Rheumatology Flashcards
Rheumatoid Arthritis is
A systemic auto-immune disease that manifests in the joints
Uncontrolled proliferation of synovial fluid
Synovitis, symmetrical, small joint
Genetics of RA
Shared epitope confers genetic risk
HLA-DRB1*04
Strongly associated with ACPA (+)
RA pathogenesis
ACPA
Anti-cutrullinated protein antibodies
Associated with RA
Anti-CCP
Why people get RA
Genetic susceptability
Insult/trigger
Criteria for RA
Morning stiffness
Arthritis in 3 joint areas
Arthritis in hand
Symmetrical arthritis
Rheumatoid nodules
Serum rheumatoid factors
Radiographic changes
must be present for more than 6 weeks
RA is
Small joint
Synovitis
Symmetrical
Small joint
Synovitis
Symmetric
Rheumatoid arthritis
RA deformity
Boutoniere Deformity
Swan neck
Ulnar subluxation
RA labs
Markers of inflammation
Anemia of chronic disease on labs
Thrombocytosis
Decreased albumin
***CCP
Rheumatoid factor
Autoantibody against Fc portion of IgG (IgM, IgG, IgA)
Higher titer= worse disease
Indolent infections (endocarditis, Hepatitis B and C virus)
B cell lymphomas
Mixed cryoglobulinemia (Hepatitis C virus)
RA X ray erosions
Located at articular surface
RA joint space
Symmetric joint space narrowing
C1-C2 subluxation
Significant risk for RA patients
Associated with RF and ACPA
RA nodules associated with
high titer RF
Other organ systems involved with RA
Caused by systemic inflammation
Lungs: nodules
Heart: pericardial effusions
RA: corneal melt
Skin: pyoderma gangrenosum
RA treatment
Many different treatment options
NSAIDS (bandaid, should not be used as monotherapy)
DMARDS
Early diagnosis and treatment to target is the GOAL
Use combinations to achieve disease remission when needed (synergistic at lower doses and result in less toxicity)
Methotrexate
ANCHOR drug for RA
Dose ONCE WEEKLY to avoid liver toxicity
Contraindicated in Renal Disease (kills bone marrow and will kill patient)
What must you screen for before giving a biologic
Tuberculosis and Hepatitis B
Triple therapy for RA
MTX + SSZ + HCQ
Raynaud’s Phenomenon (RP)
Exaggerated vascular response to cold temperature, emothional stree, vibration
tri-phasic color change with clear demarcation (white, blue, red)
Primary: younger, symmetric
Secondary: older, assymetric (may develop ulcers and secondary necrosis- autoamputation)
Raynaud’s phenomenon treatment
core body warming
Sjogrens Syndrome
systemic autoimmune disease associated with lymphocytic infiltration of exocrine glands
can be primary or secondary
Sjogrens Syndrome clinical presentation
Xerophthalmia (dry eyes)
Xerostomia (dry mouth)
Raynaunds phenomenon
vasculitis
Non-errosive inflammatory arthritis
Interstitial Lung disease
Type I renal tubular acidosis
Marked increase in risk on non-Hodgkin’s Lymphoma (heralded by decline in RF titer)
CT Scan of splenomegaly with lymphoma in SjS
Sjogrens Syndrome Serology
ANA
Anti-SSA/SSB (Ro/La)
Rheumatoid factor
Sjogrens Syndrome
Shirmer’s test: test for dry eye
Salivary Gland biopsy
Systemic Lupus Erythematosus
Prototypic immune complex deposition disease (low self tolerance)
Universally ANA Positive
For SLE you need how many criteria
4/11 positive
Photosensitive malar rash
Lupus
Lupus
Oral ulcers
photosensitivity
Lupus rash
alopecia
Discoud lupus
subacute cutaneous lupus
Jaccoud’s Arthropathy: non erosive inflammatory arthritis
pericarditis and valvular lesions
pleural effusions
pneumonitis (diffuse alveolar)
shrinking lung syndrome
SLE nephritis
look for active urinary sefiment on the urinalysis (blood and protein)
anti-dsDNA
low compliment
kidney biopsy: light miscroscapy, immunoflouresence, electron microscopy
SLE serology
Positive ANA
Suberologies:
anti-smith (most specific)
anti-dsDNA
renal disease
anti-phospholipid Abs
clotting risk
SLE Hematologic disease
lymphocytopenia
Drug induced lupus
Serology: ANA and anti-histone (in isolation)
Renal disease is very rare
Neonatal Lupus
autoimmune disease from passive transfer of autoantiboides from mother to fetus resulting fetal and neonatal disease
Anti-Ro (SSA) and Anti-La (SSB)
Rash and heart block
Anti-phospholipid antibody syndrome
