Rheumatology Flashcards

1
Q

What is Systemic Lupus Erythematosus?

A

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

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2
Q

When is SLE seen most?

A

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

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3
Q

What are the clinical manifestations of SLE mediated by?

A

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

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4
Q

What are general sx of SLE?

A

Fever, fatigue, weight loss, LAD

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5
Q

What is seen on the skin in SLE?

A

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

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6
Q

Malar (butterfly rash)

A

Fixed erythema over nasal bridge and spares nasolabial folds!

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7
Q

Discoid rash of SLE

A

Erythematous patches with keratotic scaling in sun exposed areas

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8
Q

Raynaud phenomenon

A

Seen in 30% SLE pts

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

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9
Q

What is the bicentennial disease?

A

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

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10
Q

How do you treat Raynauds?

A

Calcium Channel Blocker

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11
Q

ACR criteria for SLE Dx (4 or more)

A

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

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12
Q

Why are SLE pts at risk for MI?

A

Accelerated atherosclerosis

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13
Q

How is ANA reported?

A

Titer of antibodies with serial dilution

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

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14
Q

ANA in SLE

A

Seen in 95% of pts

Cardinal feature but not specific to just SLE

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15
Q

Describe the nuclear staining patterns

A

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

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16
Q

What are the ANA subtype antibodies we test for?

A

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

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17
Q

What is seen in the diagnostic evaluation of SLE?

A

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

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18
Q

Nonpharmacologic tx of SLE

A

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

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19
Q

How is SLE pharmacoligic tx tailored?

A

To match disease severity

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20
Q

What is the first line tx for SLE?

A

Antimalarials (hydroxychloroquine/Plaquenil daily)

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21
Q

What must you do to someone on Plaquenil?

A

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

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22
Q

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

A

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

23
Q

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

A

Procainamide, isoniazid, hydralazine

24
Q

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

A

Positive antihistone antibody

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

25
Q

Poor prognostic indicators of SLE

A

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

26
Q

What is polymyositis?

A

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

27
Q

Presentation of polymyositis

A

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

28
Q

Dermatomysotis and the classic findings

A

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

29
Q

Heliotrope rash

A

Violet color rash around the eyes/eyelids

30
Q

Gottron’s papules

A

Thickening on dorsal side of PIP, DIP and MDP joints

31
Q

Labs for polymyositis (and dermatomyositis)

A

Muscle enzymes CK and aldolase elevated

ANA + in about 80%

32
Q

First line tx of polymyositis and dermatomyositis

A

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

33
Q

When is the initial dx of Sjogren’s usually?

A

40-60 yo (much more in females)

34
Q

What is Sjogren’s?

A

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

35
Q

Sicca complex

A

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

36
Q

Constitutional sx of Sjogrens

A

Fatigue!

37
Q

Presentation of Sjogrens

A

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

38
Q

Labs done for Sjogrens

A
Schirmer test for tear production
\+ ANA in 95%
ANA subtypes: anti-Ro/SS-A, Anti-La/SS-B
Rf + 70%
ESR and CBC etc
39
Q

Tx for Sjogrens:

A

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)

40
Q

What is polyarteritis nodosa?

A

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

41
Q

Clinical presentation of PAN

A
Fever, weight loss, malaise
Sensory and motor polyneuropathy
Arthraligia/myalgia
Nodules, ulcers, purpura
Bx of lesions shows leukocytoclastic vasculitis (white cell debris)
42
Q

What is the most common systemic involvement in PAN?

A

Renal (infarctions or hypertension)

43
Q

Labs done in PAN

A

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

44
Q

Initial tx for mild or cutaneous PAN

A

Glucocorticoids

45
Q

Initial tx for moderate/severe PAN:

A

Glucocorticoids/ immunosuppressive meds

46
Q

Initial tx for PAN associated with Hep B or C

A

Primarily antivirals and maybe glucocorticoids depending on severity

47
Q

Initial tx for PAN associated with HTN

A

ACE-I

48
Q

Maintenance of PAN

A

Steroid taper or immunosuppressants

49
Q

What is systemic sclerosis (scleroderma)

A

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

50
Q

When is the onset of scleroderma usually?

A

20-50 yo, more females

51
Q

2 types of scleroderma

A

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

52
Q

Limited cutaneous SSc

A

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

53
Q

Diffuse cutaneous SSc

A

Sclerotic skin also on chest, abs, arms and shoulders

Increased risk of internal organ involvement