Rheumatology 2

Question 1

Clinical keys to diagnosis of Rheumatoid Arthritis?

Answer 1

Morning stiffness of multiple small, inflamed joints

Question 2

Specific lab dx test for Rheumatoid Arthritis?

Answer 2

Anti-Cyclic Citrullinated Protein (anti-CCP)

Question 3

What is Sicca Syndrome? What’s it found in?

Answer 3

Dry eyes, mouth, & other mucous membrane

Found in Rheumatoid Arthritis

Question 4

Rheumatoid Arthritis – type of anemia?

Answer 4

Normocytic

Question 5

Rheumatoid Arthritis – Dx criteria?

Answer 5

Need 6 or more points:

• Joint involvement (up to 5 points)
• ESR or CRP (1 point)
• Duration for longer than 6 wks (1 point)
• RF or anti-CCP (1 point)

Question 6

Rheumatoid Arthritis – most common cause of death?

Answer 6

Coronary Artery Disease

Question 7

When do RA patients need Disease-Modifying Anti-Rheumatic Drugs (DMARDs)?

Answer 7

If Erosive disease is present. Includes:

• Joint space narrowing
• Physical deformity of joints
• X-ray abnormalities

(NSAIDs & steroids great for Sx, but do NOT stop progression of RA. Stopping progression is most important issue, start w/ Methotrexate)

Question 8

Methotrexate – AEs?

Answer 8

• Liver toxicity
• Bone Marrow suppression
• Pulmonary toxicity

Question 9

anti-TNF drugs?

Answer 9

Infliximab, Adalimumab, Etanercept

Question 10

anti-TNF drugs – AEs?

Answer 10

• Reactivation of TB (screen w/ PPD prior to use)

- Infection

Question 11

Hydroxychloroquine – AEs?

Answer 11

Retina toxicity (do a dilated eye exam)

Question 12

Sulfasalazine – AEs?

Answer 12

• Rash

- Hemolysis

Question 13

Rituximab – AEs?

Answer 13

Infection

Question 14

Dx?

High, spiking fever (>104C) along w/ chest/abdomen rash in a young person w/ no identifiable cause

Answer 14

Juvenile Rheumatoid Arthritis – or “Adult Still Disease”

Rash: Often only w/ fever spikes, “salmon” colored, on chest & abdomen
Other features: Splenomegaly, Pericardial effusion, mild joint symptoms

Question 15

JRA – Tx?

Answer 15

1. NSAIDS
2. Steroids
3. TNF drugs

Question 16

SLE symptom that’s present in 90% of cases?

Answer 16

Arthritis (often first symptom)

Question 17

SLE – most specific lab finding?

Answer 17

anti-Smith antibodies

Question 18

SLE – most specific lab findings in acute flare?

Answer 18

• Complement levels drop

- anti-DS DNA levels rise

Question 19

How to determine severity of lupus nephritis?

Answer 19

Kidney biopsy (used to decide Tx based on severity)

Question 20

SLE – Most common cause of death?

Answer 20

Young patients = Infection

Older patients = MI (2/2 accelerated atherosclerosis)

Question 21

SLE – Tx?

Answer 21

• Steroids during flares
• Hydroxychloroquine controls mildly chronic disease contained to skin & joints
• Belimumab controls progression of diseae

Question 22

Antiphospholipid Syndrome – 2 main types of antibodies & their ass’d effects?

Answer 22

Lupus anticoagulant – elevated aPTT

Anticardiolipin antibodies – spontaneous abortion

Question 23

APL Syndrome – presentation?

Answer 23

Thromboses of both arteries & veins + spontaneous abortions

• • elevated aPTT w/ normal INR & PT
• • False positive VDRL or RPR (b/c reacts w/ cardiolipin)

(path = IgG or IgM antibodies against negatively charged phospholipids)

Question 24

APL Syndrome – best initial test? Most specific?

Answer 24

Best initial = Mixing study

Most specific = Russell Viper Venom Test (RVVT)

Question 25

Asymptomatic patient w/ APL Syndrome -- Tx?

Answer 25

No Tx necessary - Treat thromboses w/ heparin & warfarin

Question 26

How to prevent recurrent spontaneous abortion in APL syndrome?

Answer 26

Heparin & Aspirin **note: Warfarin is a teratogen**

Question 27

Scleroderma -- Most specific test?

Answer 27

SCL-70 (anti-topoisomerase)

Question 28

CREST syndrome -- most specific test?

