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Clin Med MSK: > Autoimmune Disorders > Flashcards

Autoimmune Disorders Flashcards

1
Q

what is epidemiology for Polyarteritis nodosa

A

M>F
10% secondary to Hep B
Medium vessel necrotizing vasculitis but spares the lungs and pulmonary arteries
increase risk with + FH

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2
Q

what is the presentation of Polyarteritis nodosa

A

generalized systemic symptoms (fever, malaise, weight loss)
the LE is most commonly affected then the renal system
the symptoms are dependent on vessel involvement

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3
Q

if a patient with polyarteritis nodosa present with myalgias and claudication what vessels are affected

A

musclar arteries

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4
Q

if pt with PN presents with arthralgias what vessels are affected

A

vessels in the joints

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5
Q

if the pt with PN presents with renal failure and hypertension what vessels are involved

A

renal artery

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6
Q

if the patient with PN has cardiac vessel involvement how would they present

A

with MI, CHF and pericarditis

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7
Q

if a pt with PN has nerve involvement how would they present

A

peripheral neuropathy and foot drop

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8
Q

if a pt with PN has skin involvement how would they present

A

Livedo reticularis, ulcerations and palpable purura

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9
Q

if a pt with PN prsents with abd pain, GI bleeding, infarction and intestinal angina what vessel is affected

A

GI tract vessels

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10
Q

if a patient with PN presents with testicular or ovarian pain what vessels are affected

A

GU vessels

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11
Q

if a patient with PN presents with stroke/TIA, seizures and AMS what vessels are involved

A

CNS

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12
Q

in order to definitively diagnose polyarteritis nodosa what needs to be done

A

biopsy or angiogram

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13
Q

What are the supportive laboratory findings you can expect with patients with Polyarteritis nodosa

A

elevated ESR, CRP and anemia +/- leukocytosis

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14
Q

what autoimmune disease should be screened for Hep B

A

Polyarteritis Nodosa

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15
Q

How many of the ACR criteria are required for diagnosing PN

A

3 or more

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16
Q

what is the mainstay of treatment for PN

A

corticosteroid such as high dose prednisone
if patient is sick on presentation - start IV

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17
Q

what medication should be added for patients with systemic or severe PN

A

cyclophosphamide which is a immunosuppressive agent

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18
Q

what underlying disease should be treated with PN

A

hepatitis B with interferons or antivirals

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19
Q

if PN is left untreated what is the prognosis

A

high mortality rate
about 90% at 5 years

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20
Q

what factors decrease prognosis of PN

A

CKD with high creatinine
proteinuria
GI ischemia
CNS involvement
Cardiac involvement

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21
Q

what is the common population for SLE presentation

A

females during childbearing years and more frequently in African Americans
increase risk with +FH

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22
Q

what immune disease is associated with HLA

A

Systemic Lupus Erythematous

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23
Q

what are the two ways that SLE damages organs

A

direct antibody mediated tissue destruction or immune complexes become trapped within the vasculature

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24
Q

what pattern does SLE follow

A

relapsing-remitting course but may also remain fulminant (constant)

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25
Q

A patient presents with rayneauds, alopecia and a malar rash, what is their likely diagnosis

A

SLE

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26
Q

what is spared with a malar rash

A

nasolabial folds

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27
Q

if a patient presents with arthralgia without characteristic erosive changes on x-ray what is a likely diagnosis

A

SLE

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28
Q

What are the eye related symptoms associated with SLE

A

conjunctivitis
photophobia
transient or permanent monocular blindness
blurry vision
cotton wool spots * requires retinal exam

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29
Q

what are the lung related symptoms associated with SLE

A

Pleuritis, pleural effusion and interstitial lung disease

30
Q

what are the kidney disease symptoms with SLE

A

glomerulonephritis and interstitial nephritis

31
Q

What are the cardiac related symptoms with SLE

A

Heart failure, Myocarditis, pericarditis, arrhythmias

32
Q

what are the GI related symptoms with SLE

A

Abdominal pain, mesenteric ischemia like pain (post-prandial-after eating) and illeus

