Rheumatology Step Up Flashcards
associated findings in ANA-negative lupus
- arthritis, Raynaud’s phenomenon, subacute cutaneous lupus
- risk of neonatal lupus in offspring
serology in ANA-negative lupus
Ro (antiSS-A) positive
ANA negative
CF in neonatal lupus
skin lesions
cardiac lesions - AV block, transposition
valvular and septal defects
criteria for dx. SLE (11)
malar rash discoid lesions photosensitivity oral/nasal ulcers arthritis pericarditis/pleuritis hematologic disease - cytopenias renal disease - proteinuria CNS - seizures/psychosis ANAs other ab - dsDNA, Smith, FP on VDRL
presence of what ab’s is diagnostic of SLE?
anti-dsDNA
anti-Smith
how do you diagnose a flare of SLE?
rise in anti-dsDNA
fall in C3/C4 levels (CH50 is more sensitive)
which ab’s are very SENSITIVE for SLE but not specific?
ANA
when Ro (SS-A) and Lo (SS-B) ab’s are found in SLE, they are associated with…
neonatal lupus subacute cutaneous lupus sjogren's syndrome complement deficiency ANA-negative lupus
major use of ESR
diagnose/rule out inflammatory processes
monitor the course of inflammatory conditions
major use of CRP
mainly infection - much more sensitive and specific for ESR
- if > 15, bacterial infection is present
best tx. for SLE pts with acute flare
steroids
best long term tx. for constitutional, cutaneous and articular manifestations of SLE
hydroxychloroquine
NSAIDs for less severe symptoms
what should you recommend for pts on hydroxychloroquine tx for SLE
yearly eye examination bc of retinal toxicity
Tx. of active lupus nephritis
cyclophosphamide
azathioprine
MCC of death in SLE
renal failure
infections
drugs that may cause lupus-like syndrome
hydralazine
procainamide
isoniazid
quinidine
which two organ systems does drug-induced lupus NOT affect?
CNS and renal
- if these sx are present, it is NOT drug-induced
pathophysiology of scleroderma
cytokines stimulate fibroblasts to produce collagen; high quantity of collagen is responsible for symptoms
what symptom(s) is/are present in almost all pts with scleroderma?
Raynaud’s
cutaneous fibrosis - thickening of skin of face and extremities
how do you dx. Raynauds?
nail-fold capillaroscopy - look for evidence of vessel damage
GI findings in scleroderma
dysphagia/reflux from esophageal immobility
delayed gastric emptying
abdominal distention
pseudo-obstruction
MCC of death in scleroderma
pulmonary involvement - pulmonary fibrosis or HTN (diffuse form only)
renal involvement in scleroderma
in diffuse form only
- renal crisis w/ rapid malignant HTN
ab specific for limited form of scleroderma
anti-centromere ab
ab specific for diffuse form of scleroderma
anti-topoisomerase I (anti-Scl70)
findings in Barium swallow in scleroderma
absence of peristaltic waves in lower 1/3 of esophagus
decreased LES tone
what predicts prognosis in scleroderma?
degree of skin involvement - difffuse form has worse prognosis than limited form
CREST syndrome
Calcinosis of digits Raynaud's phenomenon Esophageal dysmotility Sclerodactyly Telengiectasias
Tx. of scleroderma
symptomatic
- MSK = NSAIDs
- Raynauds = CCB
- skin findings = D-penicillamine
- renal = ACEi
- GI = PPIs/H2 blockers
20% of pts with scleroderma have…
Sjogrens syndrome
typical findings in anti-phospholipid syndrome
recurrent venous/arterial thrombosis
recurrent pregnancy loss
thrombocytopenia
livedo reticularis
lab findings in antiphospholipid syndrome
presence of lupus anticoagulant and/or anticardiolipin antibody
prolonged PTT or PT that is not corrected by adding plasma
Tx of antiphospholipid syndrome
anticoagulation - INR 2.5-3.5
primary Sjogren’s syndrome
dry eyes and dry mouth, along w/ lymphocytic infiltration of minor salivary glands (histology)
secondary Sjogren’s syndrome
dry eyes and dry mouth along with a connective tissue disease
MCC of death in Sjogren’s
malignancy
- increased risk of non-Hodgkin’s lymphoma
what test has high sensitivity and specificity for Sjogren’s syndrome?
