Download Rheumatology Board Review

Rheumatology Board
Review Presentation - 1
Vikas Majithia, MD, MPH
Professor of Medicine,
Division Chief and
Fellowship Program
Director, Rheumatology.
Guarantees
If you listen and remember today:
>80% of Q of rheum in board
>90% correct
(Backed By Dinner at CHAR)
- Something special
Game-Plan
Stimulate your mind and think with me
Highlight the topics
Go over “educational objectives”
These are potential questions/test
pertinent areas
 Participate
 If you want write, Use the handout from
today otherwise just listen
 I can not teach you anything but I can
help you think/learn
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Questions – Technique
to Decipher
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OBJECTIVE
1 STEP
2 STEP
WHAT YOU SEE IS WHAT YOU GET
NO AMBIGUITY
SPECIFIC INFORMATION IN VIGNETTE
LIKELY ANSWERS (Confounding)
OVERVIEW/REVIEW
Q
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A.
B.
C.
D.
E.
A 46-year-old man is evaluated because of acute pain
and swelling in his right knee. He was recently diagnosed
with hypertension and started on hydrochlorothiazide, 25
mg/d. He smokes 1 pack of cigarettes daily, and
consumes 6 to 12 cans of beer on the weekends. On
physical examination, he weighs 99.8 kg (220 lb). His
temperature is 100.6 °F, pulse rate is 90/min, and blood
pressure is 180/90 mm Hg. There is effusion of the knee
along with surrounding redness and it is exquisitely
tender. He has no other systemic findings. An
arthrocentesis resulted in 30 cc of cloudy fluid.
Preliminary report shows needle shaped crystals which
are yellow when parallel to polarizer and a negative
gram stain.
What is the next best step in the management of his
condition?
Treat him with Allopurinol 300 mg/day.
Treat him with oral erythromycin 375 mg BID.
Treat him with a combination of colchicine and naproxen.
Do nothing as you have done a therapeutic
arthrocentesis.
Order an MRI of the knee to rule out meniscal tear
Q
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
A.
B.
C.
D.
E.
A 46-year-old man is evaluated because of acute pain
and swelling in his right knee. He was recently diagnosed
with hypertension and started on hydrochlorothiazide, 25
mg/d. He smokes 1 pack of cigarettes daily, and
consumes 6 to 12 cans of beer on the weekends. On
physical examination, he weighs 99.8 kg (220 lb). His
temperature is 100.6 °F, pulse rate is 90/min, and blood
pressure is 180/90 mm Hg. There is effusion of the knee
along with surrounding redness and it is exquisitely
tender. He has no other systemic findings. An
arthrocentesis resulted in 30 cc of cloudy fluid.
Preliminary report shows intracellular needle shaped
crystals which are yellow when parallel to polarizer and a
negative gram stain.
What is the next best step in the management of his
condition?
Treat him with Allopurinol 300 mg/day.
Treat him with oral erythromycin 375 mg BID.
Treat him with a combination of colchicine and naproxen.
Do nothing as you have done a therapeutic
arthrocentesis.
Order an MRI of the knee to rule out meniscal tear
Rheumatoid Arthritis
 Epidemiology
 Etiology: Role of genetic and environmental factors
(HLA-DR4).
 Pathology: Role of inflammation, cytokines, cells and
histopathology.
 Pathogenesis: Molecular mimicry, Role of infections.
 Clinical Manifestations: Articular and extra- articular
involvement, Pattern of involvement.
 Laboratory Findings: routine laboratory changes,
inflammatory markers, rheumatoid factor and anti-CCP
antibody.
 Radiographic Evaluation: Findings.
 Clinical course and prognosis: course, disability,
chronicity, effect on lifestyle, risk for family members.
Rheumatoid Arthritis
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Diagnosis: Mostly Clinical, Classification criteria helpful
Morning stiffness (at least 1 hour)
Arthritis of three or more joint areas
Arthritis of hand joints (PIP and MCP)
Symmetric arthritis, by area
Subcutaneous rheumatoid nodules
Positive test for rheumatoid factor
Radiographic changes (hand and wrist radiography that
show erosion of joints or unequivocal demineralization
around joints)
(First Three need to be present at least for 6 weeks)
RF: May be negative in up to 1/4 patients
Anti-Cyclic citrullinated peptide (anti-CCP)
antibody- specific not very sensitive
2010-ACR/EULAR Classification Criteria
Rheumatoid Arthritis
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Clinical picture:
Articular diseasePersistent symptoms with significant morning stiffness
Involvement of hands and feet (MCP, MTP, PIP) & sparing of DIP
Cervical spine disease- risk of subluxation and myelopathy
Extra-Articular disease
Rheumatoid nodules
Vasculitis
Pulmonary involvement: Effusion (low glucose) and Fibrosis
Felty’s syndrome: RA, Neutropenia and splenomegaly
Eye involvement- Scleritis and episcleritis (not uveitis)
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Laboratory abnormalities
Rheumatoid factor- negative in 25% cases
Anti-CCP antibody- specific not very sensitive
Elevated ESR and CRP
Bony erosions
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Long term risk of CAD
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Rheumatoid Arthritis
Management:
