Rheum Exam 2

Front 1

1. Discoid and Subacute Cutaneous Lupus Erythematosus (6)

Back 1

a. Topical steroids
b. Intralesional steroid injection
c. Hydoxychloroquine
d. Thalidomide
e. Leflunomide
f. Sun block

Front 2

Neonatal Lupus

Back 2

a. Treat symptoms present

Front 3

3. Drug Induced Lupus

Back 3

a. Symptoms resolve with removal of triggering agent

Front 4

SLE: Joint Pain, skin rash fatigue, dyslipidemias, decreases flares & steroid use

Back 4

c. Hydroxychloroquine* NSAIDs

Front 5

SLE: Significant arthritis or serositis or major organ involvement

Back 5

Steroids

Front 6

SLE: Significant arthritis, steroid sparing, and maintance

Back 6

MTX

Front 7

SLE: i. Significant arthritis, rash, and perhaps major organ involvement

Back 7

f. Leflunomide

Front 8

SLE: i. Serious neuro dz, renal dz, or vasculitis

Back 8

g. Cyclophosphamide

Front 9

SLE: i. Serious major organ involvement

Back 9

h. Mofetil Mycophenlate or Azathioprine

Front 10

Adult patients with active, auto antibody positive SLE receiving standard therapy, first FDA approved human monoclonal ab-b-lymphocyte stimulator specific inhibitor

Back 10

i. Benlysta (Belimumab):

Front 11

Benlysta MOA, SE, CI

Back 11

i. Blocks the binding of soluble BLyS, a b-cell survival factor, to its receptors on B cells
ii. Indicated for adult patients with active, auto antibody positive SLE receiving standard therapy
1. Kidney CNS lupus pts excluded
iii. IV 10mg/kg at 2 week intervals for the first 3 doses, and at 4 week intervals thereafter
iv. African American & Hispanic patients do not appear to benefit
v. SE: mortality, infx, nausea, diarrhea, depression
vi. Live vaccines are a CI

Front 12

5. Anti-Phospholipid (APL) Syndrome

Back 12

a. + APL abs and no thrombosis
i. No rx, although a baby ASA (81mg) often recommended
b. +APL abs and thrombosis
i. ASA & Coumadin with INR between 2-3
1. Exceptions: no anticoagulation if platelets <50,000
ii. Continue anticoagulation even when APL becomes negative
iii. LMWH may be an option (ongoing studies)
c. Pregnancy:
i. Anticoagulation with heparin and aspirin

Front 13

6. Morphea Scleroderma

Back 13

a. Can be self limiting with gradual resolution over 3-5 years in a mild case like plaques
b. If diffuse/deep
i. Systemic IV steroids
ii. Penicillamine
iii. Photo-therapy

Front 14

7. Raynauds – CREST

Back 14

a. CCBs most effective in controlling vasospasm

Front 15

8. Progressive Systemic Sclerosis - Raynaud's? Pulm HTN? Musculoskeletal/Synovitis? Renal?

Back 15

a. Limited therapy
b. Raynaud’s
i. CCBs
c. Pulmonary HTN
i. Bosarten
d. Musculoskeletal/Synovitis
i. NSAIDs
ii. Plaquenil
iii. Steroids
e. Renal
i. ACE inhibitors
f. STEROIDS TEND TO WORSEN PSS
i. Can precipiate HTN/renal crisis
ii. If one must use them, low doses 5 to 10 mg of prednisone for as short a duration as possible

Front 16

9. Sjogren’s Syndrome (glands vs. extra)

Back 16

a. For Glands:
i. Supportive: hydration, drops, lozenges
ii. Salagen/evoxac
iii. Tear duct ablation/plugs
b. Extraglandular:
i. Plaquenil (anti-cholinesterase activity)
ii. Steroids
iii. NSAIDs

Front 17

10. Sarcoidosis (cytotoxic (4), antimicrobial (3), cytokine modulators (3))

Back 17

a. Cytotoxic agents:
i. MTX
ii. Azathioprine
iii. Cyclophosphamide
iv. Leflunomide
b. Antimicrobial agents:
i. Chloroquine
ii. Hydroxychloroquine
iii. Minocylcine
c. Cytokine modulators:
i. Pentoxifylline
ii. Thaliomide
iii. Infilixmab

