Rheumatology Board
Review Presentation-1

Guarantees
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Game-Plan
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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

 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?
A.
B.
C.
D.
E.
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

 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?
A.
B.
C.
D.
E.
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
 Diagnosis: Mostly Clinical, Classification criteria helpful
 Morning stiffness (at least 1 hour)
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Arthritis of three or more joint areas
Arthritis of hand joints (PIP and MCP)
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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) antibodyspecific not very sensitive

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Rheumatoid Arthritis
Clinical picture:
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 Articular disease-
 Persistent symptoms with significant morning stiffness
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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)
 Laboratory abnormalities
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Rheumatoid factor- negative in 25% cases
Anti-CCP antibody- specific not very sensitive
Elevated ESR and CRP
 Bony erosions
 Long term risk of CAD

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Rheumatoid Arthritis
 Management:
 NSAIDs: Non-selective & Cox-2 inhibitors (do not prevent joint damage)
 DMARDs
 Antimalarials: Hydroxychloroquine and chloroquine and Sulfasalazine - use in mild, non erosive disease
 Steroids: Low dose, Short term therapy for major flares, Bridge therapy, Vasculitis and Intra-articular injection
 Methotrexate: Preferred initial DMARD, Use of folate replacement, risk of hepatitis, pneumonitis and cytopenia
 Leflunomide: As effective as MTX, 2 nd line due to cost,
No lung toxicity, risk of hepatitis, Category X in pregnancy.
 Others — Azathioprine, Gold, D-penicillamine, cyclosporine

Rheumatoid Arthritis
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
 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
Auranofin (Ridaura)
Azathioprine (Imuran)
Cyclosporine (Gengraf,
Neoral, generic)
Methotrexate
Minocycline (Minocin)
Sulfasalazine (Azulfidine)
Dosage
3-6 mg QD
50-150 mg QD
2.5-5 mg QD
Onset of
Effect
4-6 months
2-3 months
2-3 months
D-Penicillamine
(Cuprimine)
Hydroxychloroquine
(Plaquenil)
IM Gold sodium thiomalate
(Myochrysine) Aurothioglucose
(Solganal)
Leflunomide (Arava)
250-750 mg QD 3-6 months
200-400 mg QD 2-6 months
25-50 mg IM every 2-4 weeks
20 mg QD
6-8 weeks
4-12 weeks
15-25 mg orally, sc or IM every week
100 mg BID
2-3 gm/day
1-2 months
1-3 months
1-3 months
Adverse Events
Diarrhea
Monitoring
CBC, urinalysis (u/a) every 3 months
CBC, LFTs every 2-4 weeks initially, then every 2-3 months
GI intolerance, cytopenia, hepatitis infections,
GI intolerance, cytopenia, infections, hypertension, renal disease
GI intolerance, skin rash, proteinuria, Rare: cytopenia
Creatinine (Cr) every two weeks until dose is stable, then monthly; consider CBC, LFTs, and K+ level tests
CBC- u/a every 2 weeks until dose is stable, then every 2-3 months
GI intolerance, Retinal toxicity Eye examinations every 12 months
GI intolerance, skin rash, oral ulcers, proteinuria, cytopenia,
GI intolerance, skin rash, hepatitis, cytopenia, highly teratogenic
CBC and u/a every 2 weeks until dose is stable, then with each injection
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
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
Dizziness, skin pigmentation
GI intolerance, oral ulcers, cytopenia, rash none
CBC every 2-3 months

Biologic Drugs
TNF Blockers
Adalimumab (Humira)
Etanercept (Enbrel)
Certolizumab- Cimzia
Golumimab (Simponi)
Infliximab (Remicade)
Dosage Onset of
Effect
40 mg sc EOW 2-12 weeks
Adverse Events
25 mg sc twice/ week or 50 mg sc weekly
200/400 mg sc
EOW/monthly
50 mg sc monthly
3 mg/kg at weeks 0, 2 and
4 weeks, then every 8 weeks
2-12 weeks
2-12 weeks
2-12 weeks
2-12 weeks
Monitoring
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
ISR, infection risk, TB reactivation
Rare: demyelinating disorders
Monitor for TB, fungal and other infections; CBC and
LFTs at baseline and monthly; thereafter every 2-
3 months.
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
ISR, infection risk, TB reactivation
Rare: demyelinating disorders
Monitor for TB, fungal and other infections; CBC and
LFTs at baseline and monthly; thereafter every 2-
3 months.
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 2-
3 months.