NSAIDs: Non-selective & Cox-2 inhibitors (do not
prevent joint damage)
DMARDs
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Antimalarials: Hydroxychloroquine and
chloroquine and Sulfasalazine - use in mild, non
erosive disease
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Steroids: Low dose, Short term therapy for major
flares, Bridge therapy, Vasculitis and Intraarticular injection
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Methotrexate: Preferred initial DMARD, Use of
folate replacement, risk of hepatitis, pneumonitis
and cytopenia
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Leflunomide: As effective as MTX, 2nd line due to
cost, No lung toxicity, risk of hepatitis, Category X
in pregnancy.
?? Azathioprine, Gold, D-penicillamine, cyclosporine
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Rheumatoid Arthritis
Management: BIOLOGICs
TNF blockers- Etanercept, Infliximab,
Adalimumab, Certolizumab pegol, Golimumab
B cell modulator: Rituximab
Co-stimulator blocker: Abatacept (Orencia)
IL-6 blocker- Tocilizumab (Actemra)
IL-1 blocker- Anakinra
JAK inhibitor- Tofacitinib
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Activation of latent TB
Increased risk of other infections
Possible increased risk of Malignancy,
demyelinating disease or other autoimmune
diseases, Lipid/Liver monitoring with IL-6
blocker
DMARD
Dosage
Onset of Effect
Adverse Events
Monitoring
Auranofin (Ridaura)
3-6 mg QD
4-6 months
Diarrhea
CBC, urinalysis (u/a) every 3
months
Azathioprine (Imuran)
50-150 mg QD
2-3 months
GI intolerance, cytopenia,
hepatitis infections,
CBC, LFTs every 2-4 weeks
initially, then every 2-3 months
Cyclosporine (Gengraf,
Neoral, generic)
2.5-5 mg QD
2-3 months
GI intolerance, cytopenia,
infections, hypertension, renal
disease
Creatinine (Cr) every two weeks
until dose is stable, then monthly;
consider CBC, LFTs, and K+ level
tests
D-Penicillamine
(Cuprimine)
250-750 mg QD
3-6 months
GI intolerance, skin rash,
proteinuria, Rare: cytopenia
CBC- u/a every 2 weeks until dose
is stable, then every 2-3 months
Hydroxychloroquine
(Plaquenil)
200-400 mg QD
2-6 months
GI intolerance, Retinal toxicity
Eye examinations every 12 months
IM Gold sodium thiomalate
(Myochrysine) Aurothioglucose
(Solganal)
25-50 mg IM
every 2-4 weeks
6-8 weeks
GI intolerance, skin rash, oral
ulcers, proteinuria, cytopenia,
CBC and u/a every 2 weeks until
dose is stable, then with each
injection
Leflunomide (Arava)
20 mg QD
4-12 weeks
GI intolerance, skin rash,
hepatitis, cytopenia, highly
teratogenic
Hepatitis B and C serology in highrisk patients; CBC, Cr and LFTs
monthly for 6 months, then 1-2
months; adjust dose or stop if LFTs
elevated
Methotrexate
15-25 mg orally,
sc or IM every
week
1-2 months
GI intolerance, oral ulcers,
alopecia, hepatitis, pneumonitis,
cytopenia, rash, teratogenic
CBC, Cr and LFTs monthly for 6
months, then every 1-2 months;
adjust dose or stop if LFTs elevated
Minocycline (Minocin)
100 mg BID
1-3 months
Dizziness, skin pigmentation
none
Sulfasalazine (Azulfidine)
2-3 gm/day
1-3 months
GI intolerance, oral ulcers,
cytopenia, rash
CBC every 2-3 months
Biologic Drugs
Dosage
Onset of Effect
Adverse Events
Monitoring
Adalimumab (Humira)
40 mg sc EOW
2-12 weeks
Injection site reaction (ISR),
infection risk, TB reactivation
Rare: demyelinating disorders
Monitor for TB, fungal and
other infections; CBC and
Liver panel (LFTs) at
baseline and monthly;
thereafter every 2-3 months
Etanercept (Enbrel)
25 mg sc twice/
week or 50 mg
sc weekly
2-12 weeks
ISR, infection risk, TB
reactivation
Rare: demyelinating disorders
Monitor for TB, fungal and
other infections; CBC and
LFTs at baseline and
monthly; thereafter every 23 months.
Certolizumab- Cimzia
200/400 mg sc
EOW/monthly
2-12 weeks
Injection site reaction (ISR),
infection risk, TB reactivation
Rare: demyelinating disorders
Monitor for TB, fungal and
other infections; CBC and
Liver panel (LFTs) at
baseline and monthly;
thereafter every 2-3 months
Golumimab (Simponi)
50 mg sc
monthly
2-12 weeks
ISR, infection risk, TB
reactivation
Rare: demyelinating disorders
Monitor for TB, fungal and
other infections; CBC and
LFTs at baseline and
monthly; thereafter every 23 months.
Infliximab (Remicade)
3 mg/kg at
weeks 0, 2 and
4 weeks, then
every 8 weeks
2-12 weeks
Infusion reactions, infection
risk, TB reactivation
Rare: demyelinating disorders
Monitor-TB, fungal and
other infections; CBC, Cr
and LFTs at baseline and
monthly; thereafter every 23 months.
TNF Blockers
Drug
Dosage
Onset of Effect
Adverse Events
Monitoring
Anakinra (Kineret) – IL -1 blocker
100 mg sc QD
4-12 weeks
ISR, leucopenia, Infections,
Hypersensitivity
CBC at baseline and every 3
months
Abatacept(Orencia) – CTLA 4 Ig,
Costimulator blockade
500-1000 mg
(weight based)
at 0, 2 and 4
weeks then
every 4 weeks
2-12 weeks
Infusion reactions, infection
risk, sepsis, hypersensitivity,
COPD exacerbation
Monitor for TB, other
infections; CBC and
chemistry and LFTs at
baseline and with each
infusion
Rituximab (Rituxan)- B-cell inhibitor
1000 mg at 0
and 15 days
2-12 weeks
Infusion reactions, infection
risk, reactivation and new
viral infections, respiratory
difficulty, cytopenia
Monitor for TB, other
infections; CBC and
