Rheum review

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Amyloid AA

Assoc inflammatory disorder / APR
Less cardiac

Amyloid AL

Plasma cell dyscrasia / MM
Cardiac
Liver
Renal
Neuropathy
CONGO RED...apple green birefring

Cardiac amyloid

Fat pad bx

PAN serology

None

PAN

Assoc active HBV 30% cases
Chronic vague sx
Abdom pain
Headaches
HTN
MULT ANEURYSMS S-M ARTS

Neurogenic thoracic outlet synd

4th & 5th fingers

Waxy papules in skin folds

Amyloid

Who gets RA?

Peak onset 25-50 yo
F 3:1

RA spares what joints

L-S spine

Most freq extra-articular manifestations of RA

Pulm
Vasculitis

Polyarthritis differential in general

RA**
PMR / GCA in elderly
Psoriatic
Crystal
SLE / vasculitis
Sjogren's
Any immune complex disease
Spondylitic variant
Paraneoplastic
Viral---Parvo, HepB, HCV, Rubella
Lyme (rarely diffuse)

Polyarthritis in elderly

RA
PMR / GCA
Polyarticular gout / pseudogout
Paraneoplastic

Psoriatic Arthritis

Extent of psoriasis unrelated to extent of arthritis
3 types manifestations
1. Looks like seroneg RA
2. Looks like spondylopathy (RA spares)
3. Pauciarticular...assym, 3-4 joints

DIP without involvement of PIP / MCP...characteristic

Nail pitting, onycholysis

Lupus arthritis

Bland, mild, generally
Subset looks like RA....but labs look like SLE

Lyme arthritis

Acutely can be diffuse
Chronically, PAUCIARTHRITIS...usually hip or knee.

RA xrays

Early...osteopenia near joints
Erosions later usually

DX of RA

There are criteria, but...
Have to have stiffness > 1hr
polyarthritis >3 areas
involvement of hands
symmetric swelling
ALL PRESENT >6 WKS

ANA patterns not helpful except...

centromere---limited scleroderma (CREST)
and-------------primary biliary cirrhosis

ANA centromere pattern association

1. CREST (which is limited scleroderma)
2. PRIMARY BILIAR CIRRHOSIS

anti-topoisomerase I

also called anti-Scl-70
DIFFUSE SCLERODERMA
specific
only occurs in 30%

anti-histone

r/o drug-induced lupus...95% positive

only for procainamide, hydralazine, chlorpromazine, quinidine

ANA Subtypes...ANA profile

ds DNA.............SLE
Sm (smith)........SLE
SSA..................SLE & Sjogrens
SSB..................SLE & Sjogrens
U1-RNP............MCTD
Scl-70................Diffuse scleroderma

Antibodies seen mainly in VASCULITIDES

ANCA
anti-NEUTROPHIL cytoplasmic antibodies

histone-negative drug-induced lupus...which drugs

ANCA POSITIVE
minocycline
hydralazine (also causes histone positive)

dsDNA POSITIVE
Anti-TNF drugs

4 ANCA's

2 from immunofluorescentn (pts never both)
1. c-ANCA
2. p-ANCA

2 from ELISA
1. anti PROTEASE 3 (PR 3)
2. myeloperoxidase (MPO)

c-ANCA means...

Wegener Granulomatosis

(Usually also PR3+)
50% sensitivity
90% specificity

p-ANCA positive diagnoses

depends on MPO (myeloperoxidase antibody)
pANCA alone is very nonspecific (pANCA +/ MPO-)

pANCA + and MPO+

98% specific for KIDNEY...
1. Idiopathic crescentic GN
2. Churg-Strauss
3. MPA (microscopic polyangiitis) w/ kidney involv

Low complements in Rheum

SLE
Rheumatoid VASCULITIS

Complements decreased with SLE

C4
CH50

anti CCP

anti-citrullinated cyclic peptide
EARLIER in RA than RF
More SPECIFIC for RA than RF (97%)

MORE AGGRESSIVE / EROSIVE RA

HLA- B27 incidence

A.S............................................90%
Reactive / Reiter's.....................80%
IBD, Psoriasis
Yers, Shig, Salm arthropathy.....80%
Uveitis........................................60%
RA, OA.......................................10%
Normal pop................................6-8%

HLA-D assoc

DR2-----SLE
DR3-----SLE, Sjog, Polymyos
DR4-----Severe RA

Joint aspirate with WBC > 100K

Infection
Reactive spondyloarthropathies
RA
Gout

Joint aspirate with low wbc but mostly monos and lymphs

DJD
SLE
trauma

Markers for more aggressive RA....
(therefore treat early, agressively)

antiCCP
HLA-DR4
high titer RF
constitutional sx
insidious onset
early erosions on xrays
early appearance of nodules

SYMMETRICAL & POLYARTICULAR ARTH

RA
SLE

RA DX

morning stiff 1 hr
wrists, mcp, pip...no dip
3 jts
symmetric
(6 weeks)
nodules
xrays
RF pos

Looks like RA joints but assym
(MCP, PIP, no DIP)

Hemochromatosis

RA and CAD

3X increased risk of atherosclerosis CAD

RA
Splenomegaly
Neutropenia

FELTY'S
Long-standing dz
high RF
nodules
**Splenectomy

LGL (Lg granular lymphocyte) SYNDROME
Infection suscetibility
progresses to leukemia
*No Splenectomy--makes it worse*

DMARDS FOR RA

Hydroxychloroquine
Sulfasalazine
Methotrexate

No antiinflammatory action...use concurrent NSAIDS

anti TNF

infliximab and adalimumab--monoclonal ab...
etanercept--TNF receptor blocker

ANTI-TNF...MOST BENEFICIAL COMBINED WITH MTX
HALT / POSS HEAL EROSIONS FROM RA

Histone neg anti-dsDNA+ lupus syndrome

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