Drug
Other Biologics
Anakinra (Kineret) – IL -1 blocker
Abatacept(Orencia) – CTLA 4 Ig,
Costimulator blockade
Rituximab (Rituxan)- B-cell inhibitor
Tocilizumab (Actemra)- IL-6 blocker
Dosage
100 mg sc QD
Onset of
Effect
4-12 weeks
500-1000 mg
(weight based) at 0, 2 and 4 weeks then every 4 weeks
2-12 weeks
1000 mg at 0 and 15 days
2-12 weeks
4 mg/kg or 8 mg/kg (weight based) every 4 weeks
2-12 weeks
Tofacitinib (XelJanz) – JAK Kinase inhibitor Oral daily- 5 mg or 10 mg
BID
8-12 weeks
Adverse Events Monitoring
ISR, leucopenia, Infections,
Hypersensitivity
CBC at baseline and every 3 months
Infusion reactions, infection risk, sepsis, hypersensitivity,
COPD exacerbation
Monitor for TB, other infections; CBC and chemistry and LFTs at baseline and with each infusion
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
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
Infection risk, sepsis, cytopenias, hepatitis, hypersensitivity, GI perforation
Monitor for TB, other infections; CBC and chemistry and LFTs at baseline and periodically.

Rheumatoid Arthritis
 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
 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
 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
 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.
 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?
 (A) No further change in therapy
 (B) Add etanercept, 50 mg weekly
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(C) Add prednisone, 10 mg daily
 (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
Clear/Yellow
Transparent
<2K
Inflammatory
Yellow/ White
Translucent/ opaque
2K-50K
Septic
Yellow/White
Opaque
>50K
Hemorrha gic
Red
Opaque
N/a
Crystal
Induced
Yellow/
White,
Translucent
/opaque
10K-50K
-ve Gm stain
& Culture (Cx)
-ve Gm stain &
Culture
+/- Gm stain, +/-
Culture
-ve Gm stain, Cx.
-ve Gm stain, Cx.
+ Crystals

Gout
 Clinical picture:
 Usually adult men, If occurs in adolescent- underlying enzyme disorders, Rare in pre-menopausal women
 Articular disease-
 Acute gouty arthritis
 Intercritical (or interval) gout
 Chronic tophaceous gout
 Intermittent, recurrent attacks
 Involvement of 1 st MTP is typical, Any joint can be involved.
 Spine disease: Can involve any level

Gout
 Clinical picture:
 Extra-Articular disease
 Tophi
 Renal disease: Uric acid nephrolithiasis and interstitial nephropathy
 Laboratory abnormalities
 Hyperuricemia: helpful not diagnostic
 Crystal analysis: negatively birefringence, needle shaped, yellow when parallel

Gout
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Gout
 Treatment:
 Acute Attacks
 NSAIDs: Any NSAID works fine ( Indomethacin is NOT the preferred agent)
 Colchicine: remember colchicine associated GI events and neuro-myopathy
 Steroids: Systemic and Intraarticular

Gout
 Treatment:
 Management of Hyperuricemia:
 Allopurinol: Remember hypersensitivity syndrome and dose adjustment in renal disease, prophylaxis with colchicine initially to avoid acute attack
 Uricosuric agents: probenecid and sulfinpyrazone
(Ineffective in Renal insufficiency, do not use in patients with renal stones)
 Uloric/Febuxostat (XO blocker). Lower risk of hypersenstivity
 Pegloticase (Krystexxa -recombinant porcine like uricase)

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1.
2.
3.
4.
5.
6.

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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.
 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, pseudo-
AS, 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

4.
5.
6.
Crystal Arthritis
 Questions asked
1.
Patient scenario: elderly male, multiple medical problems, outpatient, acute inpatient
2.
3.
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?

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
 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
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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.
2.
3.
4.
Patient scenario: middle aged female, AA, both outpatient and inpatient
Lupus patient with psychosis, No other active symptoms, signs. Likely cause?
Patient presents with rash, polyarthritis, recently on plaquenil. What to do next?
Lupus patient with abnormal urine. Next diagnostic test?
5.
6.
Biopsy shows Class IV disease. Appropriate treatment?
Lupus patient (longer duration) presents with chest pain. Next test? Likely diagnosis? Cause of death?

SLE

10.
Most specific autoantibody with SLE.
11.
Female patient with cough, hemoptysis, +ve
ANA. Likely diagnosis?
12.
Drug induced lupus.

SLE

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

Sjogren’s syndrome

 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

 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 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
 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

-Associated with high morbidity and mortality
-Treatment requires anticoagulation and
Possibly plasmapharesis,
IVIG, IV cyclophosphamide

SLE and related syndromes
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 2 nd 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
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
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Limited Systemic Sclerosis



 Calcinosis
 Raynaud’s phenomemnon
 Esophageal dysmotility
 Sclerodactyly
 Telengeactasias

Mat-like telangiectasia
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Systemic Sclerosis


 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

 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


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?


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?


10.
11.
12.
13.
14.
15.
SS patient with weight loss, bloating, BM irregularity, foul smelling stools. Diagnosis? Findings on a radiographic test?
SS patient with abnormal PFT. What is the worse prognosis finding?
Pulmonary HTN: treatment.
Raynaud’s+arthritis+myositis= what overlap syndrome? Associated antibody? Typical pulmonary finding?
Male patient with swollen arms, legs, orange peel appearance. Next test? Diagnosis?
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