Rheum Cram Flashcards
Best test to screen for SLE?
ANA (almost 100% positive)
Most sensitive test for SLE?
Anti-Smith (ENA)
Most specific test for lupus nephritis?
Anti-dsDNA
ENA associated w neonatal congenital heart block?
Anti-Ro
Begins crossing placenta at 16weeks
cANCA assoc w
Wegners (granulomoatosis polyangitis)
CF
pANCA assoc w
Microscopic polyangitis Polyarteritis nodosa PSC/IBD HSP KD
RF assoc with
Rheum arthritis
Sjogrens
SLE
Histone assoc w
Drug induced lupus/SLE
HLA-B27 assoc w
Enthesitis related arthritis
Ankylosing spondylitis 90% pos
Uveitis
MTX mechanism and SEs
Blocks purine synthesis Bone marrow suppression (not at rheum doses) Teratogenic Accumulates in 3rd spaces AVOID bactrim/penicillins
Leflunomide mechanism and SEs?
Blocks pyrimidine synthesis
Liver dysfunction
Parasthesia
Teratogenic
Hydroxychloroquine mechanism and SEs?
Anti-inflammatory and immunosuppressive effects
Ototoxic - colour vision
Ophthal review 6-12mthly
Mycophenalate mechanism and SEs?
Suppressive lymphoproliferation and antibody formation
Bone marrow suppression
Increased risk of neoplasia
Infliximab mechanism and SEs?
Anti-TNFalpa
Cytopenia
Demyelination
Lupus like syndrome
Rituximab mechanism and SEs?
AntiCD20 - depletes B lymphocytes
MSK pain/weakness
Infection
Progressive multifocal leukoencephalopathy
Anakinra mechanism and SEs?
IL-1 receptor antagonist
Injection site reaction
Headache
neutropenia
Tocilizumab mechanism and SEs?
IL-6 receptor antagonist
Deranged LFTs
Injection site reaction
GI perforations
>2/52 hx of: arthritis daily fever lymphadenopathy evanescent erythematous rash serositis
Systemic JIA
Most common form of JIA?
Oligoarthritis
DIP arthritis and psoriasis with nail/finger changes and Fhx of psoriasis?
Psoriatic arthritis
HLA-B27 positive and hip/low back pain
Enthesitis related arthritis
First line mx for JIA?
NSAIDs
Steroids
2nd line DMARDs - MTX, antiTNF, anakinra
When not to use etanercept?
Uveitis
Complications of JIA?
Uveitis Limb length discrepancy/growth retardation Interstitial lung disease/Pulm HTN TMJ (50%) Decreased BMD from steroids
JIA type most assoc w chronic uveitis?
Oligoarticular
Particularly ANA pos, RF neg
JIA type most assoc w acute uveitis?
Enthesitis JIA
Particularly if HLA-B27 pos
Unremitting fever, rash, arthritis
Splenomegaly
Cytopenia, High trigs, low ESR, very high ferritin
Elevated soluble CD24
Macrophage Activation Syndrome
AKA HLH assoc w rheumatic disease
Mx of choice for MAS?
High dose steroids
Anakinra
Teenage girl, not-caucasian. Prolonged hx.
Photosensitive rash, nasal ulcers, hard palate macules
Fever, arthritis/arthralgia, hepatosplenomegaly, nephritis
SLE
Marker of disease activity in SLE? Role of C3/C4 in SLE?
Anti-dsDNA marker of disease activity
low C3 marker of disease activity but low C4 indicative of genetic pre-disposition for SLE
Mx of choice for SLE?
All pts should be on hydroxychloroquine - flare reduction, improves lipid profiles also
Steroids for acute flares (topical to skin)
Aspirin/warfarin if procoagulant
Most common inflammatory myopathy of childhood?
Dermatomyositis
Weakness, lipodystrophy/calcinosis, plaques over fingers (gottron papule) and heliotrope rash?
