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109 Cards in this Set

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Why was gout thought to be a rich person's disease?
occurs more in those who have a purine rich diet with rich foods and alcohol
What is gout?
Gout is a problem with having too much uric acid causing an arthritis
Do all people with hyperuricemia get gout?
no, only 25 %. duration and magnitude of hyperuricemia are the major risk factors.
What are normal uric acid vulaues?
men-5.1 mg/dl
women-4 mg/dl
How is hyperuricemia defined?
2 standard deviations above normal
Hyperuricemia-Pathogenesis?
Production of uric acid
Loss of uric acid
Lack of uricase
How do we produce uric acid?
endogenous purine synthesis (we make it) and dietary intake (red meat, alcohol, etc.)
How do we lose uric acid?
kidney gets rid of most of the uric acid while some of it is secreted into the gut where bacteria oxidize urate (accounts for only 1/3 of excretion). So, someone with kidney failure would have a higher uric acid level.
How often is hyperuricemia due to over production versus underexcretion?
10% overproduction
90% underexcretion
What are the routes of overproduction?
Nucleic acid turnover, breakdown of cells
Derangements in purine synthesis
What are the derangments that occur?
upregulation of PRPP synthetase
downregulation of HGPRT
What enzyme can you inhibit to treat gout?
xanthine oxidase
What are some of the diseases associated with increased breakdown of cells?
Myeloproliferative/lymphoproliferative disorders, hemolytic anemia, Paget’s disease (breakdown of bone, constant bone turnover), Psoriasis
What are the causes of underexcretion leading to gout?
Hereditary factors Medications
lead intoxication increased organic acids hyperinsulinemia
What medications can lead to gout?
cyclosporine
diuretics
low-dose aspirin
When do you develop monosodium urate crystals?
when the uric acid level crosses the soluability threshold of 6.7 mg/dL
lower temperatures also which could explain tophi in ears and toes.
What are tophi?
foreign body granulomas surrounding an MSU crystal core
What is the pathogenesis of inflammation is gout?
crystals are taken up by phagocytes and synovial cells which subsequently release cytokines that attract neutriphils and trigger neutrophils-endothelial adhesion
how does gout present?
-painful, red, swollen joint or bursa
-first MTP is most commonly affected (podagra)
-tophi in chronic forms in distal joints, bursa, or pinnae
Gout: Epidemiology
-40-50 years old
-males to females with gout is 5:1; even for women post menopausal
-tophi in chronic forms in distal joints, bursa, or pinnae
What do you do with a patient with cyclosporine induced gout?
try to lower uric acid to less than 6
how do you diagnose gout?
synovial fluid sample and analyze it for cell count, culture, and crystals
What would you find in a synovial fluid sample with someone with gout?
-increased WBC
-septic arthritis may coexist of trigger gout
-negative bifringence-yellow, parallel crystals
-white cells with crystals inside
What would an xray show in gout?
punched out erosions with sclerotic borders and overhanging edges.
What color does perpindicular crystals show?
blue
Vasculitis: Definition
Vasculitis is a term that refers to a heterogenous group of disorders characterized by blood vessel inflammation
What things can the pathogenesis of vasculitis involve?
cellular immunity
complement
pro-inflam. cytokines
ANCA
antigens
What symptoms are common to all vasculatides?
fever, malaise, arthralgias, fatigue
Name two examples that are Large vessel vasculitis?
giant cell arteritis
takayasu arteritis
Name two examples that are medium vessel vasculitis?
Polyarteritis nodosa
Kawasaki’s disease
Name the examples of small vessel vasculitis?
Wegener's granulamatosis
Churg-Strauss
Henoch-Schonlein purpura
Hypersensitivity vasculitis
Specific symptoms of vascular diseases are associated with what?
size of the vessels involved
Giant Cell Arteritis patients are commonly?
“elderly white female.”
What are the symptoms of giant cell arteritis related to external carotid?
Headache
Scalp tenderness/infarction
Ischemic optic neuropathy
“amaurosis fugax” – descending of a dark curtain, can lead to blindness
Jaw claudication
TIA
What are the symptoms of giant cell arteritis related to subclavian/brachial?
Arm claudication
Pulselessness
What are the symptoms of giant cell arteritis related to aorta?
Aortic Aneurysm
aortic insufficiency
What would the labs show in giant cell arteritis?
Mild anemia (maybe)
Elevated markers of inflammation, like ESR or CRP (almost always)
How would you diagnose giant cell?
Temporal Artery Biopsy-you would see granulomatous inflammation and maybe multinucleated giant cells. Also see disruption of the internal elastic lamina
Giant Cell Arteritis: Pathophysiology
Cell mediated inflammation in cell wall, causing disruption of the internal elastic lamina.
