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Clin Med MSK: > Autoimmune Disorders > Flashcards

Autoimmune Disorders Flashcards

(71 cards)

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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
A patient presents with rayneauds, alopecia and a malar rash, what is their likely diagnosis
SLE
26
what is spared with a malar rash
nasolabial folds
27
if a patient presents with arthralgia without characteristic erosive changes on x-ray what is a likely diagnosis
SLE
28
What are the eye related symptoms associated with SLE
conjunctivitis photophobia transient or permanent monocular blindness blurry vision cotton wool spots * requires retinal exam
29
what are the lung related symptoms associated with SLE
Pleuritis, pleural effusion and interstitial lung disease
30
what are the kidney disease symptoms with SLE
glomerulonephritis and interstitial nephritis
31
What are the cardiac related symptoms with SLE
Heart failure, Myocarditis, pericarditis, arrhythmias
32
what are the GI related symptoms with SLE
Abdominal pain, mesenteric ischemia like pain (post-prandial-after eating) and illeus
33
what are the neurologic symptoms with SLE
psychosis, cognitive impairment, seizures, neuropathies, stroke
34
patient lab results are positive for anti-dsDNA and Anti-smith antibodies what is their likely diagnosis
SLE
35
what is a false positive syphilis test associated with
SLE
36
Is ANA a sensitive or specific test
Most sensitive for SLE
37
The New ACR and EULAR criteria require how many points to determine SLE
10 points
38
the old ACR criteria for SLE require how many points
4+ out of 11
39
what is the treatment for SLE
symptom management for specific organ involvement
40
what medications can be used for the treatment of SLE
Topical corticosteroids to treat rash NSAIDs for arthralgia mangement Hydroxycholoquine for rash and joint symptoms while also reducing number of flares oral prednisone
41
what are second line agents for SLE
cyclophosphamide Azathioprine Mycophenolate mofetil
42
what is the prognosis for SLE
10 year survivival rate - 85%
43
what risk is increased with SLE
increased risk for malignancy
44
what is another name for systemic sclerosis
scleroderma
45
what is systemic sclerosis
diffuse fibrosis of the skin and internal organs
46
what increases the risk for developing Systemic Sclerosis
Female, African americans and a + FH
47
what three processes are involved with Systemic Sclerosis
microvascular injury, inflammation and autoimmunity and visceral and vascular fibrosis
48
What is CREST syndrome
Calcinosis Raynauds Esophageal dysmotility Sclerodactlyly Telangiectasia
49
What is CREST syndrome associated with
limited Systemic Sclerosis
50
What is involved with limited Systemic Sclerosis
confined to face, neck and distal extremities - better prognosis
51
what type of Systemic Sclerosis is when the trunk and proximal extremities are involved
Diffuse Systemic Sclerosis
52
patient presents with Raynauds,, weight loss, fatigue and arthralgia what is assumed
Diffuse SS
53
For a patient to be considered to have Systemic Sclerosis what do they need to score on the ACR Criteria
9 or more is a definitive diagnosis
54
A patient has a positive Anti-SCL -70, Anticentromere and Anti-RNA antibodies what is their likely diagnosis
Systemic scerosis
55
What is the treatment for Systemic sclerosis
aimed for symptoms/complications - no effective treatment
56
What is used for the treatment of Raynauds
CCB or sildenafil
57
what type of SS has better outcomes
Limited has a better outcome than diffuse
58
what factors decrease the prognosis of scleroderma
male, African american, older age at onset, truncal involvement and progressive organ involvement
59
what is the common population for Sjogrens syndrome
F>M (9:1) average age 40-60
60
what other rheumatologic conditions are associated with Sjogrens
RA, SLE, Scleroderma, polymyositis, polyarteritis, hashimotos thyroiditis, interstitial pulmonary fibrosis and primary biliary cholangitis
61
what is targeted in Sjogrens syndrome
Exocrine glands - primarily lacrimal and salivary glands
62
Define Sicca symptoms
dry
63
What is keratoconjunctivitis sicca
dry eyes
64
what is xerostomia
dry mouth
65
what is polydipsia
extreme thirst
66
patient has positive Anti-Ro/SS - A antibodies and Anti-La/SS-B antibodies what is their likely disease
Sjogrens Syndrome
67
What is Schirmer's Test
measures tear production
68
What other test is used for Sjogrens syndrome
Salivary gland biopsy
69
What is the treatment for Sjogrens syndrome
Primary symptomatic management for dryness
70
What are the pharmacologic options for Sjogrens
Pilocarpine (oral) - increases exocrine gland secretion Cevimeline - increases saliva production
71
what is a patient with Sjogrens syndrome at greater risk for
lymphoma

Clin Med MSK: (14 decks)

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