chemistry and LFTs at
baseline and 2 weeks;
thereafter every 2-3 months
Tocilizumab (Actemra)- IL-6 blocker
4 mg/kg or 8
mg/kg (weight
based) every 4
weeks
2-12 weeks
Infusion reactions, infection
risk, sepsis, cytopenia,
hepatitis, hypersensitivity,
Lipid abnormalities, GI
perforation
Monitor for TB, other
infections; CBC and
chemistry and LFTs at
baseline and with each
infusion
8-12 weeks
Infection risk, sepsis,
cytopenias, hepatitis,
hypersensitivity, GI
perforation
Monitor for TB, other
infections; CBC and
chemistry and LFTs at
baseline and periodically.
Other Biologics
Tofacitinib (XelJanz) – JAK Kinase inhibitor Oral daily- 5
mg or 10 mg
BID
Rheumatoid Arthritis
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Questions asked
1. Patient scenario: middle aged female,
outpatient
2. Comes with polyarthritis, failed
NSAIDs. What to do next?
3. Inadequate response to
MTX/plaquenil
4. What test to do before starting a
biologic agent
5. Patient on MTX comes with cough.
What to do next?
6. RA Patient (on/not on) MTX/biologic
agent comes with single joint
pain/swelling. What to do next?
Rheumatoid Arthritis
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Questions asked
7. Patient on leflunomide. Wants to
become pregnant.
8. RA Patient on MTX/biologic agent
comes with cough, weight loss. What to
do next?
9. RA Patient has unilateral calf
swelling? Likely diagnosis
10. RA patient going for surgery? What
test to do?
11. RA patient with paraesthesia,
dizziness. What test to do?
12. RA patient with replaced hip/knee?
Come with pain in the prosthetic joint.
What to do next?
Rheumatoid Arthritis
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Questions asked
13. RA patient on plaquenil, presents with blurred vision.
Likely cause: retinal toxicity.
14. Long-standing RA Patient on treatment presents with
fever, weight loss, SOB, distended neck veins. What to do
next?
15. Long-standing RA Patient on treatment presents with
intermittent fever, weight loss, diarrhoea, What to do
next?
16. Long-standing RA Patient on treatment presents with
fever, weight loss, skin ulcers, foot drop. What to do
next?
17. RA patient on MTX asks risk of liver toxicity and
monitoring
18. Patient with intermittent migratory pain swelling.
Likely diagnosis?
19. long-standing RA Patient with early satiety, weight
loss, skin ulcers, LUQ fullness. What to do next?
20. Young male patient with fever, evanescent rash, sore
throat, arthralgia, lymphadenopathy and
hepatosplenomegaly. Typically –ve RF
Q
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A 52-year-old woman who has had rheumatoid arthritis for two years comes to your
office for a scheduled health evaluation. She reports that she feels well and has
minimal morning stiffness. Current medications are leflunomide, 20 mg daily, and
ibandronate 150 mg monthly.
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Physical examination reveals swelling of the second and fifth right MCPs and the third
left MCP; and swelling of the first and fourth right PIPs and the second left PIP with
minimal tenderness. The right wrist and left knee are slightly swollen, and range of
motion (ROM) is slightly painful. Tenderness to palpation of the MTPs is noted.
Radiographs of the feet obtained one year ago show erosions of the fourth and fifth
right MTPs and the fourth left MTP.
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Laboratory studies: Hematocrit 11.1% , Hemoglobin 33 g/dL, Erythrocyte
sedimentation rate 31 mm/hr, Liver function tests Normal, Disease activity score shows
moderate activity.
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Which of the following is the appropriate management at this time?
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(A) No further change in therapy
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(B) Add etanercept, 50 mg weekly
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(C) Add prednisone, 10 mg daily
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(D) Add hydroxychloroquine, 200 mg daily
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(E) Discontinue leflunomide; add methotrexate, 20 mg weekly
Crystal Induced Arthritis
 Epidemiology
 Crystals: Gout (monosodium urate), Psudogout (Ca
pyrophosphate), Basic calcium phosphate
 Etiology: Role of metabolic factors (overproduction or
underexcretion of uric acid) and genetic associations.
 Crystal analysis: shape, birefringence and color
 Clinical Manifestations: Articular and extra- articular
involvement, Pattern of involvement
 Laboratory Findings: Hyperuricemia (suggestive not
diagnostic)
 Radiographic Evaluation: Findings
 Clinical course and prognosis
Synovial Fluid Analysis
Normal
Inflammatory
Septic
Hemorrha
gic
Crystal
Induced
Clear/Yellow
Transparent
Yellow/ White
Translucent/
opaque
Yellow/White
Opaque
Red
Opaque
Yellow/
White,
Translucent
/opaque
<2K
2K-50K
>50K
N/a
10K-50K
+/- Gm stain, +/Culture
-ve Gm
stain, Cx.
-ve Gm
stain, Cx.
+ Crystals
-ve Gm stain -ve Gm stain &
& Culture (Cx) Culture
Gout
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Clinical picture:
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Usually adult men, If occurs in adolescentunderlying enzyme disorders, Rare in premenopausal women