33
Q

what are the neurologic symptoms with SLE

A

psychosis, cognitive impairment, seizures, neuropathies, stroke

34
Q

patient lab results are positive for anti-dsDNA and Anti-smith antibodies what is their likely diagnosis

A

SLE

35
Q

what is a false positive syphilis test associated with

A

SLE

36
Q

Is ANA a sensitive or specific test

A

Most sensitive for SLE

37
Q

The New ACR and EULAR criteria require how many points to determine SLE

A

10 points

38
Q

the old ACR criteria for SLE require how many points

A

4+ out of 11

39
Q

what is the treatment for SLE

A

symptom management for specific organ involvement

40
Q

what medications can be used for the treatment of SLE

A

Topical corticosteroids to treat rash
NSAIDs for arthralgia mangement
Hydroxycholoquine for rash and joint symptoms while also reducing number of flares
oral prednisone

41
Q

what are second line agents for SLE

A

cyclophosphamide
Azathioprine
Mycophenolate mofetil

42
Q

what is the prognosis for SLE

A

10 year survivival rate - 85%

43
Q

what risk is increased with SLE

A

increased risk for malignancy

44
Q

what is another name for systemic sclerosis

A

scleroderma

45
Q

what is systemic sclerosis

A

diffuse fibrosis of the skin and internal organs

46
Q

what increases the risk for developing Systemic Sclerosis

A

Female, African americans and a + FH

47
Q

what three processes are involved with Systemic Sclerosis

A

microvascular injury, inflammation and autoimmunity and visceral and vascular fibrosis

48
Q

What is CREST syndrome

A

Calcinosis
Raynauds
Esophageal dysmotility
Sclerodactlyly
Telangiectasia

49
Q

What is CREST syndrome associated with

A

limited Systemic Sclerosis

50
Q

What is involved with limited Systemic Sclerosis

A

confined to face, neck and distal extremities - better prognosis

51
Q

what type of Systemic Sclerosis is when the trunk and proximal extremities are involved

A

Diffuse Systemic Sclerosis

52
Q

patient presents with Raynauds,, weight loss, fatigue and arthralgia what is assumed

A

Diffuse SS

53
Q

For a patient to be considered to have Systemic Sclerosis what do they need to score on the ACR Criteria

A

9 or more is a definitive diagnosis

54
Q

A patient has a positive Anti-SCL -70, Anticentromere and Anti-RNA antibodies what is their likely diagnosis

A

Systemic scerosis

55
Q

What is the treatment for Systemic sclerosis

A

aimed for symptoms/complications - no effective treatment

56
Q

What is used for the treatment of Raynauds

A

CCB or sildenafil

57
Q

what type of SS has better outcomes

A

Limited has a better outcome than diffuse

58
Q

what factors decrease the prognosis of scleroderma

A

male, African american, older age at onset, truncal involvement and progressive organ involvement

59
Q

what is the common population for Sjogrens syndrome

A

F>M (9:1) average age 40-60

60
Q

what other rheumatologic conditions are associated with Sjogrens

A

RA, SLE, Scleroderma, polymyositis, polyarteritis, hashimotos thyroiditis, interstitial pulmonary fibrosis and primary biliary cholangitis

61
Q

what is targeted in Sjogrens syndrome

A

Exocrine glands - primarily lacrimal and salivary glands

62
Q

Define Sicca symptoms

A

dry

63
Q

What is keratoconjunctivitis sicca

A

dry eyes

64
Q

what is xerostomia

A

dry mouth

65
Q

what is polydipsia

A

extreme thirst

66
Q

patient has positive Anti-Ro/SS - A antibodies and Anti-La/SS-B antibodies what is their likely disease

A

Sjogrens Syndrome

67
Q

What is Schirmer’s Test

A

measures tear production

68
Q

What other test is used for Sjogrens syndrome

A

Salivary gland biopsy

69
Q

What is the treatment for Sjogrens syndrome

A

Primary symptomatic management for dryness

70
Q

What are the pharmacologic options for Sjogrens

A

Pilocarpine (oral) - increases exocrine gland secretion
Cevimeline - increases saliva production

71
Q

what is a patient with Sjogrens syndrome at greater risk for

A

lymphoma

Clin Med MSK: (14 decks)

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