Rheumatology Flashcards
(41 cards)
Sjogren syndrome
Features
Complications
Chronic inflammatory autoimmune disease
Dry eyes and mouth \+/- other organs: Trigeminal neuralgia, focal demyelinating Raynauds Pleuritis Nephrocalcinosis Arthritis, arthralgia, myalgia Episodic purpura Autoimmune thyroiditis Cytopenias
B cell lymphoma Neonatal lupus (maternal antiRo/SS-A)
Sjogren
Serology
Infiltration by
Cytokines involved
ANA (90%)
Anti-Ro/SS-A (70-90%)
- predominantly IgG
- present in 80-90% mothers with neonatal lupus offspring
- 30-50% of SLE
- also PBC
Anti-La/SS-B
- 50%
CD4 T lymphocytes
IF gamma, IL-2
Sjogren
Types (4)
Primary (10-15%) - sicca syndrome
Connective tissue (SLE, Systemic sclerosis, polymyositis)
Mixed cryoglobulinaemia
Vasculitic syndromes
Lupus
Epidemiology
Female : Male pre and post puberty
HLA associations
% dx <20years
1:2,000
Pre puberty F:M
4:1, post is 8:1
HLA-B8, HLA-DR2, HLA-DR3
Complement deficiency genes
MZ>DZ concordance
Lupus
ANA sensitivity
Specific antibodies
Others seen
98%
Ds-DNA and anti-Sm
Antiphospholipid
Antithyroid
Anti-ribosomal (cerebitis)
Mechanism
Dx
How sens/specific is dx criteria
Direct autoantibody damage Immune complex (esp renal)
Four of: Ulcers: oral/nasopharyngeal usually painless Neurological: seizures/psychosis Malar rash Arthritis Light sensitive ANA Renal involvement (80%) Rash (discoid) Autoantibodies Serositis Haematological (WCC/plt/lymphocyte/Hb)
96% sens/100% specific
Lupus
Children usually present with
Constitutional Sx (70%)
Rash (70%)
Arthritis/arthralgia (80%)
HSM/lymphadenopathy (50%)
Cytopenia
Lupus
MSK features
Non-erosive, non-deforming
Symmetrical
Jaccoud’s arthropathy (ulnar deviation of fingers)
Arthralgia
Nodules
Avascular necrosis from vasculitis
Myopathy
Myositis
Lupus
Neurological manifestations
30%
Headache most frequent
Wide areas of scattered microinfarcts & non inflammatory vasculopathy
Depression & anxiety
Psychosis Seizures Cerebellar ataxia Transverse myelitis Aseptic meningitis (oligoclonal bands)
Lupus nephritis
Frequency
Biopsy findings: deposits
Grade 1 Grade 2 Grade 3 Grade 4 Grade 5
Occurs in 75%
50% within first year
Ig deposits in glomeruli G1- near normal G2- mesangial proliferation G3- focal proliferative GN- steroid G4- diffuse proliferative GN- steroid, cyclophosphamide G5- membranous proliferative- steroid
Lupus nephritis
Early
Rapidly deteriorating renal fnx & active urine sediment- action?
Slow rise in cr, high proportion of sclerotic glomeruli- action?
Asymptommatic haematuria, proteinuria
Treatment, no biopsy
Transplant candidate, unlikely to respond to immunosuppressive
Lupus
Cardiac manifestations
Serositis
Valves thickening- MS, associated with anticardiolipin abs- inflammatory & thrombotic material
Conduction abnormalities
Cardiomyopathy
Ischaemic heart disease
Lupus
Haematological
Anaemia common
- of chronic disease
- bone Marrow suppression by drugs
- haemolytic (25% warm autoantibodies)
- blood loss (NSAIDS)
Leucopenia 50%
Lymphopenia/thrombocytopenia- parallels disease activity
APLS Recurrent thrombosis Recurrent fetal loss Thrombocytopenia Coombs+ve anaemia Livedo reticularis Valvular heart disease Prolonged APTT
Drug induced Lupus
What drugs
Sx
How to differentiate from SLE
Antiepileptic - ethosuximide, Phenytoin Antibiotic- Idoniazid Anti-hypertensive- hydralazine Anti-arrrhythmic- beta blockers Anti-schizophrenic- chlorpromazine
Fever, arthritis/arthralgia, serositis
No malar rash, CNS or renal involvement, normal ds-DNA, complement normal, +ve anti-histone ab
Neonatal lupus
What proportion of infants of mothers with antibodies?
Main manifestations
Prognosis
10%
80% mothers asymptommatic
Rash- appears by 3 months . Photosensitive
Cardiac- complete heart block (IgG deposits in conductive system)
Hepatomegaly splenomegaly
Raised transaminases
Cytopenias, haemolytic anaemia
Skin & blood transient
Conduction probs permanent: PPM
Differential is NOMID
False positive antiphospholipid syndrome seen in what infectious disease
Antibodies
VDRL
Anticardiolipin
Lupus anticoagulant
Which markers in Lupus correlate with disease activity?
Which do not?
Anti-dsDNA
C3/C4
Esr/crp
Cytopenias
Do not:
ANA & anti-SM
Lupus
Immunosuppressive agents used & indications
Azathioprine- prevent renal disease relapse
Cyclophosphamide- Lupus nephritis, CNS disease refractory to steroid, pulmonary haemorrhage
MTX- severe synovitis
IVIG- steroid resistant thrombocytopenia
Infections that cause reactive arthritis
HLA association
Onset
How many joints
ESR
Salmonella Shigella Yersinia Campylobacter Chlamydia Gonococcus
HLA B27: yersinia enteritis —> uveitis
Spondyloarthropathy
Days to weeks
Oligoarticular, lower limbs
ESR raised
No fever
Keratoderma blennorrhagica
Describe
Associated with
Hyperkeratotic rash on soles/ palms
Reactive arthritis
May have balanitis
JIA
Define
Epidemiology
Classified by…
Chronic arthritis persists for minimum 6 weeks
Active exclusion of other causes
By mode of onset during first 6 months
1 per 1,000 children
Oligoarticular onset
3 subgroups
Others presenting this way
Young child ANA positive at risk of iritis
Older boys HLA B27 positive develop enthesitis
Those that extend past 4 joints
Juvenile psoriatic
Arthritis of IBD
Reiter
Systemic onset JIA
Sex differences pre and post puberty
Clinical features
Equal less than 5
F>M over 5
Once daily fever Myalgia Arthralgia Rash- Salmon pink or red maculopapular Lymphadenopathy HSM Serositis Hepatitis Progressive anaemia DIC Arthritis
sJIA
Recurrent in ___
Progressive arthritis in ___
Amyloidosis in ____
Rx
50%
1/3rd
Persistent disease, europeans
Corticosteroids
Anakinra (anti IL-1)
Tocilizumab (anti IL-6)