Answer 28

Anticentromere antibodies - extremely specific - only ~50% sensitive

Question 29

Scleroderma -- Tx?

Answer 29

Methotrexate -- slows underlying disease process ``` Renal crisis -- ACE inhibitors Esoph Dysmotility -- PPIs for GERD Raynaud -- Ca-channel blockers Pulmonary Fibrosis -- Cyclophosphamide Pulmonary HTN -- Bosentan, Sildenafil, Prostacyclin analogs ```

Question 30

Most likely Dx? | Young woman 20s-40s w/ fibrosis of skin & internal organs such as lung, kidney, & GI tract

Answer 30

Scleroderma

Question 31

Dermatomyositis -- presentation?

Answer 31

Proximal muscle weakness - Dysphagia - Shawl sign - Heliotrope rash - Gottron papule

Question 32

Dermatomyositis -- Best initial test? Most accurate?

Answer 32

Best initial = CPK & Aldolase Most accurate = Muscle biopsy

Question 33

ANA is frequently + in Dermatomyositis. What are anti-Jo antibodies ass'd w/?

Answer 33

Pulmonary Fibrosis

Question 34

Dermatomyositis -- Tx?

Answer 34

Steroids | - if unresponsive/tolerant, use Methotrexate, Azathioprine, IVIG, or Mycophenolate

Question 35

Dermatomyositis -- Tx of skin lesions?

Answer 35

Hydroxychloroquine

Question 36

Sjogren Syndrome -- Best initial test? Most accurate? | Best initial test on blood?

Answer 36

Best initial = Schirmer test (piece of filter paper against eye looking for tears) Most accurate = Lip or Parotid gland biopsy Best initial on blood = SS-A & SS-B ("Ro" & "La")

Question 37

Sjogren Syndrome -- Tx?

Answer 37

1. Water to mouth, sugar-free gum, & Fluoride 2. Pilocarpine & Cevimeline to inc Acetylcholine (Cevimeline inc's saliva production)

Question 38

All vasculitides give what 4 Sx?

Answer 38

- Fever - Malaise/fatigue - Weight loss - Arthralgia/myalgia

Question 39

Polyarteritis Nodosa -- presentation?

Answer 39

- Disease of small & medium-sized arteries - Spares the lungs - Ass'd w/ Hep B & C

Question 40

PAN -- Tx?

Answer 40

Prednisone & Cyclophosphamide - Treat hepatitis when found

Question 41

Polymyalgia Rheumatica -- presentation?

Answer 41

Pain & stiffness in shoulder & pelvic girdle muscles - Difficulty combing hair & rising from chair - Elevated ESR (normal CK & aldolase) - Normochromic, normocytic anemia

Question 42

Polymyalgia Rheumatica -- Tx?

Answer 42

Steroids, low-dose has rapid response

Question 43

Giant Cell Arteritis -- Tx?

Answer 43

High-dose steroids ASAP (prevent blindness)

Question 44

Wegener's -- best initial test? Most accurate?

Answer 44

Best initial = C-ANCA (anti-proteinase 3) Most accurate = Biopsy (Lung > renal > sinus)

Question 45

Wegener's -- Tx?

Answer 45

Prednisone & Cyclophosphamide

Question 46

Churg-Strauss Syndrome -- presentation?

Answer 46

Pulmonary-Renal Syndrome w/: - Asthma - Eosinophilia (Tx same as others Pred & Cyclophos, biopsy is most accurate test)

Question 47

Dx? | Biopsy shows "Leukocytoclastic vasculitis"

Answer 47

Henoch-Schonlein Purpura | usually clinical dx, but biopsy is most accurate test

Question 48

Cryoglobulinemia is ass'd w/ what?

Answer 48

Chronic Hep C infection

Question 49

Dx? | Middle Eastern person w/ painful oral & genital ulcers in assoc w/ erythema nodosum-like lesions of skin.

Answer 49

Behcet Syndrome also w/: - Ocular lesions leading to uveitis & blindness - Arthritis - CNS lesions mimicking MS

Question 50

What is "Pathergy"? What Dx is it ass'd w/?

Answer 50

Pathergy = sterile skin pustules from minor trauma like a needle stick Assoc'd w/ Behcet Syndrome

Question 51

What are the non-biologic Disease Modifying Agents?

Answer 51

Methotrexate, Hydroxychloroquine, Sulfasalazine, Leflunomide, Azathioprine

Question 52

What are the biologic Disease Modifying Agents?

Answer 52

Etanercept, Infliximab, Adalimumab, Tocilizumab, Rituximab