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Articular disease-
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Acute gouty arthritis
Intercritical (or interval) gout
Chronic tophaceous gout
Intermittent, recurrent attacks
Involvement of 1st MTP is typical, Any joint can
be involved.
Spine disease: Can involve any level
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Gout
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Clinical picture:
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Extra-Articular disease
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Tophi
Renal disease: Uric acid nephrolithiasis and
interstitial nephropathy
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Laboratory abnormalities
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Hyperuricemia: helpful not diagnostic
Crystal analysis: negatively birefringence,
needle shaped, yellow when parallel
a↑
b→
Gout
 Unusual presentations:
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Poly-articular gout
Transplantation gout:
Typically patient on cyclosporine
Rapid progression of gout
Common in cardiac transplantation
Moonshine gout (saturnine gout)
Gout
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Treatment:
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Acute Attacks
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NSAIDs: Any NSAID works fine ( Indomethacin
is NOT the preferred agent)
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Colchicine: remember colchicine associated GI
events and neuro-myopathy
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Steroids: Systemic and Intraarticular
Gout
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Treatment:
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Management of Hyperuricemia:
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Allopurinol: Remember hypersensitivity syndrome
and dose adjustment in renal disease, prophylaxis
with colchicine initially to avoid acute attack
Uloric/Febuxostat (XO blocker). Lower risk of
hypersenstivity. No dose adjustment in renal Dz
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Uricosuric agents: probenecid and sulfinpyrazone
(Ineffective in Renal insufficiency, do not use in
patients with renal stones)
Pegloticase (Krystexxa -recombinant porcine like
uricase)
Gout
 Questions asked
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Patient scenario: middle aged male,
outpatient, acute illness inpatient:
Post MI, post surgical, ESRD
Read the Q- Scenarios-acute attack,
asymptomatic, chronic state
Role of 24 hour urine collection.
Patient with sudden swollen single
joint. What to do?
Aspiration reveals crystals: findings
diagnostic of an acute attack
Co-existent infection with the
crystals: Gm stain
Gout
 Questions asked
1. Patient with HTN and gout. What
medication he is likely on? What to
do?
2. Treatment of acute attack. Choices?
3. Long term management when
asymptomatic?
4. When to use - steroids in treatment
of an acute attack?
5. Treatment of tophaceous gout?
6. Allopurinol dose adjustment in
renal insufficiency?
Gout
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Questions asked
7. Allopurinol/Feboxustat-in a transplant
patient? Interaction with what drug?
What to substitute?
8. Patient with heme malignancy planning
chemo. What drug used for prophylaxis?
9. Patient on Allopurinol comes with skin
rash, cough? What test to do?
Management?
10. Drug of choice in patient with renal
stones, renal insufficiency?
11. Not to start what drug during an acute
attack?
Q
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A 60-year-old man is referred to you for management of gouty arthritis. Episodic attacks of
acute arthritis involving the feet and ankles began nine years ago and were confirmed by
urate crystal identification. Initially, the attacks responded to nonsteroidal anti-inflammatory
drugs, but symptoms became more frequent and are now chronic.
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Medical history includes hypertension, non–insulin-dependent diabetes mellitus, and
coronary artery disease. For three years the patient has taken colchicine, 0.6 mg every two
days; and allopurinol, 300 mg daily. Other current medications are furosemide, 20 mg daily;
and amlodipine, 10 mg daily.
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Laboratory studies: Hematocrit 34% Hemoglobin 11.6 g/dL, Platelet count 504,000/cu mm
Erythrocyte sedimentation rate 60 mm/hr, Blood urea nitrogen 45 mg/dL Serum creatinine
2.5 mg/dL Serum potassium 5.1 mEq/L Serum uric acid 8.5 mg/dL Serum C-reactive protein
3.8 mg/dL Creatinine clearance 25 mL/min
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Physical examination reveals blood pressure of 128/72 mm Hg. The ankles and feet are
minimally swollen. Tophaceous deposits are present in the outer helix of the right ear and
left olecranon bursa.
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Which of the following is the best treatment now to manage this patient’s gout?
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(A) Add fenofibrate, 600 mg twice daily, to the current drug regimen
