Rhuem Flashcards
Define RA and explain pathophysiology
INFLAMMATORY SYNOVITIS
autoantibodies attack host
Neutrophils and T cells in the synovial fluid cells are activated
Cytokines, Interleukin-1, TNF-alpha, Chondrocytes all attack cartilage.
Synovium then digests cartilage, inflammatory molecules released containing interleukin-1 and TNF alpha
shared epitope in RA (HLA___?)
HLA BRB1
epidemiology of RA
FM 3:1
>15 y/old
more common in smokers
what areas does RA spare?
Lower back
DIP
WHat is a Pannus?
abnormal tissue growth cause by thickening or inflammation of the synovium.
Cytokines cause new blood vessels to develop in the synovium causing growth of extra tissues eventually forms pannus
what is a characteristic of RA seen on Physical exam and where would these be found?
Nodules
seen on
hands
olecranon
Calcaneal tuberosity
2 deformities seen in RA
Boutonniere deformity: flexion of the PIP and hyperextension of the DIP joint.
Swan neck deformity: hyperextension of the PIP and flexion of the DIP joint.
What would you see on a blood test in pts w/ RA
Hypochromatic-microcytic anemia with low serum Fe
or normal iron-binding capacity almost universal in patients with active RA
what would you find on Physical exam in a pt with RA
Warm tender erythematous joints, hands and wrists
DIP are notably excluded
Ulnar Deviation of the digits
Boutonniere’s deformity
Rheumatoid Nodules
Cysts in popliteal space (Baker’s cyst)
Decrease ROM in joints
What antibodies are usually positive in pts w/ RA
Anti-CCP
ANA (20%)
RF (although does not rule out RA if negative)
what are 2 subtypes of RA
ACPA + is a more aggressive clinical phenotype (67%)
responds better to tx
ACPA - respond less to common treatments
how many points id dx for RA
6 points
first line tx for RA
DMARD
methotrexate
Leflunomide ( alt to methotrexate high GI side affects)
what do you prescribe pts w/ RA while waiting for DMARDs to take effect
corticosteroids (prednisone) for flares
NSAIDs
COX-2
complications seen w/ RA
feltys synd
baker cyst
carpal tunnel
altanto-axial subluxation
what is felty’s synd
Triad of RA + Neutropenia + Splenomegaly
complication of RA - tx RA and this should resolve
what is Polymyalgia Rheumatica (PMR)
inflammatory disorder of pain associated with the proximal muscles
seen in elderly, more common in white women
HIGH correlation w/ GCA
key feature of PMR
key feature is pain and not weakness, strength is IN TACT
Symptoms worse in AM
Does not cause swollen joints
Patient describes general aches and pains, stiffness, especially upper arms, thighs, hip girdle and shoulders. “trouble getting dressed”
Dx????
PMR
Gold standard Dx of PMR
ESR.
abnormally high.
Tx PMR
prednisone -> QUICK response
If no improvement in 2-3 weeks, unlikely PMR
NSAIDS don’t work
Who should pts w/ PMR always be referred to?
ophthalmology for blindness
What is reactive arthritis?
Auto-immune condition that can occur post infection (typically Genitourinary or Gastro-intestinal infections)
Reaction” to an infection which took place outside of the joints
what are the common pathogens responsible for reactive arthritis?
Chlamydia Campylobacter Salmonella Shigella Yersinia
what haplotype is Reactive Arthritis strongly linked to?
HLA-B27
classic triad seen in reactive arthritis
non-infectious urethritis, arthritis
conjunctivitis
CAN’T SEE, CAN’T PEE, CAN’T CLIMB A TREE
Si/Sx of reactive arthritis
Joint inflammation characterized by redness, swelling, pain and warmth in the joints.
Lower limbs and Sacro-iliac joints most effected
Inflammation of the urinary tract and the conjunctiva.
Other symptoms: fever, weight loss, lower back pain, heel pain.
how to tx reactive arthritis
Tx offending agent (antis)
prednisone for joints
DMARDS: When NSAIDs and Steroids are ineffective Sulfasalazine (good when triggered by GI)
what is JIA?