Dermatomyositis
Raynaud followed by skin changes distally, nail fold capillary change. ANA positive nucleolar staining?
Scleroderma
Anti-SCL-70 and anti-RNA polymerase positive?
Diffuse systemic sclerosis
Systems involvement in scleroderma?
GI - oesophagus (dysphagia, aspiration), rectal prolapse, constipation/fecal incontinence
Lungs - interstitial fibrosis, pulmonary HTN
Heart - dilated cardiomyopathy
Renal - HTN
Centromere ANA staining pattern?
CREST variant scleroderma
Mx of scleroderma?
UV therapy/topical steroids
Corticosteroids/MTX
Cyclophos & MMF for resp involvement
Petechiae/purpura mostly of lower limbs Diffuse abdo pain (+/- diarrhoea) Arthritis/arthralgia Proliferative GN IgA deposition
Henoch Schonlein Purpura
Rate of HSP recurrence? Risk of long term ERSD post HSP?
30% recurrence rate usually in 6mths
Renal disease in 1-2%, 10% of these will have ESRD
Fever, fatigue, LOW
Abdo pain
Haematuria/proteinuria
Purpura and livedo reticularis with painful nodules
Polyarteritis nodosa
Biopsy findings of polyarteritis nodes?
Necrotising vasculitis
Granulocytes and monocytes in small/medium sized arteries
Pulm haemorrhage/nasal ulceration/hearing loss
Haematuria/proteinuria
Palpable purpura
cANCA positive
Gramulomatosis Polyangitis AKA Wegners
Pulm haemorrhage/hearing loss
Haematuria/proteinuria
Palpable purpura
pANCA positive
Microscopic polyangitis
Refractory asthma
Proteinuria/haematuria
Eosinophilic infiltration
Churg Strauss
Genital and oral ulcers, scars from old ulcers
Uveitis
Erythema nodosum
Behcet’s
Neonatal lupus syndrome most associated with?
Maternal Sjogrens
DIagnostic criteria for fibromyalgia?
Diffuse MSK pain in at least 3 areas of the body, persisting for 3 months in the absence of an underlying condition
Must have at least 5 well defined tender points of examination
Periodic fevers lasting 1-3 days with arthritis, serositis and erysepiloid rash.
Assoc w Behcets, HSP, polyarteritis nodosa
Familial Mediterranean fever
MEFV gene
Risk of developing amyloidosis in familial Mediterranean fever?
30-50% if untreated
Treat with colchicine
Baby with fever lasting up to 1 week rash and abdo pain and lymphadenitis, triggered by immunisation/surgery.
Hyper IgD Syndrome
MVK gene
Fever with painful rash, conjunctivitis, episodes last weeks at a time and occur multiple times/year.
TRAPS
TNF receptor associated periodic syndrome
Receptor 1A gene mutation TNFRSF1A
Risk of amyloidosis in TRAPS?
25% - kidneys and liver
Colchicine does not treat TRAPS - NSAID/pred
Infant in first week of life with fevers, meningitis, rash, arthropathy of knees.
NOMID
Mx anakinra
Febrile attacked 1-3hrs post cold exposure, resolve within 14hrs?
Familial cold auto inflammatory syndrome
Intermittent fevers with SNHL?
Muckle-Wells
Fever, adeninitis, pharyngitis, apthous stomatitis lasting 4-5 days every month.
PFAPA/Marshall Syndrome
Periodic Fever, Apthous Stomatitis, Pharyngitis and Adenitis
Mx - prev, colchicine and tonsillectomy
Biopsy showing eosinophilic material that stains with Congo red dye and shows apple green biofringence in polarised light?
Amyloidosis
Rash, uveitis and polyarthritis with large boggy effusions. Non-caeseating epithelia granulomas with hypercalcaemia and hypercalcuria
Sarcoidosis
Granulomas of eyes, skin and joints?
Blau syndrome
