Rheumatological 1 Flashcards
Inflammatory response
- morning stiffness lasts how long
- synovial fluid analysis
- other lab results
- > 60 min
- leukocyte ct >2,000/uL
- elevated ESR and CRP
Non-Inflammatory response
- morning stiffness lasts how long
- synovial fluid analysis
- other lab results
- <30 min
- leukocyte ct btwn 200 and 2,000/uL
- nl ESR and CRP
Rheumatoid Arthritis
- etiology
- risk increased in relatives: genetic
- Heritability about 60%
- 20 alleles IDed
Allele associated with RA
HLA-DRB1
List the 4 systems with extra-articular manifestations in RA
- skin
- eyes
- pulmonary
- cardiac
RA affects on skin
- RA nodules, MC on pressure points like elbows
- pyoderma gangrenosum
- RA vasculitis (common)
RA affects on eyes
- keratoconjunctivitis sick (dry eyes)
- episcleritis (sclera red and inflamed)
RA affects on pulmonary system
- exudative pleural effusion
- interstitial lung disease
RA affects on cardiac system
- CAD
- CHF (dt CAD)
- pericarditis (friction rub)
RA - felty syndrome
- neutropenia
- splenomegaly (MC)
- RA (MC)
- sometimes fever, anemia, thrombocytopenia
What is most appropriate autoantibody for RA
CCP
Is RA arthritis symmetrical or unilateral?
symmetrical
How does RA affect joints
- small and large joints
- small joints will be bilateral
- might have single large joint
Osteoarthritis (OA)
- how common
- how relates to age
- men vs. women
- most common type of arthritis
- incidence increases with age
- men slightly more than women
Two classification for OA
- primary: no specific predisposing event (injury for ex)
- secondary: degeneration of previously injured joint
OA
- pain presentation
- symmetry?
- pain may be intermittent, occur with joint us
- joints may be tender
- asymmetric (diff from RA)
How does OA affect joints
oligoarthritic - 1 or 2 large joints or 1 or 2 small joints common
Two types of nodes found in OA
- Bouchard: PIP joints
- Heberden: DIP joint
Baker cyst
chronic effusion of popliteal fossa
Lab and imaging for OA
- no labs, may see elevated ESR during flair
- Imaging: plain film will show narrowing of joint space, subchondral sclerosis, osteophytes (bony outgrowths)
Overview of OA tx
- NSAIDs
- PT
- Joint injection
- steroids, esp with effusion
- joint replacement
- Glucosamine and chondroitin sulfate have little effect
Fibromyalgia
- incidence
- what is interesting about incidence
- 0.06% to 10% WIDE range
- misunderstanding about dx of fibromyalgia
- many don’t believe it exists
- many want to ignore
Fibromyalgia pathophysiology
- aberrant chronic pain reflex arcs
- centered on dorsal ganglia of spine
- reflex arcs fn independently
- may be exacerbated by acute or chronic pain inputs (arthritis or psych distress)
What do MRI studies show about fibromyalgia
- pain is same as other pain perception but more easily triggered
- elevation in pain-facilitating neurotransmitters (glutamate and substance P)
Fibromyalgia tx
- validation is important
- aerobic exercise
- PT
- SNRIs (duloxetine and milnacipram)
Spondyloarthritis
- three types
- Ankylosing spondylitis
- Psoriatic arthritis
- Inflammatory bowel disease-associated arthritis (reactive arthritis)
Spondyloarthritis
- gene
- HLA-B27 (MC with ankylosing spondylitis)
- insufficient to make dx
Ankylosing spondylitis
- m vs. f
3:1 M:F
Ankylosing spondylitis
- presentation
- insidious low back pain MC
- stiff worse after rest, better with use
- bilateral buttock pain, sacroiliitis (XR of SI joint helpful)
Psoriatic arthritis
- describe
- seen with what co-morbidity
- Severe inflammatory arthritis
- like RA permanent destruction of joint
- Oncholysis (painless separation of nail from nailbed)
- oligo or symmetric
- dactylitis (diffuse swelling of joint, tendon, ligament of digit) in 50%
- 15-20% of people with psoriasis
Psoriatic arthritis
- dx
- don’t use antibodies for dx
Inflammatory bowel disease related arthritis
- incidence and progression
- location of arthritis
- 20-30% of people with Crohn’s or UC
- up to 50% resolve in 6 months
- axial or peripheral
Inflammatory bowel disease related arthritis is dt what
- autoimmune, non-infectious
BUT, associated with a few orgs:
MC: Yersinia
also: salmonella, shigella, campylobacter, rarely E. Coli
For each type of spondyloarthritis, what is the main thing to look for?