Inflammation of the CT seen in children
6 wks of pain and swelling with exclusions of other causes
what are the 5 types of JIA
polyarticular arthritis oligoarticular arthritis systemic onset enthesis (related arthritis) psoriatic arthritis
what type is JIA manifests w/ CONSTANT pain and resembles the adult form of RA?
Polyarticular arthritis
arthritis in 5 or more joints with major symptoms of pain in the knees, ankles, wrists, fingers, elbows and shoulders.
children of ANY age
JIA that manifests w/ Arthritis in 1 or more joints,
sacroilitis, DIP joint synovitis, dactylitis and nail pitting + psoriatic rash or family hx of psoriatic rash
psoriatic arthritis
child presents w/
Repeated fevers to 103F w/ fluctuating salmon colored rash. Inflammation of the internal organs, enlarged lymph nodes and spleen?
Systemic Onset arthritis
with Systemic Onset arthritis when is malignancy suspected?
with anemia and splenomegaly
Complications involve pericarditis and pericardial effusions
young boy (8 yrs old) presents w/ Pain and inflammation where the ligaments and tendons attach to bones along w/ back pain. Dx?
Enthesis (Related Arthritis)
what type of JIA has a high risk of iritis?
Oligoarticular Arthritis
young girl presents w/ asymmetrical morning stiffness in LARGE joints knees, ankles, elbows, wrists. Does not complain of much pain, mostly stiffness. Dx?
Oligoarticular Arthritis
Pt presents w/ proximal muscle weakness, upper and lower limbs, bilaterally. she complains of difficulty raising arms, lifting object over head, combing hair. Trouble climbing stairs, getting up from sitting. Dx?
Polymyositis
Polymyositis vs PMR
polymyositis - Proximal muscle weakness, upper and lower limbs, bilaterally
PMR - pain w/o WEAKNESS
what lab values would you expect to be elevated in polymyositis
MUSCLE ENZYMES
CPK
LDH
CHECK LIVER ENZYMES
define heliotrope rash
purple discoloration on upper eyelids, flat red rash on cheeks and upper trunk
Dermatomyositis
2 identifying fts of Dermatomyositis
heliotrope rash
gorttons papules
young girl complains of muscle weakness. A muscle bx is done and reveals perivascular and perimysial inflammation. dx?
dermatomyositis
Tx for dermatomyositis and polymyositis
prednisone (corticosteroids)
extra skeletal manifestations Azathioprine Cyclophosmaide Chlorambucil Cyclosporine
INF inhibitors –infliximab
Anti-CD20 (Rituximab)
60 y/o man complains of frequent FALLING along w/ weakness of DISTAL muscles and facial muscles. Dx?
inclusion body myositis
how to differentiate inclusion body myositis from polymyo and dermatomyo
inclusion body Can have asymmetrical weakness making it different than (PM, DM)
DM - rash and seen in children
what dz is associated w/ Raynaud’s phenomenon, RA, SLE, Sjogren’s, CV dz, Cancer
drugs?
polymyositis
Name large vessel vasculitis conditions?
Giant Cell / PMR
Takayasu Arthritis
Name si/sx assoc w/ large vessel?
Limb claudication Asymmetric blood pressures Absence of pulses Bruits Aortic dilation
name medium vessel vasc conditions?
Polyarteritis Nodosa
Kawasaki Disease
name skin manifestations seen in medium vessel vasc?
palpable purpura
livedo reticularis
digital gangrene
name small vessel vasc conditions?
Microscopic Polyangiitis (MPA)
Granulomatosis with Polyangiitis GPA (Wegener’s)
IgA Vasculitis (Henoch-Schönlein)
what is standard approach to screening for large-vessel vasculitis
CT and MRI
what is a concerning sx where you MUST r/o infective endocarditis?
splinter hemorrhages
is low serum complement (C4) usually seen in vasculitis?
NO - seen in SLE
65 y/o pt comes in complaining of new onset headaches, jaw claudication (difficulty chewing food) and unexplained fever. Dx?