Describe the Cyclical fluctuation of GCA Incidence
occurs every 60 years
may be infectious
just theoretical
Polymyalgia Rheumatica
syndrome in elderly patients of constitutional symptoms and stiffness of shoulders and hips
How would you treat a patint with PMR?
low dose of steroid and see very dramatic recovery
How is PMR treated differently form GSA?
GSA is high steroid dose
How often are PR and GSA comorbid?
50% of GCA have PMR

15% of PMR have GCA
What is the typical patient with takayasu's arteritis?
“Young Asian Female”
Takayasu’s Arteritis: Symptoms
Ischemic Symptoms determined by artery affected.
Carotid/vertebral-TIA
Subclavian-pulseless/claudication
Aorta-aneurysms or insuff.
Coronary-MI in young females
Takayasu’s Arteritis: Pathology
Early, granulomatous inflammation in adventitia and outer layers of affected arteries. This progresses to include the entire cell wall.
How do takayasu's and GSA differ?
takayasu is not limited to extracranial and can therefore cause TIA
How would you diagnose Takayasu's?
angiogram-narrowing and dilation with vessel cut off past dilation.
What is palpable purpura?
characteristic slightly raised, red or purple rash we can see in medium and small vessel vasculitis. Caused by inflammation in the capillary venules allowing RBCs to leak
What would the histology report say for PP?
leukocyte occlastic vasculitis-means that there’s fibrinoid necrosis inside the vessel wall, neutrophils present, and extravasation of blood cells
What defines a small vs medium vessel?
Medium vessels: 50-150 micrometers with a muscular wall

Small vessels: Less than 50 micrometers without a muscular wall
Polyarteritis Nodosa
Inflammation of medium and small arteries
What specific organs are targeted in PN?
skin
peripheral nerves
GI
Kidney
Musculoskeltal
Testicular pain
What organ is spared in PN?
lungs
What is the pathology of PAN?
Patchy involvement
Minimal immune
This association is must less deposits
ANCA usually negative
Used to be associated w HEP B
What is the diagnosis of PAN?
Angiogram
Biopsy of involved organ
What distinguishes PAN from other vasculatides?
No glomerular nephritis, but do get involvement of small arterioles supplying the kidney, causing renin-mediated hypertension. Can also (much rarer) get infarction of kidney or renal insufficiency
Kawasaki’s Disease
A medium vessel vasculitis
Seen in childhood
Fever, Rash (often with desquamination of palms and soles), eye inflammation
Why do you treat KD aggressively with IVIG?
coronary ateries can be involved and cause aneurysms in 25% of untreated patients
What are the two main groups of small vessel vasculitides?
ANCA-associated
Cutaneous vasculitides
What disease is associated with c-ANCA (cytoplasmic)?
Wegener’s granulomatosis
What disease is associated with p-ANCA (perinuclear)?
Churg-Strauss syndrome
Microscopic Polyangitis
What are the antigens associated with ANCA?
c-ANCA 90% proteinase-3
p-ANCA 80% myeloperoxidase
Wegener’s Granulomatosis
Granulomatous necrotizing vasculitis affecting the airways and kidney
What are the clinical manifestions of WG?
Saddle nose deformity
Chronic Sinusitis
Subglottic Stenosis
Cough, hemoptysis
Glomerulonephritis
Ocular
Cutaneous
Which ANCA is WG associated with?
c-ANCA-PR3
How do you diagnose WG?
biopsy
sinus-non specific
lung-granulomatous inflammation, inflammation in capillaries
kidney-pauciimmune glomerulonephritis
Churg Strauss Syndrome occurs in the setting of what conditions?
Asthma(frequent flares) or Allergies
Eosinophils in tissue or blood
Pulmonary Infiltrates
What lab tests for CSS?
pANCA
What is the main difference between small and medium vessel vasculitis?
Difference in vessel size allows it to involve the lung and glomerulonephritis
Microscopic Polyangitis
Pauciimmune necrotizing glomerulonephritis Capillaritis
Most serious complication is alveolar hemorrhage
pANCA
Dx by biopsy
Henoch-Schonlein purpura
Tetrad of purpura, arthritis, abdominal pain, and glomerulonephritis
Most frequent in kids, and usually a self-limited process
When seen in adults, there is more kidney disease and chronic kidney disease
Mediated by IgA, you could visualize IgA via immunofluorescence
Mixed Cryoglobulinemia
90% of cases are due to Hepatitis C
Involves small vessels to skin
Immune-complex mediated-IgM and IgG and rheumatoid factor that can fix complement
What are the non ANCA diseases?
