MS - Path (SLE, Sarcoidosis, Polymyalgia rheumatica, & Fibromyalgia)

Question 1

What is the classic presentation of systemic lupus erythematosus? Give symptoms and most common patient population.

Answer 1

Classic presentation: rash, join pain, and fever, most commonly in a female of reproductive age and African descent

Question 2

What is Libman-Sacks endocarditis? With what condition is it associated?

Answer 2

Libman-Sacks endocarditis - wart-like vegatations on both sides of valve; SLE

Question 3

What type of hypersensitivity reaction is Lupus nephritis?

Answer 3

Lupus nephritis (type III hypersensitivity reaction)

Question 4

Is Lupus nephritis nephritic or nephrotic? Give specific example(s) of this.

Answer 4

Lupus nephritis (type III hypersensitivity reaction): Nephritic (diffuse proliferative glomerulonephritis) & Nephrotic (membranous glomerulonephritis)

Question 5

Name 5 types of antibodies associated with systemic lupus erythematosus.

Answer 5

(1) Antinuclear antibodies (ANA) (2) Anti-dsDNA antibodies (3) Anti-Smith antibodies (4) Antihistone antibodies (5) Anticardiolipin antibodies

Question 6

Which of the SLE-associated antibodies is sensitive but not specific?

Answer 6

Antinuclear antibodies (ANA) - sensitive, not specific

Question 7

Which of the SLE-associated antibodies is specific and indicates poor prognosis? For what organ system/disease is this prognosis?

Answer 7

Anti-dsDNA antibodies - specific, poor prognosis (renal disease)

Question 8

Which of the SLE-associated antibodies is specific but not prognostic? Against what is it directed?

Answer 8

Anti-Smith antibodies - specific, not prognostic (directed against snRNPs)

Question 9

Which of the SLE-associated antibodies is sensitive for drug-induced lupus?

Answer 9

Antihistone antibodies - sensitive for drug-induced lupus

Question 10

Which of the SLE-associated antibodies causes a false positive on test for syphilis?

Answer 10

Anticardiolipin antibodies - false positive on test for syphilis

Question 11

Besides the test for syphilis, what other test can anticardiolipin antibodies effect? Explain the paradox in this.

Answer 11

Anticardiolipin antibodies - false positive on test for syphilis, prolonged PTT (paradoxically, increased risk of arteriovenous thromboembolism)

Question 12

What 3 factors/substances are decreased in SLE, and why?

Answer 12

Decreased C3, C4, and CH50 due to immune complex formation

Question 13

What are 4 treatments for systemic lupus erythematosus?

Answer 13

(1) NSAIDs (2) Steroids (3) Immunosuppressants (4) Hydroxychloroquine

Question 14

What are the 10-12 important associations to make with systemic lupus erythematosus? Hint: Mnemonic

Answer 14

RASH OR PAIN: (1) Rash (malar or discoid) (2) Arthritis (3) Soft tissues/serositis (4) Hematologic disorders (e.g., cytopenia) (5) Oral/nasopharyngeal ulcers (6) Renal disease, Raynaud phenomenon (7) Photosensitivity, Positive VDRL/RPR (8) Antinuclear antibodies (9) Immunosuppressants (10) Neurologic disorders (e.g., seizures, psychosis)

Question 15

What are the 3 common causes of death in SLE?

Answer 15

Common causes of death in SLE: (1) Cardiovascular disease (2) Infections (3) Renal disease

Question 16

What are the 2 types of rash seen in SLE?

Answer 16

Rash (malar or discoid)

Question 17

What is an example of hematologic disorders seen in SLE?

Answer 17

Hematologic disorders (e.g., cytopenias)

Question 18

What are 2 examples of neurologic disorders seen in SLE?

Answer 18

Neurologic disorders (e.g., seizures, psychosis)

Question 19

What are 5 antibodies found in SLE? What are the major properties and/or clinical utility of each antibody?