GCA (PMR)
vision loss is also common
tenderness of temporal artery
what is gold standard for dx GCA
Temporal artery biopsy
what sx is most highly predictive of a positive temporal artery for diagnosis?
jaw claudication
Tx of GCA in pts w/o visual loss
prednisone
Pt will respond QUICKLY
Tx of GCA in pts w/ visual loss
methylprednisolone
Pts lab results show Normochromic normocytic anemia and elevated ESR / CRP. Dx?
Takayasu Arteritis
pt will appear unwell
what is Subclavian steal syndrome
Subclavian artery involvement common -> stenotic lesion proximal to origin of vertebral artery can lead to subclavian steal syndrome
Reduced blood pressure in one or both arms
Asymmetrically diminished arterial pulses in the arms and legs
epidemiology of Takayasu Arteritis
WOMEN 7:1
ASAIN
Tx for Takayasu Arteritis
acute - prednisone
chronic - angioplasty surgical bypass
what dz spares the lungs, but kidney often affected (casing renin mediated HTN) and NOT assoc w/ ANCA.
Polyarteritis nodosa (PAN)
pt comes in presenting of nonspecific complaints: fever, malaise, weight loss, anorexia, and abdominal pain (Constitutional sx), HTN, elevated ESR. dx?
Polyarteritis Nodosa- PAN
causes of Polyarteritis Nodosa- PAN?
idiotpathic
HBV
HBC
Tx of PAN
mild disease: Prednisone
mod-severe disease:
prednisone w/ Cyclophosphamide
severe disease: methylprednisolone
key finding in Kawasaki
Child w/ fever lasting >5 days with treatment
Si/Sx in Kawasaki
Bilateral bulbar conjunctival injection
Oral mucus membrane changes (injected or fissured lips, injected pharynx, strawberry tongue)
Peripheral extremity changes (erythema of pals and soles of feet, edema of hands or feet, periungual desquamation
Polymorphous rash
Cervical lymphadenopathy
Tx Kawasaki
IVIG
Aspiring - r/o viral infection Reyes
what is Behçet Disease
triad of of aphthous oral ulcers, genital lesions, and recurrent eye inflammation
presence of HLA ___ is a risk factor for Behçet
HLA B51
leading cause of blindness in Japan?
Behçet
Tx of Behçet
steroids
colchicine / thalinomide
severe CNS or ocular sx- Cyclophosphamide
define allodynia
pain due to a stimulus which does not normally provoke pain
2 most common sx related to FM
pain
FATIGUE (80%)
1st and 2nd line tx for FM
1st NSAIDS/ tramadol
2nd TCA (r/o SUICIDE) Amitriptyline, nortriptyline
3rd SSRIs/SNRIs
Duloxetine, Venlafaxine
Fluoxetine, Citalopram
3rd line tx for FM used instead of Amitriptyline
Cyclobenzaprine
define raynauds
abrupt onset of well-demarcated pallor of the digits which progresses to cyanosis with pain and often numbness—followed by reactive hyperemia on rewarming.
primary vs secondary raynauds
Primary Raynaud’s is not associated with an underlying cause
Secondary Raynaud’s is associated with or caused by some other systemic illness or disease process
Common in pts w/ CT diseases
describe the link b/w scleroderma and raynauds
90% of patients with scleroderma experience Raynaud’s phenomenon, and it may be a presenting manifestation in many of them.
physical emac findings in primary vs secondary raynauds
In primary Raynaud’s phenomenon, the physical exam is normal between attacks.
in secondary Raynaud’s phenomenon, pits or ulcerations on the fingertips may be present in patient’s with scleroderma, CREST syndrome or thromboangiitis obliterans.
The dihydropyridine Ca++ channel blockers (e.g., Amlodipine and Nifedipine) are the most effective pharmacotherapeutic agents for ?????.
Raynauds
who does sceroderma most commonly present in?
women in 30s
AAs
Pathophysiologic Triad in scleroderma
Vasculopathy, including Raynaud’s Phenomenon—the earliest vascular complication of SSc
Inflammation and Autoimmunity—Cellular Immunity: Evidence of inflammation and autoimmunity is an invariable feature that SSc shares with other rheumatic diseases such as SLE and RA.