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(B) Add probenecid, 500 mg twice daily, to the current drug regimen
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(C) Change allopurinol to oxypurinol, 100 mg daily
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(D) Increase allopurinol to 400 mg daily
CPPD
 5 patterns:
 pseudogout, pseudo-rheumatoid, pseudo-OA, pseudoAS, Pseudo-neuroarthritis.
 Involves larger joints: typically knee.
 Positively birefringent, rhomboid shaped crystals, bluish
when parallel.
CPPD
 Typical Presentations:
 Acute inflammation of knee in elderly.
 Post-op acute synovitis in elderly
 OA in unusual sites
 Associations with CPPD: 4 H – hypophosphatesia,
hypomagnesimia, hemochromatosis, hyperpathyroidism
 Treatment: NSAIDs, Intra-articular steroids, No long
term metabolic management
Basic Calcium Phosphate
disease
Patterns
 Acute calcific-periarthritis, Tendonitis, bursitis
 Idiopathic destructive arthritis of shoulder
(Milwaukee shoulder): typically elderly women
with bilateral pain, swelling and loss of joint
function, large effusion with severe destruction
on radiograph.
Crystals: appear as clumps, Alizarin red S
staining.
Treatment: NSAIDs, Intra-articular steroids, No
long term metabolic management
Crystal Arthritis
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Questions asked
1.
Patient scenario: elderly male, multiple medical
problems, outpatient, acute inpatient
Abnormal Xray: CPPD deposition, crystal
analysis.
55 year old patient with DM, episodic pain in
wrist, knees, fatigue and decreased libido. Next
test? Likely diagnosis?
Treatment of acute attack. Choices?
Long term management ?
Patient with severe R shoulder pain. Xrays
show destruction. Likely diagnosis? Differential
diagnosis?
2.
3.
4.
5.
6.
Autoantibodies
SLE
 Epidemiology
 Etiology: Role of genetic and environmental factors. DR3
association.
 Pathology: Role of B-cells and autoantibodies.
 Clinical Manifestations: Systemic manifestations with
commonly involved organs, types of skin involvement
(discoid, sub-acute cutaneous and others), renal
involvement and types, articular involvement.
 Laboratory Findings: Laboratory Findings: routine
laboratory changes, inflammatory markers, autoantibody
profiles, evaluation of pro-coagulation factors.
 Clinical course and prognosis: course, morbidity and
mortality, prognosis, risk for family members.
SLE
 MNEMONIC DOPAMIN RASH
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DISCOID RASH
ORAL ULCERS
PHOTOSENSITIVITY
ANA
MALAR RASH
IMMUNOLOGICAL PHENOMENON(Le Cell, Ds DNA, Sm, APL ab,
false + RPR, )
NEUROLOGICAL(Seizures,Psychosis)
RENAL(Nephrotic,Nephritic)
ARTHRITIS
SEROSITIS
HEMATOLOGICAL (Leukopenia, Thrombocytopenia, anemia)
 4 or more positive criteria
Malar Rash in SLE
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Discoid lupus erythematosus
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SLE
 Clinical picture:
 Articular disease - Non erosive, diffuse, +morning
stiffness, Involvement of hands and feet.
 RASH: malar eruption, discoid rash, sub-acute
cutaneous LE.
 Cardiopulmonary: pleuritis/effusion, pericarditis,
also remember pneumonitis and alveolar
hemorrhage can occur.
 Renal: nephritis or nephrosis ( active urine
sediment / proteinuria), 6 different pathological
classes upon biopsy.
 CNS: Seizures or psychosis, vasculitis/ cereberitis
can occur
 Hematological: (Leukopenia, ITP, hemolytic )
SLE
 Clinical picture:
Immunological studies:
 ANA + in high titer (100 %), Anti-Ds
DNA (60-70%), Anti Smith (30 %).
 Hypo-complementemia- low c3, c4.
 High ESR
SLE
 Management:
 NSAIDs:
 Used for a number of manifestations, (remember elevated
creatinine can occur due to their use too in addition to the
disease itself)
 Steroids:
 Initially higher dose with taper to low dose maintenance,
Short term therapy for major flares.
 IV pulse therapy – initial and for flares (?intermittent)
 Remain very important for management.
 Highlight risks and adverse events
SLE
DMARDs
 Antimalarials: Hydroxychloroquine and chloroquine helpful in all mild disease, adjunct in moderate/severe
disease.
 Azathioprine: Preferred immunosuppressive in
moderate/severe disease, monitor for cytopenia and
hepatitis.
 Mycophenolate: Increasingly popular especially in renal
disease.
 Methotrexate: used in arthritis.
 Cyclophosphamide: IV preferred over oral, Used in nephritis
and CNS disease, Also useful in any severe/life-threatening
manifestation. ( remember risk of hemorrhagic cystitis and
bladder cancer, urine monitoring for this).
Biologics
 Belumimab: Anti-Blys, useful in mild to moderate disease in
combination with DMARDs, IV monthly. No additional
evaluation/monitoring. Risk of infection, TB, cytopenias,
PML?
SLE- special Circumstances
 Drug-induced LE
 Common drug associations: procainamide, hydralazine,
isoniazid, PTU and minocycline.
 Positive anti-histone specificity
 Neonatal LE
 Transient rash, antibodies disappear by 6 months
 Associated with positive SS-A and SS-B
 Congenital heart block
 Associated with positive SS-A and SS-B too
 Risk is less than 5%
 Monitor for fetal heart rate around 18-24 weeks
 Can be treated with dexamethasone/betamethasone if
detected early.
SLE

