Rheumatic Disorders

Question 1

Are men or women more likely to be effected by SLE?

What ethnicities are more commonly effected?

Answer 1

Women

African American
Hispanic
Native America

Question 2

What are the most common causes of death in SLE patients in the USA?

Answer 2

Heart Disease and Stroke
Malignancies (Lung CA)
Infection
Renal Disease

Question 3

Should patients with discoid lupus avoid the sun?

Answer 3

Yes!

Question 4

What are DEFINITE medications that can cause drug induced lupus?

(Theres a lot)

Answer 4

Procainamide
Hydralazine
Minocycline 
Diltiazem
Penicillamina
Isoniazid 
Quinidine
Anti-TNFs
Methyldopa
Chlorpromazine

Question 5

What are the ELEVEN classifications for SLE?

How many of these are needed for a diagnosis?

Answer 5

Malar Rash
Discoid Rash
Photosensitivity
Oral/Nasopharyngeal Ulcers
Arthritis
Serositis (Pericarditis/Pleuritis) 
Renal Involvement (Proteinuria) 
Neurologic Disorder (Seizure) 
Hematologic Involvement (Anemia, Leukopenia) 
Immunologic Disorder (+ Anti-dsDNA or SM)
Positive ANA

Question 6

What are examples of medications approved for the treatment of SLE?

Answer 6

Corticosteroids 
Cyclophosphamide
Methotrexate
Mycophenolate mofetil
Azathioprine
Hydroxychloroquine 
Belimumab 
Aspirin

Question 7

Why do SLE patients taking hydroxychloroquine need eye examinations every 6-12 months?

Answer 7

Due to retinal Toxicity

Question 8

A 23-year-old Hispanic female with no past medical history presented to the emergency department (ED) with an 8-week history of joint pain and swelling in the hands, knees, and ankles; fever; myalgias; pleuritic chest pain; weight loss; and a facial rash that worsened with sun exposure. She had been seen initially at a local clinic and treated for “cellulitis” with oral Keflex. Two days prior to this presentation, she was seen in another ED, found to have a temperature of 103F, proteinuria, and anemia; she was told it was a “viral syndrome” and discharged home.

On examination today, she has painless ulceration on the palate, erythematous malar rash, diffuse lymphadenopathy, and synovitis of the MCP/PIP joints.

Her blood work today show leukopenia and elevated protein, but is otherwise unremarkable. Her urinalysis shows proteinuria with hematuria present.

What is your suspected diagnosis and what antibodies would be present to confirm this?

Answer 8

SLE

+ ANA
+ Anti-dsDNA
+ Anti-Sm

Question 9

This rheumatic disorder is characterized as a drying of mucus producing glands.

Answer 9

Sjogren’s Syndrome

Question 10

Is Sjogren’s Syndrome more common in men or women?

Answer 10

Women

Question 11

What are the ‘classic’ symptoms of Sjogren’s Syndrome?

Answer 11

Dry Eyes
Dry Mouths
Vaginal Dryness
Dental Carries
Parotid Swelling
Arthralgias

Question 12

What test in the eye is commonly used to diagnosis Sjogren’s Syndrome?

Answer 12

Schirmer Test

Question 13

What laboratory tests are specific to Sjogren’s Syndrome?

Answer 13

SS-A (Ro)
SS-B (La)
Rheumatoid Factor

Question 14

What vascular phenomenon is seen in Systemic Sclerosis (Scleroderma)?

Answer 14

Raynauds

Question 15

How would the body hair appear on a patient with Systemic Sclerosis (Scleroderma)?

Answer 15

Loss of appendicular hair

Question 16

What might the stomach be described as in a patient with Systemic Sclerosis (Scleroderma)?

Answer 16

Watermelon Stomach

Question 17

What is the ‘classic’ finding in the hands of a patient with Systemic Sclerosis (Scleroderma)?

Answer 17

Thickening, Tightening, and Hardening of the skin

Loss of the ability to create folds and creases

Question 18

T/F: Raynaud’s Phenomenon can lead to necrosis?

Answer 18

True

Question 19

What composes CREST Syndrome?

Answer 19

Calcinosis Cutis
Raynaud Phenomenon
Esophageal Dysmotilty
Sclerodactyly
Telangiectasia

Question 20

CREST Syndrome is associated with a ___________ ANA pattern.

Answer 20

Centromere

Question 21

What is the classic presentation of Polymylgia Rheumatica (PMR)?

Answer 21

Proximal Pain Without Weakness

Question 22

The pneumonic “SECRET” is often used in PMR…..

What does it stand for?

Answer 22

Stiffness
Elderly (>50, commonly 70-80)
Constitutional Sx (also causcasian) 
Rheumatism
Elevated ESR
Temporal Arteritis

Question 23

Are imaging studies need when working up Polymyalgia Rheumatica?

