Rheumatology Flashcards
Name the cardinal features of Henoch-Schonlein Purpura.
- Palpable purpura on the extensor surfaces of the bilateral lower limbs
- Abdominal pain
- Arthralgia with periarticular edema
- Hematuria
- Fever
>> Usually have a history of URTI 1-3 weeks before onset
What is the diagnostic criteria of Kawasaki Disease?
Fever of >38.5 degrees for at least 5 days AND any 4 of the following 5:
- Conjunctivitis (Bilateral nonpurulent conjunctivitis)
- Rash (polymorphic)
- Adenopathy (cervical lymphadenopathy >1.5cm)
- Strawberry tongue (and other mucosal changes like cracked lips)
- Hand skin-peeling (as well as perianal skin peeling)
>> CRASH and burn
What are the causes of limb pain in children?
BY AGE
<3 years of age
- Infections
>> Osteomyelitis
>> Septic arthritis
- Trauma
- Inflammatory causes
>> Transient synovitis
>> Juvenile idiopathic arthritis
- Neoplastic
>>.Leukemia
>> Neuroblastoma
- Hematological
>> Hemophilia
>> Sickle-cell disease
3-10 years
- Infection
- Trauma
- Inflammatory
>> Transient synovitis
>> Juvenile idiopathic arthritis
>> Systemic lupus erythematosus
>> Henoch Schonlein purpura
>> Spondyloarthritis
>> Dermatomyositis
- Neoplastic
>> Bone tumours: osteosarcoma, Ewing sarcoma
>> Leukemia
>> Neuroblastoma
- Hematological
>> Hemophilia
>> Sickle cell disease
- Orthopedic
>> Perthes disease
>> Growing pains
>10 years
- Infection
- Trauma
- Inflammatory
>> Juvenile idiopathic arthritis
>> Systemic lupus erythematosus
>> Henoch-Schonlein purpura
>> Spondyloarthritis
>> Dermatomyositis
- Neoplastic
>> Bone tumours
>> Leukemia
- Hematological
>> Hemophilia
>> Sickle-cell disease
- Orthopedic
>> Perthes disease
>> Slipped capital femoral epiphysis
>> Osgoode-Schlatter
- Psychosocial: pain syndromes
What do we need to rule out in a child presenting with limb pain?
- Infection
- Acute orthopedic conditions: e.g. fractures
- Malignancy
What investigations are useful for working up a child with limb pain/limp?
Blood
- CBC with differential count
- Blood smear: sickle cell
- ESR/CRP: for inflammation
- Clotting profile: for hematological causes
- Blood culture
- CPK: for muscular causes
- Autoimmune screening
>> ANA
>> RF
>> Immunoglobulins
>> C3/C4 levels
Synovial fluid
- Cell count
- Gram stain and culture
- Protein (>4.4mg/dL)
- Glucose (Less than blood glucose in septic arthritis)
Urinalysis
Others
- Radiograph of the limb for orthopedic disorders
- Ultrasound of the joint: especially for effusion
- TB skin test if TB arthritis is suspected
- BM aspiration if leukemia is suspected
- Slit-lamp examination of the eye if JIA suspected: for any anterior uveitis
>> Especially in joint pain: listen for heart murmurs to rule out rheumatic fever and infectious endocarditis – if the arthritis does not improve within 48 hours of therapeutic aspirin dose, it is probably not rheumatic fever.
What are the presenting features of transient synovitis of the hip?
Age: 2- 10 years; M>F
- Afebrile pain over hip and knee (referred from hip)
- Painful limp
- ROM full although painful
>> Slight limitation at internal rotation and adduction
>> No pain at rest - Preceded by an upper respiratory tract infection
- Benign and self-limiting within 7-10 days
What is the management for transient synovitis of the hip?
- Symptomatic treatment
- NSAIDs
What are the most common organisms of septic arthritis in children?
- *Neonates**
- GBS
- E. Coli
- Staphylococcus aureus
- *Infants (1-3 months)**
- Streptococci
- Staphylococci
- Hemophilus influenzae
- *Children**
- Staphylococcus aureus
- Streptococcus pneumoniae
- Group A streptococci + Neisseria gonorrhea in adolescents
What is the criteria for JIA diagnosis?
- Arthritis of more than 1 joint
- Duration of at least 6 weeks
- Onset less than 16 years of age
>> Classification defined by features/number of joints affected
in the FIRST 6 MONTHS OF ONSET
>> After exclusion of other causes of arthritis
What are the types of JIA (juvenile idiopathic arthritis)?
Oligoarticular arthritis
- Defined as arthritis of, at most, 4 joints (1-4 joints)
- Onset: 1-6 years
- Typically affects large joints
- ANA POSITIVE 60-80%
- Commonly associated with anterior uveitis
- Two types: persistent and extended
- PERSISTENT
>> No more than 4 joints after first six months
- EXTENDED
>> More than 4 joints after first six months
>> Often asymmetrical
Polyarticular arthritis
- Defined as arthritis of 5 or more joints
- Two types: RF positive and RF negative
- RF negative
>> Age of onset: 1-6 years; F>M
>> Symmetrical involvement
- RF positive
>> Age of onset: 11-16 years; F>M
>> More severe than RF negative – similar to adult RA
>> Symmetrical involvement
>> Rheumatoid nodules at pressure points
>> Unremitting – persists in adulthood
- *Enthesitis-Related Arthritis - HLA-B27 positive**
- Age of onset: 6-16 years; M>F
- Risk of developing ankylosing sponylitis in childhood
Psoriatic arthritis
- Age of onset: 1-16 years; F>M
- Either arthritis + psoriasis; or at least two of:
>> Dactylitis
>> Nail abnormalities
>> Family history of psoriasis
>> Asymmetric/symmetric large/small joint involvement
Systemic JIA/Still’s disease
- Age of onset: any age; M=F
- STILLS
S: Spiking fever – once or twice daily >38.5C >= 2 weeks
T: JoinT (arthralgia)
I: Increased size of liver/spleen
L: Lose weight (anorexia)
L: Looks like malignancy
S: Salmon-pink rash
- Lymphadenopathy
- Serositis
- Paradoxical CBC: high WBC, high PLT, low Hb - excessive activation of MAC
- Other biochemical findings: high ESR and CRP
What is the difference between oligoarticular and polyarticular JIA by definition?
