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SBL Exam 2 > Connective Tissue DO's & Vasculitides > Flashcards

Connective Tissue DO's & Vasculitides Flashcards

1
Q

What is one of the first tests that can be run to detect autoimmune disease?

A

Anti-nuclear Ab (ANA) by indirect immunofluorescence

Titer of <1.40 is normal

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2
Q

Female presents with malaise, malar rash, photosensitivity, and inflammatory arthritis. What is a possible diagnosis?

A

SLE

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3
Q

SLE common features

A 
Fatigue
Weight loss
Malar Rash 
Inflammation 
Pericarditis (substernal crushing CP, worse when supine, ST elevation)
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4
Q

Patients with SLE are at an increased risk for what potentially fatal morbidity?

A

MI due to accelerated atherosclerosis

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5
Q

What labs would indicate SLE?

A

+ ANA
+ anti-ds DNA (follow)
+ Smith
decreased compliment levels due to consumption

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6
Q

SLE treatments

A 
avoid sun exposure
NSAIDs 
Glucocorticoids
Hydroxychloroquine
Minimize risks for atherosclerosis
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7
Q

What would be seen on an ophthalmic exam in patients with SLE/APS?

A

Cotton Wool Spots

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8
Q

Drug-Induced Lupus Mechanism

A

promotes demethylation of DNA

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9
Q

Drug-Induced Lupus labs

A

+ANA

+Anti-histone Ab’s (95%)

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10
Q

What are some medications that can induce Lupus?

A 
Hydralazine
Isoniazid
Minocycline
TNF Inh
Quinidine
Methyldopa
Sulfa drugs (induce lupus but do not cause it)
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11
Q

A pregnant woman tests positive for anti-RO and anti-La. What is her newborn at risk for?

A

Neonatal Lupus

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12
Q

Describe Neonatal Lupus symtpoms

A 
Transient: 
Rashes
Thrombocytopenia
Hemolytic Anemia
Arthritis 
MONITOR FOR HEART BLOCK
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13
Q

What type of lupus is primarily seen on the head and face, and can develop into atrophic, disfiguring scars?

A

Discoid Lupus

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14
Q

What is the treatment for Discoid lupus?

A

Photoreception + anti-inflammatory agents or antimalarial drugs

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15
Q

A 50 year old F presents with thickening and hardening of the skin. Her skin is dry and itchy, and her fingers turn blue and white in response to cold. What is a possible diagnosis?

A

Scleroderma:
30-60 years
F>M
2 Raynauds Phenomenon

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16
Q

3 types of Scleroderma

A
  1. Localized
  2. Limited
  3. Diffuse
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17
Q

Which type of scleroderma is CREST syndrome seen in, and what does CREST stand for?

A 
Limited Scleroderma (IcSSc) 
Calcinosis 
Raynauds (2) 
Esophageal dysmotility
Sclerodactyly 
Telangiectasia
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18
Q

What is one of the primary risks associated with limited scleroderma?

A

Pulmonary Artery HTN

Renal Crisis uncommon

19
Q

What is the a primary risk associated with diffuse system scleroderma?

A

Renal Crisis and Interstitial Lung Dz (velcro crackles)

20
Q

What would you NOT prescribe for Diffuse Systemic Sclerosis?

A

High Dose glucocorticoids

*can induce renal crisis

21
Q

Labs for Diffuse Scleroderma

A

+ ANA
+ Anti-Scl 70 (anti-DNA topoisomerase I)
+ Anti-RNA polymerase III

22
Q

Labs for Limited Scleroderma

A

+ Anti-centromere

23
Q

What should Sjogren’s patients avoid taking?

A

Atropinic drugs and decongestants

24
Q

Dermatomyositis symptoms

A 
Weakness w/o sensory loss 
Skin lesions: 
Gottrons patches
Heliotrope rash (orbital edema) 
V-neck erythema (Shawl sign) 
Elevated CK &amp; Aldolase
25
Q

How does Dermatomyositis differ from Polymyositis?

A

NO skin changes seen with PM

26
Q

Treatment for Inflammatory Myopathies

A

Glucocorticoids

27
Q

Inclusion Body myositis

A

40-50 y/o
M>F
Weakness in finger flexion or quads
serology- anti-cN1A

28
Q

How do we characterize systemic vasculitides?

A

Characterized by affected vessel size (small, variable, medium, large)

29
Q

Small Vessel Vasculitides

A

IgAV (Hooch-Schonlein Purpura)
Good Pasture Syndrome
Granulomatosis with Polyangiitis (Wegener’s)

30
Q

Variable Vasculitides

A

Eosinophilic Granulomatosis with Polyangiitis

Behcet Syndrome

31
Q

Medium Vasculitides

A 
Thromboangiitis Obliterates (Buerger Dz)
Polyarteritis Nodosa 
Kawasaki Dz
32
Q

Large vessel vasculitides

A

Takayasu Arteritis

Giant Cell Arteritis

33
Q

What vasculitides is more common in children, and presents with the tetrad of 1) Purpura 2) Arthralgia 3) Abd pain, and 4) Renal Dz?

A

IgAV (HSP)

34
Q

What disease is characterized by anti-basement membrane autoAb’s, diagnosed by UA, and can lead to pulmonary hemorrhage if not treated?

A

Goodpasture Syndrome

35
Q

40 y/o M presents with granulomatous inflammation, glomerulonephritis, and epistaxis. Serology shows ANCA+, and vessels with granulomas. What is a possible Dx?

A

Wegener’s Granulomastosis (GPA)

36
Q

Churg-Strauss Syndrome

A

Asthma + Eosinophilia
Purpura
Differentiate from Wegener’s by the elevated Eosinophil count

37
Q

Behcet Syndrome

A 
Triad: 
1. recurrent mouth ulcers
2. genital ulcers
3. eye inflammation 
Serology: HLA-B51
38
Q

Thromboangiitis Obliterates (Buerger)

A

Young males <35
ONLY OCCURS IN SMOKERS
Thrombosis and loss of digits

39
Q

Polyarteritis Nodosa

A 
Associated with HBV
Peripheral Neuropathy with foot drop 
Renin-mediated HTN
Livedo reticularis and digital gangrene
Lungs are spared
Serology: check for HBV
40
Q

Kawasaki Dz

A

<5yo Japanese higher incidence
Aneurysm and MI
Presents with fever, inflamed “strawberry” tongue, lymphadenopathy, rash
tx: IVIG and ASA

41
Q

What is referred to as Pulseless Disease?

A

Takayasu Arteritis:
<40yo
F>M
smooth tapered stenosis of large vessels

  • renal artery stenosis
  • retinopathy (copper-wiring)
  • aortic complications
42
Q

Giant Cell Arteritis

A

Large vessel (cranial Aa and Aortic arch)
>50yo
HA
Jaw Claudication
Increased ESR
START GLUCOCORTICOIDS IMMEDIATELY due to risk of blindness
temporal artery biopsy is gold standard for dx

43
Q

Polymyalgia Rheumatica

A

Weakness secondary to pain

Elevated ESR and CRP

SBL Exam 2 (7 decks)

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