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Flashcards in McGowen Deck (72):
1

Anti-dsDNA and anti-Sm

SLE

2

Anti-Scl-70

diffuse scleroderma

3

Anti-topoisomerase 1

diffuse scleroderma

4

50 yesr old AF with fatigue and perioral lesions. Ant-dsDNA and positive ANA. Dx? Most likely cause of mortality in this?

atherosclerosis or renal

5

Oral lesions

bechets, SLE

6

45 year old WF presents with CP. Hx of SLE. Recent resting has positive phospholipid antibodies. 2D echo reveals valvular thickening and verrucous valvular lesions. What is the condition called?
Nodules are ____, meanign w/o bacteria?

Libman Sacks endocarditis

sterile nodules (caused by inflammation)

7

MALT B cell lymphomas associated with?

Sjogrens

8

Beta2GPI assoiated with?

anti-phopholipid syndrome

9

37 year old AAF with hx of mood disorder. Cotton woll spots fundoscopically. Right leg venous doppler shows DVT. Diffuse LAD presents with aRF and chest pain that is work on respiration.

anti-phopholipid syndrome - think CLOTTING!

beta2GPI, anti-cardiolipin, or anti-phospholipid anitbody

10

anti-centromere antibodies. Dx? Definition?

CREST (limited scleroderma):
Calcinosis
Raynauds
Esophageal dysmotolity (barrett's??)
Sclerodactyly
Telangiectasia

11

anti-histone antibody. Dx?

drug induced SLE

12

anti-CCP

rheumatoid arthritis

13

45 year old white female with joint pain. Recently hospitalized for pyelonephritis with Cr of 1.7 on discharge. Discharged with hydralazine for BP and Cipro for infection. Develops malar rash on discharge as well as diffuse joint pain. What is most likely seen on serology.

anti-histone ab for drug induced lupus (dt hydralazine, isoniazid)

14

25 year old hispanic female with h/o SLE. Presents 16 weeks pregnant. Noticed swelling in legs and elevated BP. Fetal echo shows complete heart block in fetus. Serology possibly seen in mom?

Ro antibodies
(assoc with SLE in mom?)
Sx: swelling and elevated BP is preeclampsia

15

anti-mitochondrial ab

SJOGRENS with primary biliary cirrhosis

16

fetal complete heart block and fetal growth restriction, what ab?

Ro antibodies

17

45 year old presents with finger stiffness. She has hard white nodules on fingers and says at times fingers go blue. on PE she has perioral furrowing with red spots on lips and tongue. What is this?

CREST (limited scleroderma)
Pt has sx: calcinosis, raynauds, telangiectasias
Could also see: esophageal dysmotility

18

What disorder is associated with Pericarditis (sharp pain that gets better; assoc with diffuse ST elevation)

SLE

19

Type3 HS, HLADR4+, >40/50yo, Classic px: headache, trouble chewing/**jaw claudication**, ischemic optic neuropathy/blindness, ↑ESR, thoracic aneurysm (years later).

Temporal/giant cell arteritis

20

parotid gland enlargement

sjogren

21

jaw claudication

giant cell arteritis (hurts to chew)

22

43 year old F with dyspnea. Noted over last few mo gets SOA quickly. She has h/o GERD Raynaud's phenomenon and carpal tunnel syndrome. CXR reveals mediastinal LAD and honeycombing. Dry crackles heard on lung exam. Topoisomerase 1 ab positive. What is the cause of SOA?

she has diffuse scleroderma - interstitial lung disease is the most common cause of death

23

pulmonary artery HTN more commonly seen in limited or diffuse scleroderma?
how about interstitial lung disease?

limited scleroderma

diffuse scleroderma

24

dry crackles (velcro like) caused by?
wet crackles caused by?

interstitial lung disease (diffuse scleroderma)
CHF

25

asthma with eosinophils

EGPA - eospinophilic granulomatous poly angiitis (Churg strauss)

26

30 year old AAF c/o dry itchy skin. She is a coal miner. She says that her fingers and mouth feel tight and she is having difficulty eating. Noticed white patches on her skin. On ends of fingers she has developed sores. She has frequent heartburn. What is her dx and the most likely etiology of her dx?