Henoch-Schonlein purpura
Mixed Cryoglobulinemia
Hypersensitivity vascultis
Cutaneous Vasculitis
What drugs can cause hypersensitivity reactions?
antibiotics such as penicillin, sulfa- drugs, NSAIDs, methotrexate, anti-depressants, anti-hypertensives
Hypersensitivity vascultis
Immune-complex (Antigen-antibody) mediated
Immune complex deposition leads to complement activation
C3a and C5a bring in neutrophils
DEFINITIONS OF SYSTEMIC LUPUS ERYTHEMATOSUS
Immunologic aberrations give rise to excessive autoantibody production, some of which causes cytotoxic damage, while others participate in immune complex formation resulting in immune inflammation
EPIDEMIOLOGY SLE
ages of 15-40
female 5:1
1 in 2000
Increased in African american
Genetics of SLE
high concordance in identical twins and increased frequency of disease among 1st degree relatives
Disturbance of what system predisposes someone to lupus?
complement-C1q if highly associated and C4 put you at increased risk
What makes us think lupus has a hormonal component?
it mainly affects women at child bear age
What is the central abnormality in pts with SLE?
autoantibody production
what are some other abnormal mechanisms of SLE?
-uncontrolled b cell prolferation
-uncontrolled T cells activating b cells
-aberrant clearing of immune complexes
-increased apoptosis and impaired clearance
What causes the morbidity and mortality in SLE?
organ failure
SLE patients should have 4 of 11. what are they?
• Malar Rash
• Discoid Rash
• Photosensitivity
• Oral ulcers
• Arthritis
• Serositis
• Renal disorder
• Neurologic disorder
• Hematologic disorder
• Immunologic disorder
• Antinuclear Antibody
What are the skin manifestations of SLE?
Malar rash
discoid rash
photosensitivity
butterfly rash
interarticular dermatitis
subacute cutaneous lupus
panniculitis
urticarial lesions
vasculitis
non-scarring alopecia
Malar Rash
fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds.
Discoid Rash
Characterized by erythematous raised patches with adherent keratotic scaling and follicular (hair follicles) plugging. Atrophic scarring may occur in older lesions
Photosensitivity
Characterized by a skin rash as a result of unusual reaction to sunlight. Patients will become sick from sun exposure and it may cause a flare in the lupus. Some believe that the sun kills skin cells, causing exposure to nuclear debris, leading to a flare in the lupus.
Classic “butterfly” rash is
an erythematous, elevated pruritic and/or painful rash in a malar distribution that is precipitated by sun exposure and spares the nasolobial folds
How can you tell if a rash is autoimmune?
immunofluoresence at the dermal epidermal junction
What are the acute skin manifestions?
malar rash
generalized erythema
bullous lesions
What is subacute lupus?
presents with annular papulosquamous rash that resembles psoriasis
How would chronic lupus manifest on the skin?
discoid lupus or lupus profundus, which is more of a panniculitis (deeper beneath the skin, an inflammation of the fat).
Mucus membrane lesions of lupus
-mouth ulcers of the hard palate behind the front teeth
vaginal ulcers
nasal septal erosions
serositis (pleuritis and pericarditis)
Musculoskeletal features of SLE
arthralgia
arthritis
Jaccoud’s arthropathy
Myopathy
What is the differnce between jaccouds and arthritis in RA?
hand position is completely reducible
What are the renal manifestations of SLE?
persistent proteinuria
cellular casts
hematuria(RBC)
pyuria(WBC)
elevated serum creatinine.
what are cellular casts?
white cells and red cells that have been filtered into the renal tubules and congeal together to take the form of the renal tubules
What are the neurological manifestations of lupus?
Seizures
psychosis
What are the pulmonary manifestations of SLE?
pneumonitis
pulmonary hemorrhage
pulmonary hypertension
What are the cardiovascular manifestations of SLE?
Pericarditis
myocarditis
endocarditis
non-bacterial verrucous disease
coronary artery disease
raynauds
nailfold capillary changes
What are the hematologic abnormalities of SLE?
hemolytic anemia
leukopenia
lymphopenia
thrombocytopenia
What are the immunological abnormalities of SLE?
ANA (95%)
anti-RO
anti-DNA
anti-Smith
false + for syphillis
anti-dsDNA
very specific for lupus, especially renal disease in lupus
anti-histone
drug induced and will usually go away after time
anti-ROLA
put patient at risk for having neonatal lupus in their child bc the abs cross the placenta and cause rash and heart block in the fetus
anti-ROLA
put patient at risk for having neonatal lupus in their child bc the abs cross the placenta and cause rash and heart block in the fetus