- ankylosing spondylitis: pain in sacroiliac joint
- psoriatic arthritis: psoriasis of skin, hand findings
- Inflammatory bowel: Sx of Crohn’s or UC
Management of spondyloarthritis
- NSAIDs
- glucocorticoid injections
- DMARDS?
- TNF-alpha inhibitors
Systemic Lupus Erythematosis
- describe
- inheritance
- M vs. F
- multi-organ
- caused by auto-antibodies including antibodies vs. intranuclear antigens
- polygenic inheritance
- 90% women
Systemic Lupus Erythematosis
- list the 7 organ systems involved
- MSK
- renal
- neuropsychiatric
- cardiovascular
- pulmonary
- hematologic
- GI
SLE
- MSK sx in acute, sub-acute, chronic dz
- Acute: malar skin rash (butterfly) on face
- Sub-acute: polycyclic rash with hyperpig. circular plaques
- Chronic: infiltrative papules, plaques, red plaques on sun-exposed surfaces
SLE
- other MSK sx
- painless oral ulcers
- alopecia
- Raynaud’s (hypersensitive vascular endothelium)
- 85% report myalgia
SLE
- MSK joint involvement
- 90% have joint issues
- 40% get osteonecrosis of hips
- peripheral or lrg joints
- usually symmetrical but can be single
SLE
- Renal incidence
- what is MC issue
- up to 70%
- usually glomerular dz
- renal bx usually indicated - have a nephrologist do this!
SLE
- What two markers associated
- Anti-double stranded DNA
- Anti-smith antibody
SLE
- neuropsychiatric involvement
- vasculitis of the brain: common and hard to dx
- esp difficult w/o arthritis or skin sx
- 19 possible manifestations, be careful to not write off as psych issue
- Can present with peripheral nervous dz
Examples of peripheral nervous dz in SLE
- mononeuropathy multiplex or single
- autonomic lesions
- cranial lesions
- polyneuropathy
What is mononeuropathy multiplex
- Peripheral neuropathy that presents with numbness
- Generally comes and goes
- Caused by small vessel inflammation of peripheral nerves. Vessels get clogged up from autoimmune response and then reopen, clog and reopen again.
- Pts most likely won’t tell you “I have numbness that comes and goes”
- more likely to catch on ROS if ask
SLE cardiovascular involvement
- 40% pericarditis: find via exam, echo, MRI/CT
- cardiac valve thickening called “Libman-Sacks endocarditis”: non-infectious vegetations on valves
SLE pulmonary involvement
- pleuritis +/- effusion
- interstitial lung dz
- shrinking lung syndrome
Define shrinking lung syndrome
- pleuritic pain
- SOB
- progressive decrease in lung volume
- unknown cause
SLE hematologic involvement
- All three cell lines possible (anemia, leukopenia, thrombocytopenia)
- hemolytic anemia
- pure red cell aplasia
- aplastic anemia
Describe warm antibodies in hemolytic anemia
- attach to the cell membrane of the red cell
- when cells try to get through sinusoids of spleen, spleen takes chunks of the membrane off
- cell folds up on itself, no longer biconcave discs, now spherocytes
- spherocytes seen on peripheral smear
What do you always test for that is related to SLE if someone has a clot/DVT from unknown cause?
- antiphospholipid antibody syndrome / lupus anticoagulant syndrome (APLA/LAC)
- antibodies to endothelium, monocytes, platelets, which produce a pro-coagulant state
SLE
- GI involvement
- esophageal imotility
- sterile peritonitis
- mesenteric vasculitis
- mesenteric thrombosis
What is mesenteric vasculitis often confused with?
acute appendicitis
What is much more common in SLE
malignancy. ..