Dx: scleroderma
Etiology: possibly silica (silicosis)

27

itchy skin ddx

scleroderma
sjogrens

28

These are possible etiologies of ___?
hormone replacement therapy, cocaine use, silica exposure, appetite suppressants, bleomycin (cancer)

scleroderma

29

30 year old AAM c/o fatigue, stiffness, and malaise. Mscle weakness, arthralgia. A h/o Raynaud's and CTS. Presents to ED with confusion and BP of 212/109. Protein, microscopic blood, and negative granular casts in urine. Anti-centromere Ab are negative. RNA polymerases 1 and 3 Ab are positive. What is his diagnosis?

diffuse scleroderma (RNA polymerases 1 and 3 Ab)

30

watermelon stomach

GAVE syndrome - Gastric Antral Vascular Ectasia

31

sinusitis, lung involvement, renal involvement

Granulomatosis with polyangitis (wegner's)

32

vasculitis more common in males, associated with Hep B.

Polyarteritis Nodosa

33

what is hypertensive encephalopathy?

altered mental status (i.e. confusion) due to hypertension (i.e. malignant hypertension)

34

7 year old F with "spot on face". Healthy with no PMH. No h/o trauma. Spot hasn't gone away for several weeks. Dx?

Pediatric localized scleroderma - only cutaneous (morphea)

35

60 year old F with facial swelling. Last week increased dry mouth and eyes and today her face swollen.

What is this?

What is she at risk of developing?

Sjogren's

thrush

36

40 year old female with chronic oral candidiasis, and multiple dental fillings presents with feeling that she has something in her eyes constantly and she can't stand to be around bright lights. Diffuse LAD is palpated on exam. She has positive ANA, anti Ro and La Ab. She was recently diagnosed with B cell NHL.

What is this?
What is most likely to be seen on serology.

Sjogren's (karatoconjuctivitis sicca - decreased lacrimal flow); anti- ro, anti-la

elevated IgG, decreased C4 levels

37

45 year old F c/o weakness progressing over last 2 mo in arms/legs and diffusely itchy skin. "Doc, I can't climb stairs." Labs show elevated CK and aldolase. PE shows violacious periorbital macular erythema and papules over dorsal MCP joints with "Shawl sign" (poikiloderma) noted.

What is the dx?

What cancer do you be wary of?

dermatomyositis

breast cancer

38

violacious periorbital macular erythema - what is this called?

hilar rash

39

Do Hep B serology if you suspect ___

PAN

40

Do esophagogastroduodenoscopy (EGD) if you suspect ___

Scleroderma (Barrett's esophagus, adenocarcinoma)

41

41 yo F with one week h/o muscle weakness. Elevated CK, muscle biopsy shows endomysial inflammation with invasion of non necrotic muscle fibers. PE normal except for 4/5 muscle strength an db/l UE and LE.

Dx?

If biopsy was normal, what would it be? Why is there weakness in this?

polymyositis (CD8+)

polymalgia rheumatica if biopsy was normal - weakness is due to pain.

42

55 year old male with weakness in **finger flexors and quads**. Sx gradually developed over past year. Difficulty walking and knees sometimes buckle. CK level normal. Biopsy shows rimmed vacuoles on H&E stain.

Dx?

What ab associated with dx?

Dx: inclusion body myositis

Ab: cN1A antibody (highly specific for this dx)

43

anti jo-1

polymyositis (CD8+)
dermatomyositis (CD4+)

44

39 year old form India. Uncontrolled HTN 175/92. Adhering to anti-HTN meds. "Copper wire" retinal infarctions seen. Radial and pedal pulses difficult to palpate. Renal doppler US shows narrowing of renal arteries bilaterally. UA normal.

Dx?

Pt is at highest risk of developing what?

Dx: Takayasu arteritis ("pulseless disease", large vessel/AORTA vasculitis, obliterating, aortic stenosis)

Risk of developing: Aortic rupture

45

#1 cause of b/l renal stenosis in india

takayasu arteritis

46

Coronary artery aneurysm is a complication of what vasculitis?

Kawasaki

47

55 year old Turk with mouth sores with recurrent mouth sores and genital sores. Herpes w/u negative. Joint pain in right shoulder and left knee and remote h/o DVT. Recent lab work HLA-B51 was present. Developed pustule at site of lab draw.

Dx?
What is lab draw site pustule called?

