Rheumatology

Question 1

What is Systemic Lupus Erythematosus?

Answer 1

Chronic multi-organ autoimmune disorder affecting the skin, joint, kidneys, lungs, nervous and CV system

Question 2

When is SLE seen most?

Answer 2

Young (reproductive) adult females (African American 4:1)

Question 3

What are the clinical manifestations of SLE mediated by?

Answer 3

Antibody formation and creation of immune complexes (they deposit in and damage tissue)

Question 4

What are general sx of SLE?

Answer 4

Fever, fatigue, weight loss, LAD

Question 5

What is seen on the skin in SLE?

Answer 5

Malar (butterfly) rash, discoid rash, mucocutaneous lesions (painless oral or nasal ulcers), alopecia, raynaud phenomenon

Question 6

Malar (butterfly rash)

Answer 6

Fixed erythema over nasal bridge and spares nasolabial folds!

Question 7

Discoid rash of SLE

Answer 7

Erythematous patches with keratotic scaling in sun exposed areas

Question 8

Raynaud phenomenon

Answer 8

Seen in 30% SLE pts

Episodic vasospasm of digital arteries with exposure to cold temps or stress

Question 9

What is the bicentennial disease?

Answer 9

Raynaud phenomenon
White (pallor)
Blue (cyanosis)
Red (erythema following rewarming)

Question 10

How do you treat Raynauds?

Answer 10

Calcium Channel Blocker

Question 11

ACR criteria for SLE Dx (4 or more)

Answer 11

Malar rash, photosensitivity (rash), polyarthritis, renal disorders, hematologic disorders, + anti-DNA/Sm/Antiphosholipid Ab, mucosal ulcers, serositis, neurological disorders, +ANA

Question 12

Why are SLE pts at risk for MI?

Answer 12

Accelerated atherosclerosis

Question 13

How is ANA reported?

Answer 13

Titer of antibodies with serial dilution

Staining pattern of antibodies (loosely associated with underlying autoimmune disease)

Question 14

ANA in SLE

Answer 14

Seen in 95% of pts

Cardinal feature but not specific to just SLE

Question 15

Describe the nuclear staining patterns

Answer 15

Homogenous: entire nucleus stained
Speckled: fine, course speckles through nucles
Centromere: dots localize to chromosomes in dividing cells
Nucleolar: homogenous staining of nucleolus

Question 16

What are the ANA subtype antibodies we test for?

Answer 16

Anti-dsDNA, anti-Sm, antiphospholipid (anticardiolipin Ab, beta 2 glycoprotein Ab, lupus anticoagulant)

Question 17

What is seen in the diagnostic evaluation of SLE?

Answer 17

CBC: anemia, leukopenia, thrombocytopenia
Serum creatinine: elevated with renal dysfunction
U/A: hematuria, proteinuria, cellular casts
Liver function tests
ESR/CRP: elevated with inflammation
C3 and C4: low complement levels indicate active lupus
ANA and other specific antibodies done after + test

Question 18

Nonpharmacologic tx of SLE

Answer 18

Sun protection, diet/nutrition, exercise, smoking cessation, immunizations, tx of cormobiditeis, pregnancy and contraception

Question 19

How is SLE pharmacoligic tx tailored?

Answer 19

To match disease severity

Question 20

What is the first line tx for SLE?

Answer 20

Antimalarials (hydroxychloroquine/Plaquenil daily)

Question 21

What must you do to someone on Plaquenil?

Answer 21

Must have them do regular ophthalmologic follow up (also renal toxic)

Question 22

What drugs can be added to tx of SLE based on severity?

Answer 22

NSAIDs, systemic corticosteroids, immunosuppressive agents (methotrexate, azathioprine etc)

Question 23

What are some common drugs that may lead to drug induced SLE?

Answer 23

Procainamide, isoniazid, hydralazine

Question 24

What is the different diagnostic seen in drug-induced SLE but not regular SLE?

Answer 24

Positive antihistone antibody

Will probably have + ANA but no other positives and complement levels are not low

Question 25

Poor prognostic indicators of SLE

Answer 25

Renal disease, HTN, male, antiphospholipid Ab, high overall disease activity, low socioeconomic status

Question 26

What is polymyositis?

