Rheumatology - Hurley Flashcards
what is rheumatoid arthritis?
chronic inflammatory d/o that MAINLY attacks joints producing INFLAMMATORY SYNOVITIS
what does rheumatoid arthritis produce?
inflammatory synovitis
what is rheumatoid arthritis highlighted by?
severe pain
name of rheumatoid arthritis comes from?
rheumatic fever
what organs (besides joints) can RA affect?
lungs, pericardium, pleura, sclera
what cells play a role in RA?
APC, B cells, MHC, CD4+ T cells
what does RA’s pain intensity and deterioration of joint structures lead to?
deformities and disabilities
what race is affected more with RA? smokers or non-smokers affected more? gender?
Native Americans
Smokers more
Women 3x more
what happens to synovial membrane in RA?
becomes hyperplastic
gets infiltrated with immune and inflammatory cells (macrophages, B and T cells, dendritic cells, plasma cells)
what cells play a role in the continuous inflammation of RA?
increased level of cytokines
pts with what genetic sequence have higher RA and anti-CCP positivity? what MGC class is it?
HLA-DRB: “shared epitope” -> MHC class 2
what is synovitis?
inflammation of the synovial membrane that lines the joints and tendon sheaths
what happens to joints with synovitis?
joints become swollen, tender, warm, and stiff -> limited movement
does RA affect multiple or single joints?
MULTIPLE
what joints is RA commonly seen in?
small joints of hands, feet, cervical spine
larger joints - shoulders and knees
where is RA NOT seen in the back?
lower back
joints in RA are affected in what pattern?
symmetrical fashion
what is characteristic of RA?
rheumatoid nodule that’s subcutaneous
what is the central of the rheumatoid nodule like?
central area is of fibroid necrosis (pattern looks like fibrin)
what surrounds the central necrosis in the rheumatoid nodule?
layer of palisading macrophages and fibroblasts
how big is the rheumatoid nodule and where is it found?
3mm-few cm in diameter
found over bony prominences
- the olecranon
- the calcanea tuberosity
- the meta-carpophalangeal joints (hands)
sx’s of cervical spine affected by RA?
neck stiffness and general loss of motion
sx’s of shoulders affected by RA?
loss of motion
sx’s of elbow affected by RA?
evidence synovitis - palpate fullness and thickening in the radiohumeral joint
sx’s of hand and wrist affects by RA?
wrists ALWAYS affected
MCP and PIP joints involved
DIPS ARE SPARED
what joints in the hands are spared in RA?
DIPS
what joint in foot/ankle are affected by RA?
MTP, talonavicular and ankle joints
what CLASSIC hand deformities are seen in RA?
Swan neck deformity (flexion at DIP and hyperextension at PIP)
Boutonniere deformity (flexion at PIP and hyperextension of DIP)
what happens to the lungs in RA?
develop excess fibrous connective tissue
what happens to the eyes in RA?
keratoconjunctivitis sick, episcleritis, sleritis
what happens to the skin in RA?
rheumatoid nodules, dermal vasculitis lesions
what happens to the heart in RA?
pericardial effusion but no sx’s
what happens to the nervous system in RA?
instability of C1-C2, peripheral nerve entrapment and vasculitis -> results in mono neuritis multiplex
what happens to the blood in RA?
hypochromatic-microcytic anemia with low serum ferritin or normal iron-binding capacity
cardiac complications of RA?
MI, stroke, atherosclerosis, pericarditis, endocarditis, left ventricular HF, vasculitis
what is seen on PE of RA?
warm tender erythematous joints, hands, and wrists
ulnar deviation of digits
Boutonniere’s deformity
Rheumatoid nodules
Baker’s cyst (cyst in popliteal space)
criteria for classification of RA
at least 4 must be met:
- morning stiffness >1 hr for most mornings for 6 weeks
- arthritis and soft tissue swelling of more than 3 of 14 joints, present for at least 6 weeks
- swelling of hand joints, present for at least 6 weeks
- symmetric joint swelling for at least 6 weeks
- subcutaneous nodules
- Rheumatoid factor level above 95th percentile
- joint erosion seen on radiology
what is the BEST initial dx step for RA?
other dx for RA?
radiographs - x-ray or CT
others:
- Rheumatoid factor
- anti-cyclic citrullinated peptide antibody (MOST SPECIFIC)
what is MOST SPECIFIC for Ra dx?
anti-cyclic citrullinated peptide antibody
if pt has only 1 inflamed joint, or asymmetric joint inflammation, what should you check to r/o RA?
check synovial fluid to r/o RA
-may be SEPTIC ARTHRITIS
what is the rheumatoid factor (RF)? if negative does it rule out RA?
RF is a specific antibody in the blood
if negative RF, does NOT r/o RA (common to be negative in 1st year of disease)
what is RF negative arthritis called? when is it common to be negative?
SERONEGATIVE
common to be seronegative in the 1st year of the disease
when does damage of RA occur in most pts? death?
damage occurs early
- joint space narrowing in first 2 years
- disabled at 10 years
death comes early - women lose 10 years of life, men 4
what is the MAINSTAY of tx for RA?
