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4.26 Rheumatic disease
By Dr. Saketkoo
64
Pathology
Professional
04/26/2012

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Term
Why do autoimmune diseases often lead to cardio and cerebrovascular events?
Definition
because of the uncontrolled inflammation
Term
What types of hematological derangements are seen in lupus?
Definition
leukopenia, anemia, and thrombocytopenia
Term
T/F Lupus can cause pericarditis.
Definition
true
Term
Names some signs/symptoms of dermatomyositis?
Definition
proximal muscle weakness, shawl sign, heliotrope rash, calcinosis, interstitial lung disease; increased creatine kinase and aldolase
Term
What is the pathogenesis of scleroderma?
Definition
endothelial dysfunction and vascular insult, unbridled inflammation and the laying down of reparative collagen causes disruption of vascular architecture and subsequently architecture of adjacent tissues.
Term
Why is scleroderma associated with GI bleeds?
Definition
vascualar injury causes dilated capillary loops on the skin and along the GI tract that can abrade and cause a slow bleed as in watermelon stop or a large bleeding ectasia
Term
What are the clinical manifestations of scleroderma?
Definition
pulmonary hypertension, pulmonary fibrosis which can cause heart failure
Term
What should you suspect if a patient with scleroderma experiences an abrupt blood pressure elevation?
Definition
scleroderma renal crisis
Term
What are the 2 leading causes of death in scleroderma?
Definition
1) pulmonary fibrosis 2) pulmonary hypertension
Term
Besides synovitis, nodules and erosions on xray, what are some symptoms of RA?
Definition
pulonary fibrosis, vasculitis, constrictive pericarditis, inflammatory eye disease
Term
T/F Symmetrical polyarticular hand joint pain that produces joint deformity can be diagnosed as RA.
Definition
false! gout can also present this way
Term
What's the difference between osteoarthritis and rheumatoid arthritis of the fingers?
Definition
osteo= bony enlargment of PIP and DIP joints without much inflammation; RA= spongy swelling of PIP joints
Term
T/F Fibromyalgia is an inflammatory disase.
Definition
false
Term
What are the laboratory derangements associated with fibromyalgia?
Definition
none
Term
What causes fibromyalgia?
Definition
muscle tension prompted by stress and continued stress; muscles get tighter and tighter as we try to hold 'it' together
Term
Name the spondarthropathies.
Definition
inflammatory back disease, ankylosing spondylitis, psoriasis, inflammatory bowel disease, uveitis
Term
The spondarthropathies is related to which HLA type?
Definition
HLA-B27
Term
What are the laboratory markers associated with teh spondarthropathies?
Definition
elevated acute phase reactants such as ESR or CRP
Term
What is a contraindication for joint aspiration?
Definition
putting the needle through cellulitic or psoriatic skin to get to the joint
Term
What is the normal appearance of synovial fluid?
Definition
clear, pale yellow, viscid, and does not clot
Term
What is the glucose level of synovial fluid?
Definition
similar to serum
Term
Why is the viscosity of synovial fluid high?
Definition
because of homogenously diffuse hyaluron
Term
What is a normal protein content and cell number of synovial fluid?
Definition
protein count < 500 mg/dL and cell number < 1000/cc
Term
What causes a low viscosity of synovial fluid?
Definition
when components that contribute to viscosity clump in response to inflammation; also enzymes concurrent with inflammation degrade hyaluron, making the synovial fluid less viscous
Term
What is the glucose level of a septic joint?
Definition
lower than serum level
Term
What is the protein content and cell number in inflammed synovial fluid?
Definition
>1 gram and cell number is >10,000 to >200,000
Term
What does "birefringence" mean?
Definition
means that the substance breaks up a light ray into two rays; depending on the direction of the new rays it can be called negative or positive birefringence
Term
Describe the appearance of monosodium urate cyrstals?
Definition
needle-shaped, strongly negative birefringent crystals
Term
Describe the appearance of calcium pyrophosphate crystals.
Definition
rhomboid-shaped weakly positive birefringent crystal
Term
What are the positive acute phase reactants?
Definition
sed rate, c-reactive protein, thrombocytosis, fibrinogen
Term
What are the negative acute phase reactants?
Definition
albumin
Term
What are the units of erythrocyte sedimentation rate?
Definition
mm/hour
Term
How does inflammation cause an increased sed rate?
Definition
inflamation changes the charge of RBCs and they no longer repel each other
Term
T/F ESR is elevated in multiple myeloma.
Definition
true
Term
T/F ESR can be elevated due to renal disease.
Definition
true
Term
What is C-reactive protein?
Definition
a protein produced by the liver when stressed by inflammation
Term
What inflammatory marker is elevated in heart disease?
Definition
CRP
Term
Foaming urine is a sign of...
Definition
150 grams of protein lost in the urine/day
Term
What percent of RA patients are RF+?
Definition
75-80%
Term
What is the significance of high titers of RF in patients with RA?
Definition
more severe disease and extra-articular disease
Term
What percent of RF+ patients have a disease other than RA?
Definition
25%
Term
What diseases besides RA cause patients to be RF positive?
Definition
hepatitis C, chronic infection, other rheumatologic disease (SLE, Sjorgren's), normal process of aging, pulmonary fibrosis
Term
Is rheumatoid factor titer useful in following the course of rheumatoid arthritis?
Definition
no
Term
What ist he sensitivity and specificity of anti-CCP?
Definition
senstivity= 70-80%; specificity= 88-96%
Term
What other marker is used for RA and may actually appear earlier than RF?
Definition
anti-CCP
Term
Patients with anti-CCP are more likely to have...
Definition
poorer prognosis and extra-articular disease
Term
What are antinuclear antibodies?
Definition
antibodies specifically directed against various components of the nucleus (or cytoplasm) of tissue cells
Term
Peripheral or rim ANA is likely seen with what types of antibodies?
Definition
anti-DNA
Term
Peripheral or rim ANA is likely seen with which disease?
Definition
SLE
Term
Homogenous (diffuse) ANA is seen with what kinds of antibodies?
Definition
anti-DNA, anti-histone, anti-DNP (nucleosomes)
Term
Homogenous (diffuse) antibodies are seen in what disorders?
Definition
RA and SLE; miscellaneous disorders
Term
What kinds of antibodies are associated with speckled ANA?
Definition
anti-SM and RNP, anti-Ro and La, anti0Jo-1 and Mi-2, anti-Scl-70
Term
What diseases are associated with speckled ANA?
Definition
SLE and SS, PM/DM, PSS (Systemic)
Term
What disease is associated with anti-centromere antibodies?
Definition
PSS (crest)
Term
Which dieseases are associated with anti-nucleolar antibodies?
Definition
SLE and PSS
Term
At what uric acid level do crystals precipitate?
Definition
7 mg/dl
Term
At what serum uric acid level do tophi desolve?
Definition
<5.5 mg/dl
Term
What finding is the gold standard for diagnosing gout?
Definition
crystals in synovial fluid
Term
What percent of caucasians have HLA-B27?
Definition
5-7% of caucasians
Term
What percent of spondarthropathies are positive for HLA-B27?
Definition
85-90%
Term
What is another term for c-ANCA?
Definition
anti-proteinase 3
Term
What is the antibody associated with Wegerner's granulomatosis?
Definition
c-ANCA
Term
What is another name for p-ANCA?
Definition
anti-myeloperoxidase
Term
What diseases are associated with p-ANCA?
Definition
churg strauss, systemic vasculitides, microscopic polyangitis
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