Rheumatology Flashcards
What effect does allopurinol have on azathioprine?
Enhances the effects of azathioprine and hence the dose of azathioprine should be reduced by 75%
In a pt with SLE who is ANA negative, what antibody are the usually + for?
Anti Ro
What is the first treatment for oligoarticular JIA
1st NSAIDs + intra articular steroids
In osteogenesis imperfecta alk phos levels are usually elevated T/F
F - usually blood tests in OI show no particular abnormality
Osteogenesis imperfecta can be associated with hearing impairment T/F
T
Other extra skeletal features: blue/grey sclera, discoloured teeth that break easily (dentinogenesis imprefecta), ligamental laxity, increased skin laxity
Bisphosphonate infusions are used in the treatment of osteogenesis imperfecta T/F
T
Myasthenia gravis is associated with what antibodies?
Antibodies against acetylcholine receptors
Criteria of systemic onset juvenile idiopathic arthritis excluding the characteristic rash (SOJIA)
At least 2 weeks of fever, followed by arthritis in one or more joints PLUS generalised lymphadenopathy or hepato or splenomegaly or serositis
5 features of Kawasaki disease in addition to fever
- Lymphadenopathy in neck at least 15mm
- Lip or oral cavity erythema or cracked lips
- Conjunctival injection
- Rash on trunk
- Swelling/erythema of hands or feet
Need 4 of these + 5 days of fever for kawasaki diagnosis
Criteria for the diagnosis of juvenile dermatomyositis?
Pathognomonic rash + 3 of
- Proximal muscle weakness
- Elevated serum levels of muscle enzymes
- Typical electromyographic changes
- Histopath changes on muscle biopsy
Note: if option between biopsy and electromyography do electromyography first as it is less invasive
In juvenile dermatomyositis calcinosis occurs in the majority of children T/F
F - in 40%
Note: JDM is not associated with malignancy
How many joints need to be involved to diagnosis polyarticular JIA?
5 or more joints involved in the first 6 months.
JIA is assoc with what eye pathology?
Chronic anterior uveitis
Note: often clinically silent
Behcet’s disease is assoc with what eye pathology?
Anterior, posterior or pan uveitis
Note: often severe recurrent attacks, usually presents about 3-4 yrs after oral and genital ulcers
What is Still’s disease AKA?
Systemic onset juvenile idiopathic arthritis
What would be expected for coags and plt count in HSP?
Normal
In a pt with active SLE serum complement levels are invariably reduced T/F
T - this is due to the formation of immune complexes
What is the most common sub type of JIA?
Oligoarticular
Note: 50-60% of ALL cases of chronic arthritis of childhood
Polyarteritis nodosa is assoc with what antibody and other disease?
ANCA (positive in 50% of cases)
Hepatitis B
Note: medium vessel vasculitis
Which ANCA is positive in granulomatosis with polyangitis and microscopic polyangitis?
Granulomatosis = cANCA
Micro polyangitis = pANCA
Note: granulomatosis with polyangitis affects nasal/sinuses but micropolyangitis does not. Both affect lung and kidneys. Micro polyangitis may also have non specific abdominal and joint pain
Neonatal lupus may be present at birth but can also develop months later T/F
T
Is the facial rash in neonatal lupus a malar distribution?
No, it is not the typical malar distribution that is seen in older children/adults with lupus
Note: rash may be precipitated by phototherapy for hyperbili
In a patient with polyarticular JIA what is the next medication after the have failed NSAIDS?
Methotrexate
Children with ank spond may experience inflammatory back pain. Common clinical features of this type of pain?
Pain at night with morning stiffness and improvement throughout the day with exercise (No improvement with rest)
Insidious onset
Good response to NSAIDs
How does ank spond typically present in children?
Enthesis and peripheral arthritis of lower limbs
Axial disease and inflammatory back pain are less common at disease ONSET than in the adult pop
What cardiac abnormality is often present in ank spond?