pregnancy loss, vascular thrombosis, circulating anti-phospholipid antibodies
Antiphospholipid Abs include:
Lupus anticoagulent (clotting test with mix)
PTT or dRVVT (will be increased)
mix with normal serum
factor defiency (PTT corrects)
inhibitor (PTT won’t correct)
Anti-cardiolipin Ab
B2-Glycoprotein-I Ab
Clinical findings APS
DVT
recurrent fetal loss or other OB complications
livedo reticularis
Avascular necrosis
CAPS
Avascular necrosis
cellular death in bone due to the loss of blood supply
cause by glucocorticoid use
inflammatory/autoimmune disease
Inflammatory Myopathies
indifious, progressive and usually painless proximal muscle weakness
immune mediated myopathies
polymyositis (PM) and dermatomyositis (DM)
noted to coexist with cancer especially DM
Dermatomyositis
Heliotrope rash on eye lids
gottrons papules on knuckles
Rash can occur at any time
V sign or shawl sign around the neck
periungual erythema
mechanics hand
Clinical findings of PM and DM
dysphagia
dyspnea from interstitial lung disease (ILD)
Antisynthetase syndrome (subset of PM with rapidly progressive ILD and mechanics hands)
Anti-Jo-1
mechanics hands
PM and DM labratories
Elevated markers of muscle damage: CPK, aldolase, LDH
LTFs may also be up (AST and ALT are released by muscle)
Anti-Jo-1 is an antisynthetase Ab
DM and PM diagnosis
MRI or MRI STIR
Muscle biopsy
cancer testing
left-Polymyositis: perifascular inflammation
right- dermatomyositis: perivascular inflammation
Progressive Systemic Sclerosis (Scleroderma or PSS)
multisystem disease resulting in fibrosis of the skin, internal organs, and other connective tissues
Abnormal pathways:
vasculopathy
Inflammation: cell mediated and humoral
Fibrosis: fibroblast undergo permanent changes
Scleroderma clinical findings
RP, GERD, arthralgia/myalgias
puffy painful skin
sclerodactyly
telangiectasia
end stage leads to contraction and ulcers
RP is nearly universal
fibrosis or pulmonary hypertension
Gastrointestional: reflux, GAVE leading to bleeding
malignant hypertension
Scleroderma renal crisis which is treated with ACE inhibiots (life saving)
onion skin appearancw of renal blood vessels
Scleroderma diagnosis
positive ANA
Diffuse:
Centromere: CREST syndrome
Limited:
Scl-70: systemic sclerosis (scleroderma or PSS)
RNA Polymerase III (anti-POL III): rapidly progressive scleroderma
Spondyloarthritis (SpA)
Ankylosing spondylitis (AS)
Reactive Arthritis (ReA)- formerly Reiter’s syndrome
Psoriatic arthritis (PsA)
Arthritis associated with inflammatory bowel disease
SpA has a strong association with
HLA-B27
SpA: etiology and Pathogenesis
higher prevelance in males
Reactive arthritis is tiggered by
Salmonella, Shigella, Campylobacter, Yersinia, chlamydia
SpA common features
inflammatory low back pain
insidious onset
duration greater than 3 months
significant morning stiffness and improvement with exercise
SIJ involvement
Associated findings: Ocular (Uveitis), Dactylitis (sasuage digit), Enthesopathy
Dactylitis
flexor tenosynovitis
SpA common feature
Ankylosing Spondylitis
spinal fusion
SIJ inflammation and fusion
Inflammatory arthritis (large joint below the belt)
Head to wall test, bamboo spine
SpA: reactive arthritis
Classic Triad: Conjunctivitis, urethritis, and arthritis (cant see, pee, or climb a tree)
asymmetric arthritis
enthesopathy and achilles tendonitis (lovers heel)
circinate balanitis/keratoderma blennorrhagica (rash)
oral ulcers
Psoriatic Arthritis characteristics
enthesopathy
dactylitis
exuberant syndesmophytes
nail pitting and onycholysis
DIP joint affected
pseudorheumatoid
oligoarticular
arthritis mutilans (pencil in cup erosions
oil spots and nail pitting
IBD arthritis
sacroiliitis
enthesitis
uveitis
stomatitis
pyoderma gangrenosum
erythema nodosum
panniculitis on LLimb
Vasculitis
inflammation in blood vessels
always try to biopsy for diagnosis
biopsy tissues fed by artery when vessel can’t be biopsied
Giant cell (temporal) arteritis
headaches, vision loss, jaw claudication, constitutional symptoms (fever, weight loss, malaise)
Lab: Elevated ESR/CRP
Diagnosis: biopsy temporal artery