Schirmer test - filter paper inserted into eye to measure lacrimal gland output
most accurate test for dx Sjogren’s
salivary gland biopsy (lip or parotid)
- not needed for diagnosis
Tx. for Sjogrens
- pilocarpine or Cevimeline
- artificial tears
- NSAIDs/ steroids - arthritis
ab found in mixed CT disease
anti-U1-RNP ab
predom. cells implicated in RA
T helper cells - therefore, RA is not seen in pts with HIV/AIDs
which joints are characteristically NOT involved in RA?
DIP joints
what symptom is present in all patients?
morning stiffness
cutaneous finding that is pathognomic for RA
subcutaneous rheumatoid nodules (sacrum, elbows, occiput, achilles tendon)
pulmonary findings in RA
pleural effusions (low glucose)
pulmonary fibrosis
rheumatoid nodules in lung
cardiac findings in RA
rheumatoid nodules - conduction block
pericarditis
eye findings in RA
scleritis
scleromalacia - softening of sclera
dry eyes and mucous mbs
nervous system findings in RA
mononeuritic multiplex - damage to one or more peripheral nerves
Felty syndrome
triad of RA, splenomegaly and neutropenia
associated clinical findings in Felty’s syndrome
anemia, thrombocytopenia
LAD
recurrent infections
high titres of RF
type of anemia seen in RA
normocytic, normochromic anemia
what must you screen for in every pt with RA prior to undergoing surgery or intubation?
cervical radiograph of spine to assess for evidence of C1-C2 subluxation/instability
poor prognostic indicators in RA
high RF titres
subcutaneous nodules
autoantibodies to RF
erosive arthritis
lab findings in RF
high titres of RF - more severe disease
anticitrullinated peptide (ACPA)
elevated ESR/CRP
normocytic, normochromic anemia
what are pts with positive RF and ACPA at risk for? how should you intervene?
erosive joint damage
- early tx. with DMARDs indicated
criteria for dx. of RA
- inflammatory arthritis > 3 joints
- sx. lasting 6 weeks
- high ESR/CRP
- serum RF or ACPA positive
- radiographic changes
general tx. approach to RA
when pt presents with sx, start them on NSAIDs (steroids if NSAIDS not adequate relief); at the same time, start pt on DMARD therapy, taper NSAIDs/steroids after approx 6 weeks of tx and then use those PRN
first-line DMARD to start in RA
Methotrexate/Folate
side effects of Methotrexate
GI upset, oral ulcers mild alopecia bone marrow suppression hepatocellular injury pulmonary fibrosis
what should the patient have monitored while on Methotrexate therapy?
CBC every 2-3 months
LFTs
renal function
alternate first-line DMARD agents
leflunomide - same efficacy
hydroxychloroquine
sulfasalazine
when can you consider using anti-TNF inhibitors in RA?
if methotrexate cannot control disease - i.e. resistant RA
what can you do for a pt with severe RA with severe pain unresponsive to conservative measures?
joint replacement surgery
clear synovial fluid with < 200 WBCs and PMNs < 25%
normal
clear/yellow synovial fluid with < 2000 WBCs and < 25% PMNs
noninflammatory - OA/trauma
cloudy yellow synovial fluid with > 5000 cells and 50-70% PMNs
inflammatory arthritis
turbid, purulent synovial fluid with > 50 000 WBCs and > 75% PMNs
septic arthritis
what cell type plays key role in acute inflammation of gout?
PMN
radiographic changes in gout
punched out erosions with overhanging rim of cortical bone
- if no Hx of trauma, do not need XR for diagnosis
what happens if you do not treat an acute gouty attack?
nothing really…it lasts 7-10 days and resolves
pt comes in because of severe pain in his foot that caused him to awake from sleep; the joint appears erythematous, swollen and tender..what should you consider?
gout!!!
what is a tophi?
aggregations of urate crystals surrounded by giant cells in an inflammatory reaction; occurs after years of uncontrolled gout
common locations of tophi
extensor surfaces of forearms, elbows, knees
achilles tendon
pinna of ear
definitive diagnostic test in gout
joint aspiration and synovial fluid analysis
tx. of choice in acute gout
NSAIDs - esp. indomethacin
- avoid aspirin and acetaminophen
alternative drug in acute gout for pts who cannot take NSAIDs or did not respond to NSAIDs
colchicine
- 2nd line due to common GI side effects which result in low compliance
colchicine is contraindicated in..