Methotrexate (MTX) with or without TNF inhibitors (infliximab, etanercept, adalimumab)
newly dx RA pts may need what meds to hold over until MTX works?
corticosteroids
what corticosteroid and when can corticosteroids be used in RA?
Prednisone can be used to achieve rapid response or when there are “flares”
what meds are FIRST LINE for pain in RA? intra-articular injection of?
NSAIDs - FIRST LINE FOR PAIN
intra-articular injection of Triamcinolone
when is hydroxychlorquine used with MTX for RA? what is it NOT considered?
used early in mild disease
NOT considered a true DMARD b/c doesn’t slow progression
what is the DMARD of choice for tx of RA?
Methotrexate
MOA of methotrexate?
inhibits DNA synthesis thru inhibition of dihydrofolate reductase -> decreasing de novo production of purines and pyrimidines
induces adenosine release
monitoring of DMARDS? what are sites of adrs? monitoring when?
frequent monitoring - 4-8 weeks intervals
blood, liver, lung, and kidneys are frequent sites of adrs
see pt 3-6x/year and if get ill, see more urgently
MAIN complication of RA?
Felty’s syndrome
what is Felty’s syndrome?
complication of RA
Triad of RA + Neutropenia + splenomegaly
how do you dx Felty’s syndrome?
on PE and CBC
when does Felty’s syndrome resolve?
when treat RA
if pt with Felty’s syndrome and fever, how do you treat?
treat with neutropenic precautions and IV abx
why is spleen enlarged in Felty’s syndrome?
d/t inflammation
other complications of RA besides Felty’s syndrome?
Baker’s cysts, Carpal Tunnel syndrome
Atlanto-axial subluxation
if pt has Atlanto-axial subluxation, what should they be cautious with? avoid who? what can they develop?
caution with cervical motion, avoid chiropractors, can develop hand and foot paresthesia and paraplegia
mechanism of Tocilizumab?
blocks IL-6
mechanism of Abatacept?
inhibits co-stimulation of T cells by blocking CD28-CD80 and signaling of APC cells
mechanism of Rituximab?
targets B cells, deplete B cells, no inflammation
mechanism of Tofacitinib?
JAK1 and 3 inhibitors
what is polymyalgia rheumatica?
idiopathic, inflammatory d/o of pain associated with PROXIMAL muscles (shoulder, hip, neck)
onset of polymyalgia rheumatica? self-limiting?
abrupt onset and self-limiting
unlike polymyositis and dermatomyositis, key feature of polymyalgia rheumatica is what?
PAIN, not weakness
when are sx’s of polymyalgia rheumatica worse?
AM
high correlation b/w polymyalgia rheumatica and what?
temporal arteritis
who gets polymyalgia rheumatica?
elderly (50+)
what will pt with polymyalgia rheumatica describe about their sx’s?
general aches and pains, stiffness, especially upper arms, thighs, hip girdle, and shoulders
TROUBLE GETTING DRESSED
does polymyalgia rheumatica cause swollen joints?
no!!!
strength is ___ in polymyalgia rheumatica
intact
dx of polymyalgia rheumatica
dx of exclusion - made clinically
best test for polymyalgia rheumatica?
ESR (will be increased)
what is the FIRST LINE tx of polymyalgia rheumatica?
oral corticosteroids (lowest effective dose) -Prenisone
since polymyalgia rheumatica has high correlation with temporal arteritis, what should all pts have?
a temporal artery bx or consult with ophthalmologist
what is reactive arthritis?
autoimmune response that can occur post-infection (typically GU or GI infections)
what GU/GI infections are associated with reactive arthritis?
Chlamydia, campylobacter, salmonella, shigella, yesinia
what protein is found on surface of wbcs and pre-disposes you to autoimmune diseases like Reactive Arthritis?
HLA-B27 - positive in Reactive Arthritis test
what is the classic triad of reactive arthritis?
non-infectious urethritis, arthritis, conjunctivitis
CAN’T SEE, CAN’T PEE, CAN’T CLIMB A TREE
dx of reactive arthritis
no dx test to confirm
dx based on hx and clinical findings
-CBC and CRP increased, UA, blood, urine, stool and wound cultures r/o infection, HIV testing, echo for aortic regurg
HLA-B27 + in 75%
tx for reactive arthritis
Abx (if infection), NSAIDs, corticosteroids
DMARDs (when NSAIDs and steroids don’t work) - use Sulfasalazine or MTX
what DMARDs are used in reactive arthritis when NSAIDs and steroids don’t work?
Sulfasalazine or MTX
what is one of the leading causes of preventable visual loss in developed countries?
anterior uveitis (seen in reactive arthritis)
sx’s of anterior uveitis?
red eye, pain worsens when reading, progressive, blurred vision, photophobia, excess tear production, abnormally shaped pupils
what is juvenile idiopathic arthritis? primary pathology?
arthritis in pts <16 y/o (commonly 1-6 y/o)
primary pathology is inflammation of the connective tissues (autoimmune, non-infectious, inflammatory condition)
what is the FIRST sx of uvenile idiopathic arthritis?
limping
how many joints does juvenile idiopathic arthritis involve?
