of Kawasaki’s Disease and why?
- What are the complications of Kawasaki’s Disease?
- A medium vessel vasculitis with an unknown trigger
- Common in young boys age < 5, of Asian (Korean / Japanese descent)
- Persistently high fever (>39C) greater than 5 days, resistant to antipyretics, Bilateral conjunctivitis, Widespread erythematous maculopapular rash and desquamation (skin peeling) on the palms and soles, Strawberry tongue, Bright red, cracked lips, Cervical lymphadenopathy
- Full blood count: anaemia, leukocytosis and thrombocytosis
- Liver function tests: hypoalbuminemia and elevated liver enzymes
- Inflammatory markers:(particularly ESR) are raised
- Urinalysis: Sterile pyuria
- Echocardiogram: coronary artery pathology
- High dose aspirin to reduce risk of DVTs, IV IG to reduce risk of coronary artery aneurysm, Echo to screen for coronary artery aneurysm
- Coronary artery aneurysm
In simple terms, what is Juvenile Idiopathic Arthritis?
An autoimmune condition affecting the joints, seen in patients under 16 years old, lasting more than 6 weeks
What are the five subtypes of Juvenile Idiopathic Arthritis? JOPES
- Juvenile psoriatic arthritis
- Oligoarticular JIA aka Pauciarticular JIA
- Polyarticular JIA
- Enthesitis related arthritis
- Systemic JIA
What is Systemic Juvenile Idiopathic Arthritis also known as?
- Still’s disease
What is Still’s disease also known as?
Systemic Juvenile Idiopathic Arthritis
What are the features of Systemic Juvenile Idiopathic Arthritis?
- Subtlesalmon-pink rash
- High swinging fevers
- Enlarged lymph nodes
- Weight loss
- Joint inflammation and pain
- Splenomegaly
- Muscle pain
- Pleuritis and pericarditis
What are the values of RF and ANA in Systemic Juvenile Idiopathic Arthritis?
Negative
Polyarticular Juvenile Idiopathic Arthritis
- Involves five joints or more
- Can affect large and small joints
- Joints affected tend to be symmetrical
- Milder systemic symptoms than Systemic Juvenile Idiopathic Arthritis
- Tend to be seronegative (RF negative)
Oligoarticular Juvenile Idiopathic Arthritis
- Involves four joints or less
- Usually only affects one joint → monoarthritis
- Tends to affect larger joints i.e. knee, ankle
- Tends to occur in girls under the age of six
- ASSOCIATED FEATURE = ANTERIOR UVEITIS
- Generally no systemic symptoms, ANA positive, RF negative
Enthesitis-Related Arthritis
- More common in male children over 6 years old
- Paediatric version of seronegative spondyloarthropathies
- Majority of patients have the HLA-B27 gene
- Enthesitis → Inflammation of enthesis (point at which tendon of muscle inserts onto bone)
- Can be caused by traumatic stress i.e. in sports
- Can be auto-immune mediated
- Tend to have associated conditions i.e. Psoriasis, IBD
- ASSOCIATED FEATURE = ANTERIOR UVEITIS
Juvenile Psoriatic Arthritis
- Seronegative inflammatory arthritis related to psoriasis
- Can be symmetrical or asymmetrical
- Nail signs → Plaque psoriasis, nail pitting, onycholysis, dactylitis, enthesitis
How is Juvenile Idiopathic Arthritis managed?
- NSAIDs, such as ibuprofen
- Steroids, eitheroral,intramuscularorintra-artricularinoligoarthritis
- Disease modifying anti-rheumatic drugs(DMARDS), such as methotrexate, sulfasalazine and leflunomide
- Biologic therapy, such as thetumour necrosis factor inhibitorsetanercept, infliximab and adalimumab
What is Ehler-Danlos Syndrome?
- Umbrella term that encompasses a group of genetic conditions that cause defects in collagen III (primarily), resulting in hypermobility of the patient’s joints and abnormalities in connective tissue such as the skin, bones, blood vessels and organs
What is the inheritance pattern of Ehler-Danlos Syndrome?
Autosomal dominant
What are the two primary symptoms of Ehler-Danlos Syndrome?
What are other symptoms of Ehler-Danlos syndrome?
Hypermobility, and joint pain
Other symptoms: Joint dislocations, for example the shoulders or hips, aortic regurgitation, mitral valve prolapse and aortic dissection, subarachnoid haemorrhage, soft stretchy skin, easy bruising, poor healing of wounds, autonomic dysfunction causing dizziness and syncope, gastro-oesophageal reflux, menorrhagia and dysmenorrhea, premature rupture of membranes in pregnancy
Outline the Beighton Score of Hypermobility?
- Ability to touch palms flat to floor with knees straight (one point)
- Elbow extension >10° (one point for each side)
- Knee extension >10° (one point for each side)
- Ability to touch thumb to forearm (one point for each side)
- Fifth finger MCP joint extension >90°
How is Ehler-Danlos diagnosed?
- For adults, must be 5 out of 9 points → positive
- For children, must be 6 out of 9 points → positive
How is Ehler-Danlos managed?
- Follow up with relevant specialties depending on the associated complications
- Physiology to strengthen and stabilise joints
- Occupational therapyto maximise function
- Maintaining good posture in the joints
- Moderating the intensity of activityto minimise flares
- Psychologymay be required to a help manage the chronic condition and pain
What is Postural Orthostatic Tachycardia Syndrome (POTS)?
- POTS is associated with EDS
- Autonomic dysfunction → tachycardia on sitting / standing up → presyncope / syncope / headaches / nausea / tremor
What is Henoch-Schonlein Pupura?
What is it also known as?
HSP is an igA vasculitis, which causes a purpuric rash on the lower limbs and buttocks
Also known as Berger’s Disease
What are the features of Henoch-Schonlein Purpura?
When does it usually present?
- Purpura, joint pain, abdominal pain, renal involvement (nephrotic syndrome)
- Typically 1-2 days after a viral infection
What are some differentials of Henoch-Schonlein Purpura?
- Must exclude: Meningococcal sepsis, Leukaemia, ITP and HUS
What is the management and prognosis of Henoch-Schonlein Purpura?
- Mainly supportive: analgesia, fluids, rest. Poor evidence of steroids and immunosuppressants
- Prognosis: Usually good, will get better after 4-6 weeks if no renal involvement. 1/3rd will relapse by 6 months