renal insufficiency
cytopenia
when do you use oral steroids for acute gout?
if pt has no response to or cannot tolerate NSAIDs/colchicine
- 7-10 days of oral prednisone
what can probenicid or sulfinpyrazone be used for?
uricosuric drugs that increase renal excretion of uric acid; can use for gout prophylaxis in pts who are undersecretors
what are the uricosuric drugs contraindicated in?
if pt has history of renal stones
preferred drug for prophylaxis of gout
allopurinol - once daily dosing, 300 mg
what do you need to watch for when tx. pt with allopurinol?
Steven Johnson syndrome / rash
who can Febuxostat be Rx. to?
pts who are in need of gout prophylaxis but intolerant to allopurinol
what are serum uric acid levels useful for?
not used for diagnosis
- can monitor response to chronic gout tx.
RF of pseudogout (6)
age osteoarthritis hypothyroidism hemochromatosis hyperparathyroidism Bartter's syndrome
what can you see on radiograph in pseudogout?
chondrocalcinosis - linear radiodense deposits in dark joint space on XR
tx. of pseudogout
symptomatic tx. - same as gout
slowly progressive weaknes and wasting of both distal and proximal muscles (arms/legs), MC in men and associated with absence of autoab’s and low CK levels
inclusion body myositis
characteristic clinical feature of polymyositis/dermatomyositis (4)
symmetrical proximal muscle weakness
decreased muscle strength on testing
fine motor tasks usually preserved
myalgia and dysphagia common
which muscles are never involved in polymyositis/dermatomyositis?
ocular muscles
- ddx. w/ myasthenia and Lambert-Eaton
what features are unique to dermatomyositis?
Heliotrope rash
Gottron’s papules
V sign/Shawl sign
associated findings in both dermatomyositis and polymyositis
conduction defects/ CHF
arthralgias
interstitial lung disease - one of leading causes of death
associated findings in dermatomyositis only
vasculitis - in children
increased incidence of malignancy
what additional measures should you take with a dermatomyositis patient?
ensure patient undergoes age appropriate cancer screening
what is the first step in diagnosis in suspected dermatomyositis/polymyositis?
check aldolase and CPK levels - will be elevated
what lab test can be used to monitor disease severity in myositis?
CK level
nonspecific markers of cell injury in myositis
aldolase, LDH, AST, ALT
what kinds of antibdodies are found in the inflammatory myopathies? (3)
anti-Jo1
anti-signal recognition particle
anti-Mi-2
when do you do an EMG study in inflammatory myopathy?
when biopsy is inconclusive
- will show short duration and low amplitude
definitive diagnosis of inflammatory myopathy
muscle biopsy
muscle biopsy showing perivascular and perimysial inflammation and fibrosis
dermatomyositis
muscle biopsy showing endomysial inflammation and fibrosis
polymyositis
earliest and severely affected muscle groups in poly/dermatomyositis
neck flexors
shoulder girdle
pelvic girdle
initial tx. in inflammatory myopathy
corticosteroids - monitor with CPK levels; continue until symptoms improve
what additional CF is found in inclusion body myositis that is not seen with dermato/polymyositis?
loss of deep tendon reflexes - nerves are not affected in the others
older female patient presents with stiffness in shoulder and hip regions after a period of inactivity; upon testing, you notice that muslce strength is normal - dx?
polymylagia rheumatica
what other disease is polymyalgia rheumatica associated with?
temporal arteritis
how can you diagnose polymyalgia rheumatica?
clinical diagnosis
what lab finding correlates with disease activity in polymyalgia rheumatica?
ESR - usually > 100
tx. of polymyalgia rheumatica
corticosteroids - response usually w/in 1-7 days; disease is generally self-limiting
CF in fibromyalgia
stiffness/body aches all over body
fatigue
disturbed sleep
anxiety/depression
diagnostic criteria of fibromyalgia
- widespread pain including axial pain for atleast 3 months
2. pain in atleast 11/18 possible tender point sites
what drugs have shown beneficial in fibromyalgia?