Questions asked
1. Patient scenario: middle aged female, AA,
both outpatient and inpatient
2. Lupus patient with psychosis, No other
active symptoms, signs. Likely cause?
3. Patient presents with rash, polyarthritis,
recently on plaquenil. What to do next?
4. Lupus patient with abnormal urine. Next
diagnostic test?
5. Biopsy shows Class IV disease. Appropriate
treatment?
6. Lupus patient (longer duration) presents
with chest pain. Next test? Likely diagnosis?
Cause of death?
SLE
 Questions asked
7. SLE patient now pregnant: what
drugs to stop/continue?
8. Lupus pregnancy: monitor for-----?
9. SLE patient presents with hip pain:
diagnostic test of choice? Diagnosis?
10. Most specific autoantibody with SLE.
11. Female patient with cough, hemoptysis,
+ve ANA. Likely diagnosis?
12. Drug induced lupus.
SLE

Questions asked
13. Patient with ITP also has arthralgia,
fever. Next test?
14. Patient with +ve ANA, fatigue presents
with weakness of lower extremities, BB
incontinence. Next Test? Diagnosis?
15. Lupus nephritis patient treated with
cyclophophamide. Presents with
hematuria (+ UA). Cause & Next test?
16. SLE patient presents with high fever,
SOB, changing heart murmur. Diagnosis?
17. SLE patient with shortness of breath and
diffuse reticular infiltrates on chest
Xray/CT. Diagnosis?
Q
 A 43-year-old woman is evaluated for increasing headaches and memory
problems. She has had systemic lupus erythematosus (SLE) for ten years.
Currently, she has a moderately active level of SLE with malar rash,
arthritis, proteinuria, and serum creatinine of 1.8 mg/dL. Her medical
history includes lupus nephritis for which she was treated with prednisone
and mycophenolate mofetil.
 She has multiple medication allergies including sulfa, penicillin,
hydrochlorothiazide, and angiotensin-converting enzyme inhibitors.
Magnetic resonance imaging (MRI) of the brain is ordered to assess the
patient’s neurologic symptoms. Later in the day, the neuroradiologist
requests that MRI be performed without contrast.
 For which of the following complications is the patient at risk if she has an
MRI with contrast?
 (A) Renal failure
 (B) Contrast allergic reaction
 (C) Hypotension
 (D) Nephrogenic systemic fibrosis
55
Sjogren’s syndrome
Classification criteria:
 Ocular symptoms: dry eyes
 Oral symptoms: Xerostomia
 Abnormal Schirmer's test. Five millimeters
of unstimulated wetting of filter paper in 5
minutes
 Rose bengal staining of the cornea
 Abnormal parotid salivary flow rate
 Abnormal radio nuclide scan of the parotid
glands
 Abnormal parotid gland sialography
 Abnormal Biopsy of minor salivary glands
(usually lip biopsy).
 Positive SS-A and SS-B
Sjogren’s syndrome
Systemic manifestations:






Articular disease
Hyperglobulinemia
Leukocytoclastic vasculitis
Peripheral neuropathy/ MS like illness
Cryoglobulinemia
Risk of lymphoma
Treatment:
 Careful management of oral and ocular symptoms
 NSAIDS and Plaquenil
Antiphospholipid Antibody
syndrome
 Classification criteria:
 Definite APS = 1 clinical + 1 laboratory criteria
 Clinical —

1 or more episodes of venous, arterial, or small vessel thrombosis and/or

Pregnancy morbidity — 1. Otherwise unexplained death at 10 weeks gestation of a
morphologically normal fetus, or 2. One or more premature births before 34 weeks
gestation because of eclampsia, preeclampsia, or placental insufficiency, or 3. Three
or more embryonic (<10 week gestation) unexplained pregnancy losses.

Laboratory — The presence of aPL , on two or more occasions at least 12 weeks

Treatment:

Lifelong anticoagulation goal of INR is close to 3 or higher

Use heparin or LMW heparin during pregnancy to avoid morbidity
associated with it

Plaquenil may be helpful
apart and no more than five years prior to clinical manifestations, as demonstrated
by one or more of the following - 1. Positive IgG and/or IgM anticardiolipin antibody
2. Positive Antibodies to ß2-glycoprotein I of IgG or IgM isotype 3. Positive lupus
anticoagulant
Catastrophic Anti-phospholipid
Antibody syndrome
-Associated with
high morbidity and
mortality
-Treatment requires
anticoagulation and
Possibly
plasmapharesis,
IVIG, IV
cyclophosphamide
SLE and related syndromes
Questions asked.
1.
Female patient with fatigue, arthralgia, dry
eyes, dry mouth. What test to order next?
Likely diagnosis? Confirmatory test?
2. Patient with Sjogren’s presents with fever,
weight loss, LN +. What to do next?
3. Symptomatic treatment of dry eyes, dry mouth.
4. Male patient with parotid swelling, Biopsy CD 8+
lymphocytic infiltrate. Test? Diagnosis?
5. Pregnant patient has fetal bradycardia- CHB,
what test to do & RX?
6. Pregnant patient with 2nd or later pregnancy loss.
what test to do?
7. Patient with APLS on coumadin now wants to be
pregnant. What is appropriate therapy?
Systemic Sclerosis and related
syndromes
Systemic Sclerosis
One major criterion or two or more minor criteria
were found to be 97% sensitive & 98% specific.
Major criterion:
Proximal scleroderma (skin thickening) is the single
major criterion (proximal to MCPs).
Minor Criteria:
Sclerodactyly,
Digital pitting scars of fingertips or loss of
substance of the finger pad,
Bilateral basilar pulmonary fibrosis.
Systemic Sclerosis
 Clinical picture:
 Skin: Proximal skin thickening ( above wrists and below
neck)
 Articular disease - Non erosive, diffuse
 Vascular: Raynaud’s – severe, pitting, ulcerations and
necrosis. Nailfold capillary abnormalities
 Pulmonary: ILD and Fibrosis with abnormal CT scan and
restrictive pattern on PFT
 Cardiac: conduction system fibrosis with arrhythmias
 Renal: Remember SCLERODERMA RENAL CRISIS and its
features ( rapid rise in creatinine, severe HTN and bland
urine sediment, onion skinning on pathology)
 GI System: esophageal dysmotility, intestinal dysmotility
and wide mouth diverticulae.
 Immunological studies:
 ANA +ve (70-80%), Anti SCL-70 +ve (30-60%).
Sclerodactyly
©Copyright Science Press Internet Services
Limited Systemic Sclerosis
 Limited skin involvement: Distal to MCPs
(still systemic disease-organs involved)
 Non-CREST syndrome
 CREST syndrome: association with anticentromere antibody.
 Calcinosis
 Raynaud’s phenomemnon
 Esophageal dysmotility
 Sclerodactyly
 Telengeactasias