ultrasound arteries (non-compressible)
Anti-neutrophil cytoplasmic antibodies (ANCA)
C=cytoplasmic
P=perinuclear
C3PO
C-ANCA PR3
P-ANCA MPO
small to meduim sized blood vessels
GPAl MPA, EGPA
pulmonary renal involvement
must biopsy for disgnosis (lung is ideal)
GPA
sinusitis
tracheitis
ocular involvement
C-ANCA (PR3)
MPA
P-ANCA (MPO)
EGPA
Asthma
Eosinophilia
P-ANCA (MPO)
Mixed cryoglobulinemia
Cyroglobulins (IgGs that precipitate below 37 degrees and dissolve upon rewarming
HCV
B cell cancers
Polyarteritis Nodosa (PAN)
systemic vasculitis affecting medium sized muscular ateries
can be triggered by Hepatitis B
Takayasu’s Arteritis (TA)
systemic vasculitis of the aorta and its branches
extremity claudication “pulseless disease”
Rheumatoid factor
RA
SjS
HCV
Cryo
B-cell malignancy
Anti-Pol III
scleroderma
Anti-centromere
CREST
C-ANCA (PR3)
GPA
HLA B27
Seronegative SpA
Anti-CCP
RA
Anti-Smith
SLE
LAC
APS
P-ANCA (MPO)
MPA
EGPA
Anti-Scl 70
Scleroderma
Anti-DS DNA
SLE nephritis
Anti-histone (in isolation)
Drug induced lupus
In inflammatory arthritis joints are
Warm, swollen, erythematous
AM stiffness
Gelling phenomenon (pain better with activity)
Non-inflammatory arthritis joints are
Cool
Bony
Pain is worse with activity and better with rest
Key questions to determine if arthritis is inflammatory or non-inflammatory
What time of day is the pain the worst?
Is your pain better or worse with activity?
Arthritis lab evaluation
Urinalysis to look for blood or protein
Inflammatory marjers
Markers of inflammation
IL-6
Albumin should decrease
Westergren Sedimentation rate (ESR)
Blood placed in verticle tube and measure distance RBC fall over an hour
-fibrinogen
-immunoglobulin
Rheumatic factor
Autoantibody against Fc portion of IgG
Prognostic of RA
Anti-CCP antibody
High specificity for RA
Anti-Nuclear antibody
NOT A SCREENING TEST
Flourescent ANA test is the gold standary
SLE
dsDNA= renal disease
Smith= specific for SLE
Ro/SSAand La/SSB= cutaneous SLE and neonatal SLE
histone alone= drug induced SLE
Scleroderma
centromere= CREST
Scl-70 (topo-isomerase) & RNA pol III
Myositis
Jo-1 (tRNA synthetase)
Myositis
Jo-1 (tRNA synthetase)
Sjogren’s Syndrome
Ro/SSA & La/SSB
Anti-Phospholipid Ab Syndrome
lupus anti-coagulant
anti-cardiolipin
ANCA (anti-nuclear cytoplasmic antibody)
antibodies directed to cytoplasmic enzymes
P-ANCA and myeloperoxidase (AGPA and MPA)
C-ANCA and serine protease 3 (GPA)
Arthrocentesis
fluid obtained and sent for gram stain, cell count, crystal analysis, PCR
low risk of infection
Normal synovial fluid is
see through
MSU
CPPD
RA radiologic findings
Marginal (joint line) erosions
OA radio graphic findings
Osteophytes
EOA radio graphic findings
Gull-wing central erosions
Gout radiographic findings
Juxta-articular erosions with sclerotic and over-hanging edges
Pseudogout radiographic findings
Punctuate or linear densities in fibro or hyaline cartilage
Hooking on radial side of MCP
SLE radiographic findings
Jaccoud’s arthropathy
Psoriatic Arthritis
Pencil in a cup body erosion
Ankylosing spondylitis radiographic findings
symmetric sacroiliitis (iliac side, distal 1/3 of joint)= fusion
Syndesmophytes
RA vs OA
RA affects any joint other than DIP
OA affects hips, knees, spinal, all hand joints, and the MTP joint of big toe
Acute Monoarticular arthritis
Septic or crystalline arthritis
Signs and symptoms of septic arthritis
Fever
Rigor
Heart murmur
Cellulitis
Instrumentation
Guarding ROM
Syngs and Symptoms of crystalline Arthritis
Tophi
Labs and imaging for acute monoarticular arthritis
CBC
ESR, CRP
Blood, urine cultures
Serum creatine and urin acid
Imagining (yield is generally disappointing)
What is central to the evaluation of an inflamed joint
Synovial fluid analysis
Cell counts with diff, gram stain, culture, crystal analysis
Synovial fluid WBC
Normal <200 WBC <25% PMN
Non-inflammatory <2000 WBC <25% PMN
Inflammatory 1,000 to 75K WBC >50% PMN
Septic 50K to 100K WBC >85% PMN
Light microscopy
WBC
fibrillation materials= rice bodies
Phagocytized WBCs
Crystals
Yellow parallel crystal
Gout
mono sodium rate mono hydrate
Negative birefringence