1 or many joints
what is often seen with juvenile idiopathic arthritis?
fever and rash
duration of juvenile idiopathic arthritis? when does it resolve?
> 6 weeks
-resolves by puberty
juvenile idiopathic arthritis is characterized by what sx’s?
major changes in joints including inflammation, contractures and joint damage which affect mobility, strength, and endurance
lethargy, reduced activity, poor appetite
all children with juvenile idiopathic arthritis experience what?
experience periods when sx’s reduce in severity or disappear, but may go from sx free to extreme pain quickly
what are extra-articular sx’s of juvenile idiopathic arthritis?
iridocyclitis (inflammation of iris and ciliary body), growth disturbances, leg length discrepancies
when all children go sx free with juvenile idiopathic arthritis, what should they be encouraged to participate in?
developmental activities
4 types of juvenile idiopathic arthritis?
systemic onset arthritis
polyarticular arthritis
oligoarticular arthritis
enthesis-related arthritis
sx’s of systemic onset arthritis?
repeated fevers to 103F w/fluctuating salmon colored rash
inflammation of the internal organs and joints, anemia, and leukocytosis
sx’s of polyarticular arthritis?
arthritis in 5 or more joints with major sx’s of pain in the knees, ankles, wrists, fingers, elbows, and shoulders
CONSTANT PAIN
sx’s of oligoarticular arthritis?
arthritis in 4 or fewer joints w/in the first 6 months of onset
females > males
those dx before age 7, best chance of disease subsiding
large joints (knees, ankles, elbow, wrists) affected
what is anthesis-related arthritis? aka?
form of juvenile idiopathic arthritis that involves the ligaments as well as spine
aka spondyloarthritis
work-up for juvenile idiopathic arthritis?
- CBC (normal)
- ESR (normal)
- ANA (positive)
- RF (negative)
- Synovial fluid (Class 2 - inflammatory)
-X-rays (soft tissue swelling, periarticular osteoporosis, growth disturbances, loss of joint space)
tx of juvenile idiopathic arthritis
refer to rheumatology
sx control, prevent joint damage and maintain fxn
intra-articular steroid injections if only a few joints involved
PO prednisone (lots of adrs)
DMARDs +/- biologics
what are 3 inflammatory myopathies?
polymyositis, dermatomyositis, and inclusion body myositis
what is polymyositis?
immune mediated muscle disease that involves inflammation of the muscles and associated tissues in response to cell damage
polymyositis associated with? what race?
Raynaud’s phenomenon, RA, SLE, Sjogren’s, CV disease, cancer
drugs like D-penicillamine, statins, AZT
black race
sx’s of polymyositis
PROGRESSIVE SYMMETRICAL PROXIMAL MUSCLE WEAKNESS BILATERALLY
difficult raising arms, lifting objects, combing hair
trouble climbing stairs, getting up from sitting
trouble lifting objects over head
systemic sx’s of polymyositis?
GI (dysphagia, bloating, constipation)
Cardiac (arrhythmias, conduction defects)
Pulmonary (aspiration pneumonia, interstitial lung disease - anti-jo1, bronchiolitis obliterates)
Renal (ATN)
dx work-up for polymyositis
CBC - leukocytosis
elevated CK, LDH (muscle enzymes)
elevated liver enzymes
Electromyography (EMG)
muscle bx (looks for inflammation, tissue damage, enzyme deficiencies)
FIRST line tx for polymyositis? monitor what?
Corticosteroids (Prednisolone) - monitor CK levels, muscle strength
SECOND line tx for polymyositis if Corticosteroids fail in 4 weeks?
Immunosuppressive agents
- if no response to steroids in 4 weeks
- if extra-skeletal manifestations
Aza, cyclophosphamide, chlorambucil, cyclosporine
what is dermatomyositis?
rash accompanying muscle weakness
pathognomonic rashes for dermatomyositis?
HELIOTROPE RASH
-purple discoloration on upper eyelids, flat red rash on cheeks and upper trunk
GROTTON’S PAPULES
-raised violaceous scaly rash on knuckles
dx of dermatomysotitis?
muscle bx reveals perivascular and perimysial inflammation
tx for dermatomysoitis?
Corticosteroids (Prednisolone)
what is inclusion body myositis?
> 50 y/o, most common inflammatory myopathy in older pts
what makes inclusion body myositis different from polymyositis and dermatomyositis?
can have asymmetrical weakness in inclusion body myositis
what muscle weakness/atrophy in inclusion body myositis causes falling? what other muscle weakness is common?
quadriceps weakness and atrophy -> causes falling
facial muscle weakness (but not ocular)
what is VERY COMMON sx of inclusion body myositis?
weakness and atrophy of distal muscles (foot extensors and deep finger flexors)
pts with inclusion body myositis can’t do what?
tie knots or hold golf club
what is seen in 60% of cases of inclusion body myositis?
dysphagia and choking
how long does it take inclusion body myositis to progress? what other disease does it look like?
takes years to progress
-looks like MD
inclusion body myositis resistant to what therapy?
immunotherapy
what meds are tried for a few months for inclusion body myositis tx?
Aza and Prednisone (low dose)