Oligoarticular: <=4 joints involved
Polyarticular: 5 or more joints involved
What should one ask for in the history to rule out/rule in JIA?
- Gelling: stiffness after prolonged rest
- Morning stiffness
- Pain over the joints
- Intermittent limp
- Deterioration in behaviour/mood disturbances
- Avoidance of previously enjoyed activities
What is the management of JIA?
Dependent on the type of JIA
Mainly articular
- NSAIDs
- Intra-articular corticosteroids
- DMARDs: methotrexate, sulfasalazine, lefunamide
- Biologics
>> Anti-TNFa: etanercept, adalimumab
>> Anti-CD20: rituximab
If systemic: oral/systemic corticosteroids instead
What are the common complications of JIA to look out for?
Joints
- Knee flexion contracture
- Leg-length discrepancy
- Osteoporosis
Systemic
- Growth disturbances
- Anemia of chronic illness
- Amyloidosis >> proteinuria/renal failure
What is the diagnostic criteria for SLE (systemic lupus erythematous)?
SCLICC classification criteria
Biopsy-proven lupus nephritis with ANA or anti-dsDNA
OR
4 of the listed criteria (at least 1 clinical and 1 immunological)
ACR revised criteria for SLE classification
Any 4 of the following criteria (MD SOAP BRAIN)”
M: Malar rash
D: Discoid rash
S: Serositis
O: Oral ulcers
A: Arthritis (>= 2 peripheral joints)
P: Photosensitivity
B: Blood
- Hemolytic anemia
- Leukocytopenia
- Thrombocytopenia
R: Renal
- Proteinuria
- Cellular casts: RBC, granular, tubular
A: ANA positive
I: (Other) Immunological markers
- Anti-dsDNA
- Anti-smooth mucsle
- Anti-phospholipid
- Low C3/C4 levels
N: Neurological
- Seizures
- Psychosis
- Mononeuritis multiplex
- Myelitis
- Peripheral/cranial neuropathy
- Cerebritis
What is the difference between childhood-onset and adult-onset SLE?
- More active disease
- More renal diseases
- More intensive drug therapy>> more damage
What are some possible SLE treatment?
- *Dermatological**
- Avoid UV lights
- Topical steroids
- Topical hydroxychloroquine
- *Musculoskeletal**
- NSAIDs
- Hydroxychloroquine
- Bisphosphonates, calcium & vit D to prevent osteoporosis, esp. in steroid usage
Organ-threatening disease
- High-dose oral prednisolone/IV methylprednisolone
- Steroid sparing immunosuppressants
>> Azathioprine
>> Methotrexate
>> Mycophenolate
>> Mofetil
>> IVIG
- IV cyclophosphamide for cerebritis and nephritis
- Biologics
>> Rituximab
>> Belimumab
What is the most common vasculitis in childhood?
Henoch-Schonlein Purpura
What is the common age of onset of HSP?
4-10 years; M>F
What is the management of HSP?
- Mainly supportive
>> NSAIDs for joint pain
>> Corticosteroids - Monitor urine protein every 6 weeks for 4-6 months to check for any renal disease needing immunosuppressive therapy (may present late)
Usually self-limiting within 4 weeks
Recurrence in about one third of patients
What is atypical Kawasaki disease?
Less than 5 of the 6 diagnostic features but has coronary artery involvement
What is the peak age of onset for Kawasaki disease?
3 months - 5 years
What is the management for Kawasaki disease?
- High-dose aspirin while febrile
- Lose-dose aspirin in subacute phase until platelets normalize (anti-platelet function
- IVIG within 10 days of onset (2g/kg over 8-12 hours)
>> Baseline ECHO and follow-up ECHO at 6 weeks
>> 50% of coronary aneurysms regress within 2 years
What are the risk factors for coronary disease in Kawasaki disease?
- Male
- Age: <1 year or >9 years
- Prolonged fever >10 days
- Recurrence of fever after 48 hours of being afebrile
- Cardiomegaly
- Thrombocytopenia
- Hyponatremia
What are the possible laboratory findings in Kawasaki disease?
- *CBC**
- Increased WBC
- Increased PLT
- Normocytic normochromic anemia
- *CRP**
- Elevated for >80% from normal levels LRFT
- Hyponatremia
- Hypoalbuminemia
- Increased ALT
- *Urine
- Pyuria without bacteruria**
- *Lumbar puncture/CSF**
- Pleocytosis
- Normal protein
- Normal glucose
Name the drugs that cause drug-induced SLE.
SHIPP
S: Sulfonamides
H: Hydralazine
I: Isoniazid
P: Procainamide
P: Phenytoin