Answer 19

(1) Antinuclear antibodies (ANA) - sensitive, not specific (2) Anti-dsDNA antibodies - specific, poor prognosis (renal disease) (3) Anti-Smith antibodies - specific, not prognostic (directed against snRNPs) (4) Antihistone antibodies - sensitive for drug-induced lupus (5) Anticardiolipin antibodies - false positive on tests for syphilis, prolonged PTT (paradoxically, increase risk of arteriovenous thromboembolism)

Question 20

With what SLE complication are Anti-dsDNA antibodies associated?

Answer 20

Anti-dsDNA antibodies - specific, poor prognosis (renal disease)

Question 21

Against what are Anti-Smith antibodies targeted? In what condition are they found?

Answer 21

Anti-Smith antibodies - specific, not prognostic (directed against snRNPs); SLE

Question 22

In what condition are anticardiolipin antibodies found? For what test can they create a false positive? What hematologic lab finding do they cause? What risk do they increase?

Answer 22

SLE; Anticardiolipin antibodies - false positive on tests for syphilis, prolonged PTT (paradoxically, increase risk of arteriovenous thromboembolism)

Question 23

What 3 factors are decreased in SLE, and why?

Answer 23

Decreased C3, C4, and CH50 due to immune complex formation

Question 24

What are 4 treatments for Systemic lupus erythematosus?

Answer 24

(1) NSAIDs (2) Steroids (3) Immunosuppressants (4) Hydroxychloroquine

Question 25

What histologic finding characterizes sarcoidosis? What lab finding characterize sarcoidosis?

Answer 25

Characterized by immune-mediated, widespread noncaseating granulomas and elevated serum ACE levels

Question 26

In what patient population is sarcoidosis common?

Answer 26

Common in black females

Question 27

How does sarcoidosis often present?

Answer 27

Often asymptomatic except for enlarged lymph nodes

Question 28

What are 2 CXR findings that indicate sarcoidosis, and how are they found?

Answer 28

Incidental findings on CXR of BILATERAL HILAR ADENOPATHY &/or reticular opacities

Question 29

What are 7 conditions associated with sarcoidosis?

Answer 29

Associated with (1) restrictive lung disease (interstitial fibrosis), (2) erythema nodosum, (3) lupus pernio, (4) Bell palsy, (5) epithelioid granulomas containing microscopic Schaumann and asteroid bodies, (6) uveitis, and (7) hypercalcemia (due to increased 1alpha-hydroxylase-mediated vitamin D activation in macrophages).

Question 30

What causes hypercalcemia in sarcoidosis?

Answer 30

Hypercalcemia (due to increased 1alpha-hydroxylase-mediated vitamin D activation in macrophages)

Question 31

What type of lung disease is associated with sarcoidosis? Give an example.

Answer 31

Restrictive lung disease (interstitial fibrosis)

Question 32

What is the treatment for sarcoidosis?

Answer 32

Treatment: Steroids

Question 33

What are 5 symptoms of polymyalgia rheumatica?

Answer 33

(1) Pain and (2) stiffness in shoulders and hips, often with (3) fever, (4) malaise, and (5) weight loss

Question 34

In what patient population is sarcoidosis more common?

Answer 34

More common in women > 50 years old

Question 35

With what condition is polymyalgia rheumatica associated?

Answer 35

Associated with temporal (giant cell) arteritis

Question 36

What are 3 lab findings that characterize polymyalgia rheumatica?

Answer 36

(1) high ESR (2) high C-reactive protein (3) normal CK

Question 37

What is the treatment for polymyalgia rheumatica, and how do patients respond to this?

Answer 37

Rapid response to low-dose corticosteroids

Question 38

In what patient population is fibromyalgia most commonly seen?

Answer 38

Most commonly seen in females 20-50 years old

Question 39

What kind of pain characterizes fibromyalgia? What are 4 other symptoms associated with this?

Answer 39

Chronic, widespread musculoskeletal pain associated with stiffness, paresthesias, poor sleep, and fatigue.

Question 40

What are 3 treatments for fibromyalgia?

Answer 40

Treat with regular exercise, antidepressants (TCAs, SNRIs), and anticonvulsants.

Question 41

What are 2 examples of antidepressants used to treat fibromyalgia?

Answer 41

Antidepressants (TCAs, SNRIs)