Answer 26

Idiopathic progressive, inflammatory condition causing symmetric proximal muscle weakness *deltoid and hip flexors are common sites

Question 27

Presentation of polymyositis

Answer 27

Progressive symmetric muscle weakness of proximal upper and lower extremities (painless usually) Cough or SOB (due to interstitial lung disease of scarring of lung tissue) Raynauds

Question 28

Dermatomysotis and the classic findings

Answer 28

All sxs/signs of polymyositis + cutaneous eruptions (heliotrope rash, Gottron's papules, shawl sign) May have occult malignancy

Question 29

Heliotrope rash

Answer 29

Violet color rash around the eyes/eyelids

Question 30

Gottron's papules

Answer 30

Thickening on dorsal side of PIP, DIP and MDP joints

Question 31

Labs for polymyositis (and dermatomyositis)

Answer 31

Muscle enzymes CK and aldolase elevated | ANA + in about 80%

Question 32

First line tx of polymyositis and dermatomyositis

Answer 32

Glucocorticoids for the inflammation (azathioprine or methotrexate added in severe cases)

Question 33

When is the initial dx of Sjogren's usually?

Answer 33

40-60 yo (much more in females)

Question 34

What is Sjogren's?

Answer 34

Chronic dysfunction of the exocrine glands (primarily lacrimal and salivary)

Question 35

Sicca complex

Answer 35

Sjogrens, combo of xerophthalmia (dry eyes) and xerostomia (dry mouth)

Question 36

Constitutional sx of Sjogrens

Answer 36

Fatigue!

Question 37

Presentation of Sjogrens

Answer 37

Keratoconjunctivitis sicca, parotid gland enlargement, dryness of other surfaces, athraligia/arthritis, Raynauds, LAD etc

Question 38

Labs done for Sjogrens

Answer 38

``` Schirmer test for tear production + ANA in 95% ANA subtypes: anti-Ro/SS-A, Anti-La/SS-B Rf + 70% ESR and CBC etc ```

Question 39

Tx for Sjogrens:

Answer 39

F/u with dentist and ophth. Dry eyes: artificial tears, lubricating ointments, cyclosporine drops (Restasis) Xerostomia: saliva substitute (Biotene OTC), water, sugarfree candy Extraglandular: NSAIDs (may need steroids or immunosuppressive)

Question 40

What is polyarteritis nodosa?

Answer 40

Systemic necrotizing vasculitis causing transmural inflammation of muscular arteries causing narrowing of lumen Result in thrombosis, ischemia or infarct

Question 41

Clinical presentation of PAN

Answer 41

``` Fever, weight loss, malaise Sensory and motor polyneuropathy Arthraligia/myalgia Nodules, ulcers, purpura Bx of lesions shows leukocytoclastic vasculitis (white cell debris) ```

Question 42

What is the most common systemic involvement in PAN?

Answer 42

Renal (infarctions or hypertension)

Question 43

Labs done in PAN

Answer 43

ESR, CRP elevated Negative (antineutrophil cytoplasmic antibodies) *positivie ANCA points to other types of vasculitis Might see microaneurysms on arteriography

Question 44

Initial tx for mild or cutaneous PAN

Answer 44

Glucocorticoids

Question 45

Initial tx for moderate/severe PAN:

Answer 45

Glucocorticoids/ immunosuppressive meds

Question 46

Initial tx for PAN associated with Hep B or C

Answer 46

Primarily antivirals and maybe glucocorticoids depending on severity

Question 47

Initial tx for PAN associated with HTN

Answer 47

ACE-I

Question 48

Maintenance of PAN

Answer 48

Steroid taper or immunosuppressants

Question 49

What is systemic sclerosis (scleroderma)

Answer 49

Autoimmune disorder causing diffuse fibrosis (tightening and thickening) of the skin and internal organs "Stone skin" Immunologic mechanisms leading to vascular endothelial damage and activation of fibroblasts

Question 50

When is the onset of scleroderma usually?

Answer 50

20-50 yo, more females

Question 51

2 types of scleroderma

Answer 51

Limited cutaneous (80%) or diffuse cutaneous (20%)

Question 52

Limited cutaneous SSc

Answer 52

CREST syndrome Skin changes limited to hands, forearm, face, neck Better prognosis

Question 53

Diffuse cutaneous SSc

Answer 53

Sclerotic skin also on chest, abs, arms and shoulders | Increased risk of internal organ involvement