Connective Tissue DO's & Vasculitides

Question 1

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

Answer 1

Anti-nuclear Ab (ANA) by indirect immunofluorescence

Titer of <1.40 is normal

Question 2

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

Answer 2

SLE

Question 3

SLE common features

Answer 3

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

Question 4

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

Answer 4

MI due to accelerated atherosclerosis

Question 5

What labs would indicate SLE?

Answer 5

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

Question 6

SLE treatments

Answer 6

avoid sun exposure
NSAIDs 
Glucocorticoids
Hydroxychloroquine
Minimize risks for atherosclerosis

Question 7

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

Answer 7

Cotton Wool Spots

Question 8

Drug-Induced Lupus Mechanism

Answer 8

promotes demethylation of DNA

Question 9

Drug-Induced Lupus labs

Answer 9

+ANA

+Anti-histone Ab’s (95%)

Question 10

What are some medications that can induce Lupus?

Answer 10

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

Question 11

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

Answer 11

Neonatal Lupus

Question 12

Describe Neonatal Lupus symtpoms

Answer 12

Transient: 
Rashes
Thrombocytopenia
Hemolytic Anemia
Arthritis 
MONITOR FOR HEART BLOCK

Question 13

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

Answer 13

Discoid Lupus

Question 14

What is the treatment for Discoid lupus?

Answer 14

Photoreception + anti-inflammatory agents or antimalarial drugs

Question 15

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?

Answer 15

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

Question 16

3 types of Scleroderma

Answer 16

1. Localized
2. Limited
3. Diffuse

Question 17

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

Answer 17

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

Question 18

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

Answer 18

Pulmonary Artery HTN

Renal Crisis uncommon

Question 19

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

Answer 19

Renal Crisis and Interstitial Lung Dz (velcro crackles)

Question 20

What would you NOT prescribe for Diffuse Systemic Sclerosis?

Answer 20

High Dose glucocorticoids

*can induce renal crisis

Question 21

Labs for Diffuse Scleroderma

Answer 21

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

Question 22

Labs for Limited Scleroderma

Answer 22

+ Anti-centromere

Question 23

What should Sjogren’s patients avoid taking?

Answer 23

Atropinic drugs and decongestants

Question 24

Dermatomyositis symptoms

Answer 24

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

Question 25

How does Dermatomyositis differ from Polymyositis?

Answer 25

NO skin changes seen with PM

Question 26

Treatment for Inflammatory Myopathies

Answer 26

Glucocorticoids

Question 27

Inclusion Body myositis

Answer 27

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

Question 28

How do we characterize systemic vasculitides?

Answer 28

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

Question 29

Small Vessel Vasculitides

Answer 29

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

Question 30

Variable Vasculitides

Answer 30

Eosinophilic Granulomatosis with Polyangiitis | Behcet Syndrome

Question 31

Medium Vasculitides

Answer 31

``` Thromboangiitis Obliterates (Buerger Dz) Polyarteritis Nodosa Kawasaki Dz ```

Question 32

Large vessel vasculitides

Answer 32

Takayasu Arteritis | Giant Cell Arteritis

Question 33

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

Answer 33

IgAV (HSP)

Question 34

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

Answer 34

Goodpasture Syndrome

Question 35

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

Answer 35

Wegener's Granulomastosis (GPA)

Question 36

Churg-Strauss Syndrome

Answer 36

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

Question 37

Behcet Syndrome

Answer 37

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

Question 38

Thromboangiitis Obliterates (Buerger)

Answer 38

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

Question 39

Polyarteritis Nodosa

Answer 39

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

Question 40

Kawasaki Dz

Answer 40

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

Question 41

What is referred to as Pulseless Disease?

Answer 41

Takayasu Arteritis: <40yo F>M smooth tapered stenosis of large vessels * renal artery stenosis * retinopathy (copper-wiring) * aortic complications

Question 42

Giant Cell Arteritis

Answer 42

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

Question 43

Polymyalgia Rheumatica

Answer 43

Weakness secondary to pain | Elevated ESR and CRP