Dx: Behcet Syndrome (**mouth sores**, genital sores, HLA-B51, aa/vv of ALL SIZES)

Lab draw site pustule: Pathergy


48

Bechet triad

oral lesions, genital lesions, eye inflammation

49

palpable purpura, abdominal pain, arthralgias, renal

HSP

50

lung, renal, anti-GBM ab

anti-GBM disease

51

dry mouth, dry eyes, parotid gland involvement, risk of MALT lymphoma, Ro and La ab

Sjogren syndrome

52

HLA-B51 presence

Behcet Syndrome

53

35 year old male with h/o chronic hep B and HTN. Concerns of fatigue and myalgias. Concerned about numbness in hands and feet. Shown livido reticularis (net or lace like skin discoloration).

Dx?
What organ system is not affected by this dz?

Dx: PAN

Organ systems NOT involved: pulmonary (spares the lungs)

Organ systems involved:
-cardiac (CHF, MI)
-GI (intestinal angina/chronic abd pain)
-skin
-renal (HTN and renal infarcts)

54

4 year old Asian female with diffuse LAD. 102 fever for 2 days. Palmar erythema and some desquamation of finger tips. Rapid strep negative. Congested eyes, prominent tongue papillae.

Dx?

Most likely mortality in late disease?

Dx: Kawasaki (aka mucocutaneous LN syndrome)

Mortality: Coronary artery aneurysm

55

Pulmonary embolism seen in what two syndromes?

phospholipid antibody syndrome
Behcet's

56

CHF associated with what vasculitis?

PAN

57

50 year old WF with rash on legs. H/o chronic sinusitis. CXR reveals lung nodules with necrotizing granulomas. Positive C-ANCA.

Dx:
Also see?

Dx: Wegner's (Granulomatosis with polyangiitis - GPA)

PE: saddle nose due to cartilage inflammation/collapse

58

30 yo F with h/o asthma, allergies, allergic rhinitis for 20 years. Rash, tingling on arms and legs. ANCA postiive. Rash shows necrotizing granulomas.

Dx?

What is seen on lab work?

Dx: Churg Strauss (esoinophilic granulomatosis with polyangiitis - EGPA)

Labs: eosinophilia

59

25 year old with finger ulcer that has been worsening. Smoker for 10 years.

Dx?
Etiology?
Tx?
Start colonoscopy at?

Dx: burger's disease (thromboangiitis obliterans)

Etiology: smoking

Tx: stop smoking

Colonoscopy at: 40 years

60

granuloma formation

Wegners, Churg strauss,
-possible: giant cell arteritis, takayasu

61

IgA deposition - what vasculitity?

HSP

62

Basement membrane Ab - what vaculitity?

goodpasture

63

Primary v. secondary raynauds

-Primary: no PMH/healthy person, just when in stress/cold weather fingers turn red/white/blue
-Secondary: SLE/CREST, digital ulcers (tx: CCB)

64

55 year old female with 2 day hx of acute intermittent vision changes and complete vision loss at times. Low grade fever and fatigue. Recalls "migraine" last week. Pain chewing food. ESR 55.

Dx?
tx? when?

Dx: Giant cell arteritis (vision change, HA, jaw claudication, elevated ESR)

Tx: glucocorticoid steroids immediately BEFORE biopsy

65

50 year old female with constant, severe neck and shoulder pain and feeling weak. She reports weight loos of 15 pounds over the last 3 mo. She has difficulty standing from a seated position due to pain. Muscle biopsy reveals no inflammation and is considered normal.

Dx?
Associated with hx of ___ and ___?

Polymyalgia rheumatica

Associated with hx of Giant Cell arteritis and CTS

66

DVT associated with what two syndromes?

antiphospholipid and Behcets

67

55 year old female with persistent HA and can't sleep due to the pain for weeks. Constantly tired and 10 lb weight loss. Painful to chew food.

Dx?

Temporal arteritis

68

most common cause of death in SLE pts

DPGN.

69

What has a steroid response v. not: dermato/polymyositis v. inclusion body myositis

Y - dermato/polymyositis
N - inclusion body myositis

70

IVIg w/in 10 days and HD ASA --- this is tx of?

Kawasaki disease (mucocutaneous LN syndrome)

71

Tx of __: LD CS

Behcets

72

biopsy shows ­ segmental granulomatous vasculitis w/ multinucleated giant cells. What is this classic biospy finding for and what is the tx?

Temporal/Giant Cell arteritis

steroids BEFORE biopsy