- 7 times increase in non-hodgkin lymphoma
How to diagnose SLE
- consider in anyone with otherwise unexplained multi system dz
- antibodies: many, Anti-double stranded DNA and Anti-Smith are two tow know
SLE how to interpret ANA results
- in light of pre and post test probability
ESR vs. CRP in SLE
- ESR preferred over CRP (CRP can be negative in acute flare)
What else should be tested for if suspect SLE
- C3 and C4 dt potetential for immune complex dz
Sjogren syndrome
- describe
- immune-mediated
- unknown cause
- infiltrative inflammation damages exocrine glands
- major/minor salivary glands, lacrimal glands, possibly others like pancreas
- 1.5% prevalence
Sjogren’s pathophys
- inflammatory infiltrates of CD-4 positive T lymphocytes
- some B cell action
Sjogren’s clinical manifestations
- Keratoconjunctivitis sick (dry eyes): dx via shimmer test (strip under lower eyelid)
- Xerostomia (dry mouth)
What are the best markers for Sjogrens
- Anti-Ro / SSA
- Anti-La / SSB
What is the definitive dx test for Sjogrens?
Lip bx
- would do if still suspect but negative antibodies
Sjogren’s management
- symptomatic
- local (eye drops, etc)
Mixed Connective Tissue disease (MCTD)
- describe
- markers
- overlap syndrome
- includes features of SLE, systemic sclerosis, polymyositis
- anti-U1-ribonucleoprotein (RNP) antibodies
- ANA with speckled pattern
Mixed Connective Tissue disease (MCTD)
- presentation
- 50% hand swelling and synovitis
- also: esophageal reflux, interstitial lung dz, pulmonary HTN
Mixed Connective Tissue disease (MCTD)
- recommended testing
- high resolution CT of chest
- PFTs
- Cardiac echo
Mixed Connective Tissue disease (MCTD)
- management
- individualized
- glucocorticoids, azathioprine, methotrexate
- help from a rheumatologist!
what are the two crystalopathies?
- Gout
- Calcium pyrophosphate deposition (pseudo gout)
Gout
- description
- intermittent attacks of inflammatory arthritis
- results from crystallization of excessive levels of uric acid
Gout
- incidence
- increasingly common in older people
- 13% >80 yo
Gout
- pathophys
- Elevated serum uric acid
- when in cooler joints, crystalizes
- crystals recruit inflammatory response (IL-6 , IL-1B, TNF alpha, neutrophils)
- uric acid is end product of purine metabolism
What do free purines arise from (3)
- ATP turnover
- purine metabolism
- cell turnover
What is the rate limiting step to purine metabolism?
Xanthine oxidase
**know this one
What is the saturation concentration of serum rate? what does this mean
- 6.8 mg/dL
- crystals unlikely to form at concentration lower than 6.8
- above 6.8 is considered hyperuricemia
What 5 variables affect crystal formation in gout?
- serum concentration
- temp
- other unknowns
- some people overproduce
- some people underexcrete
Gout Pathophys
- what is the hallmark finding of established gout attack?
- neutrophil infiltration
Acute gout
- when MC
- presentation
- MC in early am (lowest temp of toe)
- extreme pain, even sheet hurts
- red toe
Gout
- dx
- clinical presentation
- crystal analysis (microscope), gram stain, culture
- Elevated ESR and CRP
What do gout crystals look like under microscop
- negatively birefringent urate crystals
- yellow when parallel and blue perpendicular
- long like a spear
Three gout presentations
- acute - dx via microscopic exam of fluid
- inter critical: time between acute attacks
- chronic: frequent attacks
Gout
- acute management
- colchicine
- once acute phase over, start allopurinol and treat until serum uric acid <6.5 mg/dL
- continue allopurinol for life
*allopurinol is a xanthine oxidase inhibitor
Gout
- common reasons for tx failure
- failure to treat to target (<6.5 mg/dL)
- not using high enough dose, can go up to 800 mg per day
What is the best name for pseudo gout?
osteoarthritis with calcium pyrophosphate deposition (CPPD)
CPPD pathophysiology
- mech not understood
- deposition of calcium pyrophosphate crystals in CT
What should consider if in young person who presents with CPPD?
- hyperparathyroidism
- hemochromatosis**
- hypomagnesemia
- hypothyroidism
What physical finding is associated with CPPD?
- calcium deposits in cartilage
- linear density parallel to surface of cartilage
CPPD
- management
- symptomatic
- decrease inflammation
- nothing to reduce likelihood of crystal depostion
Why does acute mono-articular arthritis require needle aspiration for dx?
differentiate between
- gout
- CPPD
- infection