Mat-like telangiectasia
©Copyright Science Press Internet Services
Systemic Sclerosis
 Management:
 Avoid high dose steroids ( > 15 mg)
unless absolutely needed
Organ based therapy:
 Raynaud’s phenomenon: Lifestyle
changes, Calcium channel blockers,
Sympathectomy, endothelin-1 blockers
 Renal disease: ACE inhibitors (remember
continue despite early elevation in
creatinine during renal crisis)
 GI disease: PPIs, Prokinetic agents, Flagyl
as needed
 Arthritis: Methotrexate
Systemic Sclerosis
 Management:
 Specific Therapy:
 Alveolitis: Corticosteroids, IV
Cyclophosphamide, Azathioprine.
 Pulmonary Hypertension:
 Endothelin-1 receptor antagonists:
Bosentan, Sitaxsentan
 Prostacyclin analogues: Epoprostenol,
Treprostinil, iloprost – I.V. and inhaled
 Phosphodiesterase type 5 inhibitors:
Sildenafil-Revatio
Lung Transplantation
Systemic Sclerosis and related
syndromes
 Questions asked
1. Patient scenario: middle aged female
2. Patient presents with skin thickening,
cough, + ANA. Work-up should include?
Antibody? Likely diagnosis?
3. Pulmonary findings
4. Patient with scleroderma, presents with
HTN. Drug of choice?
5. Same patient comes with severe HTN,
MS changes and renal failure. Diagnosis?
Treatment?
Systemic Sclerosis and related
syndromes
 Questions asked
6. Primary Raynaud’s vs Secondary
7. Patient with Raynaud’s, skin thickening,
GERD. Antibody? Also has syncope and
SOB. What test? Diagnosis? Treatment?
8. Patient with Raynaud’s. Treatment?
Lifestyle changes?
9. Raynaud’s patient asks about long term
prognosis? Findings on
examination/office based testing
associated with a worse prognosis?
Systemic Sclerosis and related
syndromes

Questions asked
10. SS patient with weight loss, bloating, BM
irregularity, foul smelling stools. Diagnosis?
Findings on a radiographic test?
11. SS patient with abnormal PFT. What is the
worse prognosis finding?
12. Pulmonary HTN: treatment.
13. Raynaud’s+arthritis+myositis= what overlap
syndrome? Associated antibody? Typical
pulmonary finding?
14. Male patient with swollen arms, legs, orange
peel appearance. Next test? Diagnosis?
15. Patient with DM- swelling/skin thickening on
the back. Diagnosis?
Q

A 35-year-old woman has been under your care for nine months for systemic sclerosis
characterized by Raynaud’s phenomenon, sclerodactyly, esophageal reflux,
hypopigmentation of the upper back and chest, and arthralgias of the elbows and hands.
One month ago, physical examination showed pulse rate was 70 per minute and blood
pressure was 114/70 mm Hg. Diffuse subcutaneous thickening of the skin on the trunk and
extremities was noted, as well as a small ulcer over right index finger pulp. Nailfold
capillaroscopy showed dilated peripheral capillary loops. All laboratory studies were
normal. She now presents for a follow-up visit, and reports malaise and fatigue. Blood
pressure is 138/88 mm Hg, but results of her physical examination are otherwise
unchanged from those of her previous visit.

Laboratory studies: Hemoglobin 10.2 g/dL, Leukocyte count 7500/cu mm, Platelet count
105,000/cu mm, Blood urea nitrogen 12 mg/dL, Serum creatinine 0.9 mg/dL, Peripheral
blood film RBC fragmentation; helmet cells; basophilic stippling

Direct (Coombs’ test) Negative, VWF protein Normal, U/A Trace protein; 0–2 RBC/hpf

Which of the following complications is most likely to occur in this patient now?

A. Cerebral thrombosis

B. Macrophage activation syndrome

C. Pulmonary alveolar hemorrhage

D. Pulmonary hypertension

E. Renal crisis