Aortic regurgitation
In patients with SLE who have a non photosensitive rash what antibody is most specific and what antibody tends to be negative
Anti Ro is most specific
ANA tends to be negative
What is the difference in eye disease in those with JIA vs enthesitis related arthritis?
JIA - 70% of patients will have bilateral
Enthesitis - more likely unilateral
HSP is associated with IgA deposits in the mesangium T/F
T
Note: HSP is a small vessel vasculitis
Enteropathic arthritis is associated with positive IgM rheumatoid factor T/F
F - it is a seronegative arthritis
Note: patients with IBD have increased risk of arthritis when disease is active
What is the remission rate of polyarticular JIA
About 30%
The low dose OCP is CI in those with SLE
False - if they have low disease activity and a documented negative anti phospholipid ab
Note: avoid OCP with high dose oestrogen
In a pt with SLE neutropenia is the most common haem abnormality T/F
F- a mild leukopenia is the most common abnormality usually caused by lymphopenia, neutropenia is rare
In a pt with SLE lymphopenia is the most common haem abnormality T/F
T
What is the most common form of JIA?
Persistent oligoarthritis
Usually girls, around 3 yrs old
Rheumatoid factor and ANA are usually positive in systemic JIA T/F
F - they are both usually negative
Is systemic JIA more common in girls or boys?
Equal
Note: compare to polyarticular or oligoarticular which are more common in females
The rash of systemic JIA often reappears with stroking or minor trauma T/F
T
Note: this is known as Koebner phenomenon
Side effects of cyclophosphamide?
Myelosuppression
Infertility
Alopecia
HLA B27 is often positive in which arthritis
Psoriatic arthritis
Enthesitis-related arthropathy
Features of hyper-IgD
Fever, headache, nausea, vomiting, oral and vaginal ulcers.
Large joint arthritis, diffuse non-migratory erythematous rash, splenomegaly
Usually presents by one year of age
Cryopyrin-assoc periodic syndromes are assoc with what gene mutation?
NLRP3
Note: 3 types - familial cold auto inflammatory syndrome, Muckle-Wells syndrome and neonatal onset multi system inflammatory disease
Autosomal recessive mutation in MEFV, what pathology?
Familial Mediterranean fever
Treatment of familial Mediterranean fever?
If persistent treat with colchicine
Criteria for diagnosis of familial Mediterranean fever?
3 or more episodes Lasting up to 96 hours 2 or more of: fever abdo pain chest pain arthritis family hx of the disorder
Skin findings in reactive arthritis
Keratoderma blennorrhagicum on palms and soles
Oral and genital ulcer and balanitis
Pathergy is a manifestation on Behcet disease T/F
T
What is the most common long term complication of HSP?
Chronic renal disease
Factors that increase the risk of chronic renal disease in HSP?
HTN at presentation Females Older age Persistent/recurrent purpura Haematuria and proteinuria (compared to haematuria alone)
Note: tx of HSP is with NSAIDs provided renal function is ok
Patients with reactive arthritis usually have an elevated CRP and ESR T/F
F - CRP usually normal. ESR may be elevated
Note: other lab abnormality sterile pyuria
Antihistone antibody is associated with what pathology?
Drug induced lupus
What to antibodies are specific for SLE?
Anti double stranded DNA
Anti Smith
What complications of Behcets cause the most morbidity and mortality?
CNS complication (stroke, seizures, meningoencephalitis)
Blindness
Large vessel aneurysm or thrombus
Recurrent episodes of fever, anterior cervical lymphadenopathy, exudate pharyngitis, aphthous ulcers, what pathology and what treatment?
PFAPA
Tx: steroids, usually respond to a single dose 2mg/kg but can be repeated if symptoms recurrent in 2-3 days. Tx is indicated if children are missing school or adults need to miss work to stay home with child.
Tonsillectomy only if refractory to treatment
Involvement of what organs are poor prognostic indicators in SLE?
Renal
Cerebritis
Drug Rash with Eosinophila and Systemic Symptoms is associated with reactivation of what virus in 80% of cases
HHV 6
Anti-U1 RNP is a distinctive autoantibody for what disease?
Mixed connective tissue disease