Fibrosis: Fibrosis of the skin and multiple internal organs is the distinguishing feature of SSc.
what is the hallmark of scleroderma
thickening and hardening of the skin (scleroderma), but most patients also manifest characteristic abnormalities in the lungs, g.i. tract, kidneys and heart.
characteristically develop extensive skin induration and early progressive internal organ involvement.
with diffuse cutaneous systemic sclerosis (dcSSc)
commonly present with long-standing Raynaud’s phenomenon, modest skin changes and indolent internal organ dz.
limited cutaneous systemic sclerosis (lcSSc)
where on the face is spared during SLE butterfly rash
nasolabial folds
who antibodies are positive in SLE
ANA test is positive in virtually all patients;
Anti Sm Abs are highly specific for SLE.
tx for sle
Prednisone
hydroquinone
Immunosuppressive Agents, alkylating agents:
Cyclophosphamide; purine synthesis inhibitors:
Azathioprine and MMF; folate antagonist: methotrexate
what age group is Sjogren’s Syndrome ABSENT in
children
what diagnostic test is used for sjogens ?
Schirmer test, performed without anesthetic eye drops.
Pharmacological Tx of sjogens -
Topical cyclosporine
although there is a concern for herpesvirus infections.
Oral pilocarpine and cevimeline may stimulate functional exocrine glands and improve sx.
Nystatin utilized for thrush complication.
what age group and gender is sjorgens seen in?
women 30-50s
composition of crystals in gout vs psueodgout
gout: monosodium urate
pseudo: Ca++ pyrophosphate dihydrate (CPPD)
arthrocenteiss shows:
Needle shaped
Negative birefringence under polarized light microscopy
dx?
Gout
after a 24 hour urine test of a pt suspected of Gout. Results show elevated level.
is this person an overproducer or underexcreter?
overproducer
90% of pts are underexcreters and will have a NORMAL level
what are examples of Xanthine oxidase inhibitor
Allopurinol - 1st line, not in mod-severe renal failure
Febuxostat
Uricosuric: underexcretor prophylaxis
Probenecid - 1st line in pts w/ CKD (cannot tolerate allopurinol)
Sulfinpyrazone
Tx for acute gout attack
NSAID - indomethacin
Colchicine
corticosteroids (r/o infection)
pt comes in w/ valgus (angulation toward midline) deformity of the knee. what is this HIGHLY suggestive of
CPPD
pseudogout
arthrocentesis shows
positive birefringence, rhomboid crystals
dx?
pseudogout
what radiographic finding is DIAGNOSTIC of pseudogout?
Radiographic findings of joint cartilage calcified and calcium deposits in joint spaces are DIAGNOSTIC!
acute tx of pseudogout
NSAID
colchicine
steroids
chronic tx of pseudo
colchicine
NSAID
90% of pts w/ gout are ________?
underexcretors
most common joint affected in gout vs pseudo
gout 1st MTP (podarga)
pseudo knee
Most common cause of Necrotizing glomerulonephritis
MPA
what vasc syndrome presents w/ splinter hemh
MPA
pt presents w/ Purpura Pulmonary hemorrhage Interstitial lung fibrosis Ulcers Splinter hemorrhages
dx?
MPA
Pt presents w/ Classic triad of upper and lower respiratory tract disease with glomerulonephritis
dx?
GPA
crusting, ulceration, bleeding, and/or perforation of nasal septum common
GPA
what is the most common vasculopathy in children
IgA
describe classic tetrad and what sx it relates to?
IgA Vascu
Purpura: ALL
Arthralgia/arthritis: Joint symptoms second most common manifestation, occur in over 50% of pts (usually affects LOWER extremities )
Abdominal pain: Colicky pain occurs in approx. 50% of patients and GI bleeding in approx. 20-30% (intussusception most common complication)
Renal disease: Frequency of renal involvement ranged from 21-54% (more common in older children)
Pt presents w/
Proptosis:
Ptosis:
Ophthalmoplegia:
Scleritis
dx?
GPA
Tx of GPA
Cyclophosphamide PO plus Prednisone
Rutuximab PO plus Prednisone
Continue Cyclophosphamide with prednisone