Front 18

11. Polymyositis and Dermatomyositis Inflammatory Myopathy (6)

Back 18

a. Steroids
b. MTX
c. Azathioprine
d. Lefluonmide
e. Cyclophosphamide
f. IVIG

Front 19

12. Giant Cell Arteritis

Back 19

a. High dose steroids for at least 1 month
b. Rule = treat first with steroids to avoid visual loss, then bx within 1 week before the histology is affected
c. May require immunosuppressive therapy  MTX, azathioprine
d. Usually resolves in 6-24 mos

Front 20

13. Granulomatosis with Polyangitis (4)

Back 20

a. Steroids
b. Cyclophosphamide
c. MTX
d. ? TNF inhibitors, ? Sulfa abx (bactrim)

Front 21

14. Allergic Angitis & Granulomatosis (Churg-Strauss Syndrome) (2)

Back 21

a. Steroids – IV pulse high doses
b. IV Cyclophosphamide
c. Even with therapy, survival is not high

Front 22

16. Leukocytosis Vasculitis (LCV)

Back 22

a. Treat underlying cause

Front 23

17. Henoch-Schonlein Purpura (HSP)

Back 23

a. Usually self limited in 4-16 weeks
b. Supportive – ensure adequate hydration & replacement of excess blood loss
c. Search for/tx underlying or predisposing factors
d. Corticosteroid controversies:
i. Reduce severity/duration
ii. Decrease risk of glomerulonephritis
iii. Increase releapse rate
e. Corticosteroid consensus:
i. Glomerulonephritis: systemic steroids to protect kidneys even though relapses more frequent
ii. GI: pathology associated with his or her HSP, systemic steroids NOT helpful!

Front 24

18. Mixed Connective Tissue Disease (MCTD) (4)

Back 24

a. Continual observation – watch for signs of pulm HTN
b. Supportive care depending on extent of organ/joint involvement
c. If myositis – consider steroids
d. If pulm HTN – use Bonsentan, iloprost

Front 25

19. Relapsing Polychondritis

Back 25

a. Systemic corticosteroids
i. Prednisone 20-60 mg/d in acute phase
1. Tapered to 5-25 mg/d for maintenance – most patients need low daily dose for maintenance
2. Severe flares may need 80-100 mg/d
b. Other agents:
i. MTX
ii. Dapsone
iii. Imuran
iv. Alpha TNF inhibitors (Infliximab)

Front 26

20. Weber-Christian Disease – aka Idiopathic Lobular Panniculitis

Back 26

a. No uniformly effective therapy exsists
b. Therapeutic responses resported with:
i. Fibrinolytic agents
ii. DMARDS: hydroxychlorquine, azathioprine, thalidomide, cyclophosphabmide, tetracycline, cyclosporine, mycophenolate, clofazimine
iii. Systemic steroids (prednisone) may be effective to suppress acute exacerbations
iv. NSAIDs may reduce fever, arthralgias, other signs of malaise

Front 27

21. Rhabdomyolysis

Back 27

a. Fluids
b. Prevention of acute renal failure from myoglobin pigment (mannitol diuresis) and urine alkalinization
c. Correct electrolyte imbalances (K+)
d. Supportive care, tx underlying injury

Front 28

ANA fraction patterns

Back 28

Peripheral (rim), homogenous (diffuse), speckled, centromere, nucleolar

Front 29

Peripheral (rim)

Back 29

anti-DNA (not seen on HEp-2) = SLE

Front 30

Homogenous (diffuse)

Back 30

anti DNA = RA & SLE
anti-histone = Misc. disorders
anti-DNP (nucleosomes) = anti-ssDNA

Front 31

Speckled

Back 31

anti- Sm & RNP + anti-ro/la = SLE & SS

anti-Jo-1 & Mi-2 = PM/DM

anti-Scl-70 = PSS (systemic)

Front 32

Centromere

Back 32

anti-centromere = PSS (Crest)

Front 33

Nucleolar

Back 33

anti-nucleolar = SLE & PSS