Answer 23

No

Question 24

A 76 y.o. female presents 4 weeks of neck pain, bilateral shoulder pain, and a new onset of bilateral hip pain. She complains that these symptoms are worse in the morning. She has been able to ambulate fairly well, but the pain in her shoulders has effected her ROM. Prior to this, she denied any injuries and was otherwise healthy. On examination, she has slightly decreased ROM in the shoulders but she has 5/5 muscle strength throughout all extremities. Otherwise, her exam is unremarkable.

You order labs wisely, and her CBC is normal, but she has an elevated ESR. She has a negative ANA, Anti-dsDNA, and RF as well.

What is your suspected diagnosis?

Answer 24

Polymyalgia Rheumatica

Question 25

A 80 y.o. female presents to the ED with complaints of a right temporal headache. She noted the the headache has gradually worsened over the last week. She described it as a dull, throbbing, ache. Interestingly, she has recently noted a cramping pain with eating, which is new. In addition, brushing her hair on the right side is painful and bothersome. She denied any history of similar in the past. She denied any fever, chills, neck pain, or motor weakness associated with the headache. On examination, you note the the right temple is tender to palpation and the right temporal pulse is reduced. She also has the presence of carotid bruits. Her visual acuity is WNL however there is the presence of cotton wool spots on fundoscopy. What lab would you expect to see elevated?

Answer 25

ESR

Question 26

A 80 y.o. female presents to the ED with complaints of a right temporal headache. She noted the the headache has gradually worsened over the last week. She described it as a dull, throbbing, ache. Interestingly, she has recently noted a cramping pain with eating, which is new. In addition, brushing her hair on the right side is painful and bothersome. She denied any history of similar in the past. She denied any fever, chills, neck pain, or motor weakness associated with the headache. On examination, you note the the right temple is tender to palpation and the right temporal pulse is reduced. She also has the presence of carotid bruits. Her visual acuity is WNL however there is the presence of cotton wool spots on fundoscopy. What would be most diagnostic in this patient's case

Answer 26

Temporal Artery Biopsy is the most definitive diagnostic study for Giant Cell Arteritis (GCA)

Question 27

A 80 y.o. female presents to the ED with complaints of a right temporal headache. She noted the the headache has gradually worsened over the last week. She described it as a dull, throbbing, ache. Interestingly, she has recently noted a cramping pain with eating, which is new. In addition, brushing her hair on the right side is painful and bothersome. She denied any history of similar in the past. She denied any fever, chills, neck pain, or motor weakness associated with the headache. On examination, you note the the right temple is tender to palpation and the right temporal pulse is reduced. She also has the presence of carotid bruits. Her visual acuity is WNL however there is the presence of cotton wool spots on fundoscopy. Temporal artery biopsy confirms GCA. What would be the most concerning complication of this disease?

Answer 27

Vision Loss

Question 28

T/F: GCA is an ophthalmological Emergency

Answer 28

True

Question 29

How is PMR treated when compared to GCA?

Answer 29

PMR: 15 mg/day Prednisone Up to 1-2 year taper GCA: 60 mg/day prednisone Long time

Question 30

______ _______ is described as a systemic necrotizing vasculitis typically affecting medium-sized muscular arteries.

Answer 30

Polyarteritis Nodosa

Question 31

What organs are typically involved in polyarteritis nodosa?

Answer 31

``` Kidney Skin Joints Muscles Peripheral Nerves GI Tract ```

Question 32

Polyarteritis Nodosa is typically idiopathic, but it can be associated with what diseases?

Answer 32

Hep B Hep C Hairy Cell Leukemia

Question 33

What are examples of skin manifestations of polyarteritis nodosa?

Answer 33

Tenderness Erythematous Nodules Pupura Livedo Reticularis

Question 34

Would a patient with polyarteritis nodosa have symmetrical or asymmetrical polyneuropathy?

Answer 34

Asymmetrical

Question 35

What is a GI manifestation of polyarteritis nodosa?

Answer 35

Mesenteric Arteritis (Intestinal Angina)

Question 36

What is a cardiac manifestation of polyarteritis nodosa?

Answer 36

CAD

Question 37

Can aneurysms be present in polyarteritis nodosa?

Answer 37

Yes

Question 38

What nerve in the upper extremity can be effected in polyarteritis nodosa? What is a clinical manifestation of this?

Answer 38

Radial Nerve Wrist drop

Question 39

What are classic clinical manifestations of Polymyositis?

Answer 39

``` Heliptropic Rash (Eye Lids) Periungal Abnormalities (Nails) Gottron's Papules (Hands) ```

Question 40

________ is a syndrome associated with chronic-wide spread pain, fatigue, stiffness, poor sleep, and discreet tenderness.

Answer 40

Fibromyalgia

Question 41

For a diagnosis of fibromyalgia, a patient must have a total of _______ tender points for longer than __ ______ without another discernible cause of the pain.

Answer 41

> 11 tender points for longer than 3 months

Question 42

What THREE medications are FDA approved medications for fibromylagia?

Answer 42

Pregabalin (Lyrica) Duloxetine (Cymbalta) Milnacipran (Savella)