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Can you name the MDTI - ALL OF RHEUMATOLOGY?

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lack of DHEA
A20 mutation leads to too much NfKb
90% male, correlated with obesity and alcohol use
Comes in both diffuse/limited versions
hypertrophy of chondrocytes, but decrease in collagen production
3 stages, last one develops B cell Lymphoma
Cytokines Involved: IL-1, IL-6, IL-17, TNFalpha
two causal reasons: highly vascularized synovium, no basement membrane
Can be caused by increase in enzyme APRT
cast nephritis
fibroblasts upregulate PDGFr in response to TGFb
30% associated with HepB infection
malar rash
HLADRB1 association
Defect in Complement Receptor 1, FCyR
40% twin concordance
Interferon Signature (1)
Cytokines Involved: IL-1, IL-6, IL-17, TNFalpha, with TNF-alpha being the boss
Can be caused by deficiency of HGPRT
Anti-SCl-70 (Topoisomerase 1)
polymorphisms in HLA DR2/DR3
Uric Acid Kidney Stones
treat acute flair up with NSAIDs, colchicine, corticosteroids
TGFb downregulated metalloproteinase release
crescentic glomerulonephritis
spares lung/kidneys
early, early disseminated, late disseminated spread
Occurrence linked to ingestion of purine/pyrimidines, endogenous production of urate, and breakdown of tissues
environmental triggers are UV exposure/DNA or RNA viruses/Medication
associated with previous damage (RA, OA), diabetes mellitus, immunosuppression
Possible mimicry between retroviruses and anti-SCL70
Vital Dye test
CXCL 13 - B-cells
African Americans have particularly high anti-SCL70, bad prognosis
Pathway: Nalp3 -> IL-1b -> NfkB -> attracting of neutrophils
problems in the WNT pathway
lymphepithelial lesion
fibrinoid kidney necrosis causes hypertension
msu crystals
Vascular injury may be initiating factor
estrogen and prolactin cause activation/proliferation of lymphocytes
>50,000 cells, >95% PMN's
antibiotic resistant arthritis possibly due to mimcry between OspA and LFA
Can be caused by increase in enzyme XO
200-2000 white cells in joint fluid
HLA DQ7/DR5 associated
Mainly affects DIP, Knee, and 1st MCP joints
slow progression over time
monoarthritic arthritis, usually 1st MTP/Knee/Ankle
TGFb/SMAD signaling
discoid rash
Bouchard's Node
Heberden's Node
ANA test involves permeabilizing cells
cells express Bax, lymphocytes express BCLxl
IFN-g/TNFa both inhibit SMAD activity
TGFb/CTGF sensitize fibroblasts and increases collagen production
transmural inflammation
2000-100,000 white cells in joint fluid
cytokines: TGFb, CTGF, PDGF(r)
ectopic lymphoid follicles in salivary glands
Pyrazinamide, Nicotinate, Lactate all stimulate URAT1
4:1 female to male ratio
fibroblasts turn to myofibroblasts
6% concordance among MZ twins
Anti-centromere antibodies seen in limited version
Anti-Smith, Anti-dsDNA, Anti-SSB
Defect in clearance of apoptosis
cells inappropriately express both MHC1 and 2
Acute Uric Acid nephropathy (especially in cancer patients with chemo drugs)
Belimumab (2)
20-200 white cells in joint fluid
Genetic defect in COL1A2, TGFb, Fibrillin 1
Interferon Signature (2)
Fibrosis leading to ischemia and organ damage
interferon signature connects viral infection to lymphocyte invasion
Schirmer's test
polymorphisms in C2/C4 genes
Cytokines: IL-6, IL-17, IFN-alpha
subchondral sclerosis
Characterized by immune dysregulation/vascular dysfunction/fibrosis
CXCL 12 - T-cells
cytoplasmic and perinuclear antibodies
75% of people get this
treat with physical therapy, braces, NSAIDs
cartilage fragments mediate inflammation
Type I/III collagen production
IFN signal induced by DNA/RNA containing complexes
increase vascularization of cartilage which allows cytokine spread
anti-ANA are first important sign
Alcohol increases conversion of ATP to AMP; produces lactate that acts on URAT1
Raynaud's Phenomenon precedes it
Can be caused by increase in enzyme PRPP
anti-ssa/ssb (not lupus)
renal hypertension due to medium vessel closure
IFNalpha upregulates TLR7/IRF7 to help uptake of DNA/RNA containing complexes
Belimumab (1)
Tophi in olecranon bursa
environmental influence of vinyl plastics, hair dyes
Sex hormones/chromosomes are important

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