SSRIs/TCAs
what is a prerequisite for the diagnosis of ankylosing spondylitis?
bilateral sacroilitis
young male comes in with low back pain and stiffness that is worse in the morning and gets better with activity; he also appears to have inflammation of the achilles tendon..what should you consider?
ankylosing spondylitis
aside from low back pain/stiffness, what else do pts with ankylosing spondylitis experience?
fractures with minimal trauma in late disease
chest pain and diminished chest expansion
constitutional symptoms
acute anterior uveitis/iridocyclitis
Schober test
measures spinal flexion - positive in ankylosing spondylitis
what is diagnostic of ankylosing spondylitis?
imaging showing sacroilitis or bamboo spine
- ESR may be elevated
- HLAb27 not needed
Tx. of ankylosing spondylitis (3)
NSAIDs - indomethacin (Sx)
Anti-TNF meds - for axial disease
Physical therapy
patient presents with asymmetric inflammatory oligoarthritis of LE 2 weeks after enteric or genitourinary infection
reactive arthritis
what should you consider if any patient has acute asymmetric arthritis that progresses sequentially from one joint to another
Reactive arthritis
patient presents with arthritis, urethritis and ocular inflammation - dx?
Reiters syndrome
undifferentiated spondyloarthropathy
when a patient has features of reactive arthritis but not evidence of previous infection
diagnosis of reactive arthritis
synovial fluid analysis
first line therapy for reactive arthritis
NSAIDs
what should you give a pt with reactive arthritis if NSAIDs do not provide relief?
try sulfasalazine or immunosuppressives (azathioprine)
should you treat patient with reactive arthritis with antibiotics?
usually not given - but may be beneficial in non-gonococcal urethritis (tetracycline)
pt presents with arthritis involving mainly the DIP joints; his nails also show pitting - what should you look for in this patient?
psoriasis - usually have skin disease for months-years before arthritis
finding on biopsy of temporal artery in GCA
giant cells; mononuclear cell infiltration or granulomatous inflammation
first line tx. of temporal arteritis
high dose steroids
- IV if visual loss is notes
- oral if no visual symptoms
young Asian woman presents to you with constitutional symptoms and pain/tenderness over vessels; on P/E you note decreased peripheral pulses, discrepancies of BP as well as arterial bruits
Takayasu arteritis
what is Takayasu arteritis?
granulomatous vasculitis of aortic arch and its major branches leading to stenosis or narrowing of vessels
how do you diagnose Takayasu arteritis?
arteriogram
Tx. of takayasu arteritis
steroids
treat HTN
surgery or angioplasty/bypass grafting - if severe
middle aged pt presents with new onset asthma and skin lesions (palpable purpura) - what should you consider?
Churg Strauss
definitive diagnosis of Churg Strauss
biopsy of lung/skin - prominence of eosinophils
positive p-ANCA
Tx. of Churg Strauss
steroids - prednisone
lab findings in Wegener’s granulomatosis
elevated ESR normochromic normocytic anemia hematuria positive C-ANCA thrombocytopenia
how can you confirm diagnosis of Wegeners?
open lung biopsy
- often biopsy of nasal septum is done (less invasive)
what Tx. is used to induce remission in Wegeners?
cyclophosphamide + corticosteroids
what drugs can be used in systemic resistant Wegener’s?
rituximab or IVIG AND
plasmaphoresis
CF in PAN
fever, weakness weight loss myalgias/arthralgias abdominal pain - bowel angina HTN mononeuritic multiplex - foot/wrist drop livedo reticularis
what should you consider in a diabetic with mononeuropathy (foot or wrist drop)?
PAN
which organ system is spared in PAN?
lungs
Dx. of PAN
nerve biopsy
mesenteric angiography
what can PAN be associated with?
hep B
HIV
drug reactions
tx. of PAN
start with steroids
- if severe, add cyclophosphamide
- tx. underlying HepB
pt comes in with recurrent oral and genital ulcerations, arthritis, uveitis, CNS involvement, fever and weight loss - dx?
Behcet’s syndrome
Tx. of Behcet’s syndrome
steroids
young smoker presents with cold, cyanotic distal extremities, paresthesias and noticeable ulcerations of digits - dx?
Buergers disease
young child develops palpable purpura on butt/legs, arthralgias and GI symptoms following an upper respiratory infection
Henoch-Schonlein purpura
Tx of Henoch-Schonlein purpura
usually self limited
- give steroids if severe
