Autoimmune Disorders Flashcards
what is epidemiology for Polyarteritis nodosa
M>F
10% secondary to Hep B
Medium vessel necrotizing vasculitis but spares the lungs and pulmonary arteries
increase risk with + FH
what is the presentation of Polyarteritis nodosa
generalized systemic symptoms (fever, malaise, weight loss)
the LE is most commonly affected then the renal system
the symptoms are dependent on vessel involvement
if a patient with polyarteritis nodosa present with myalgias and claudication what vessels are affected
musclar arteries
if pt with PN presents with arthralgias what vessels are affected
vessels in the joints
if the pt with PN presents with renal failure and hypertension what vessels are involved
renal artery
if the patient with PN has cardiac vessel involvement how would they present
with MI, CHF and pericarditis
if a pt with PN has nerve involvement how would they present
peripheral neuropathy and foot drop
if a pt with PN has skin involvement how would they present
Livedo reticularis, ulcerations and palpable purura
if a pt with PN prsents with abd pain, GI bleeding, infarction and intestinal angina what vessel is affected
GI tract vessels
if a patient with PN presents with testicular or ovarian pain what vessels are affected
GU vessels
if a patient with PN presents with stroke/TIA, seizures and AMS what vessels are involved
CNS
in order to definitively diagnose polyarteritis nodosa what needs to be done
biopsy or angiogram
What are the supportive laboratory findings you can expect with patients with Polyarteritis nodosa
elevated ESR, CRP and anemia +/- leukocytosis
what autoimmune disease should be screened for Hep B
Polyarteritis Nodosa
How many of the ACR criteria are required for diagnosing PN
3 or more
what is the mainstay of treatment for PN
corticosteroid such as high dose prednisone
if patient is sick on presentation - start IV
what medication should be added for patients with systemic or severe PN
cyclophosphamide which is a immunosuppressive agent
what underlying disease should be treated with PN
hepatitis B with interferons or antivirals
if PN is left untreated what is the prognosis
high mortality rate
about 90% at 5 years
what factors decrease prognosis of PN
CKD with high creatinine
proteinuria
GI ischemia
CNS involvement
Cardiac involvement
what is the common population for SLE presentation
females during childbearing years and more frequently in African Americans
increase risk with +FH
what immune disease is associated with HLA
Systemic Lupus Erythematous
what are the two ways that SLE damages organs
direct antibody mediated tissue destruction or immune complexes become trapped within the vasculature
what pattern does SLE follow
relapsing-remitting course but may also remain fulminant (constant)
A patient presents with rayneauds, alopecia and a malar rash, what is their likely diagnosis
SLE
what is spared with a malar rash
nasolabial folds
if a patient presents with arthralgia without characteristic erosive changes on x-ray what is a likely diagnosis
SLE
What are the eye related symptoms associated with SLE
conjunctivitis
photophobia
transient or permanent monocular blindness
blurry vision
cotton wool spots * requires retinal exam
what are the lung related symptoms associated with SLE
Pleuritis, pleural effusion and interstitial lung disease
what are the kidney disease symptoms with SLE
glomerulonephritis and interstitial nephritis
What are the cardiac related symptoms with SLE
Heart failure, Myocarditis, pericarditis, arrhythmias
what are the GI related symptoms with SLE
Abdominal pain, mesenteric ischemia like pain (post-prandial-after eating) and illeus
what are the neurologic symptoms with SLE
psychosis, cognitive impairment, seizures, neuropathies, stroke
patient lab results are positive for anti-dsDNA and Anti-smith antibodies what is their likely diagnosis
SLE
what is a false positive syphilis test associated with
SLE
Is ANA a sensitive or specific test
Most sensitive for SLE
The New ACR and EULAR criteria require how many points to determine SLE
10 points
the old ACR criteria for SLE require how many points
4+ out of 11
what is the treatment for SLE
symptom management for specific organ involvement
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
what are second line agents for SLE
cyclophosphamide
Azathioprine
Mycophenolate mofetil
what is the prognosis for SLE
10 year survivival rate - 85%
what risk is increased with SLE
increased risk for malignancy
what is another name for systemic sclerosis
scleroderma
what is systemic sclerosis
diffuse fibrosis of the skin and internal organs
what increases the risk for developing Systemic Sclerosis
Female, African americans and a + FH
what three processes are involved with Systemic Sclerosis
microvascular injury, inflammation and autoimmunity and visceral and vascular fibrosis
What is CREST syndrome
Calcinosis
Raynauds
Esophageal dysmotility
Sclerodactlyly
Telangiectasia
What is CREST syndrome associated with
limited Systemic Sclerosis
What is involved with limited Systemic Sclerosis
confined to face, neck and distal extremities - better prognosis
what type of Systemic Sclerosis is when the trunk and proximal extremities are involved
Diffuse Systemic Sclerosis
patient presents with Raynauds,, weight loss, fatigue and arthralgia what is assumed
Diffuse SS
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
A patient has a positive Anti-SCL -70, Anticentromere and Anti-RNA antibodies what is their likely diagnosis
Systemic scerosis
What is the treatment for Systemic sclerosis
aimed for symptoms/complications - no effective treatment
What is used for the treatment of Raynauds
CCB or sildenafil
what type of SS has better outcomes
Limited has a better outcome than diffuse
what factors decrease the prognosis of scleroderma
male, African american, older age at onset, truncal involvement and progressive organ involvement
what is the common population for Sjogrens syndrome
F>M (9:1) average age 40-60
what other rheumatologic conditions are associated with Sjogrens
RA, SLE, Scleroderma, polymyositis, polyarteritis, hashimotos thyroiditis, interstitial pulmonary fibrosis and primary biliary cholangitis
what is targeted in Sjogrens syndrome
Exocrine glands - primarily lacrimal and salivary glands
Define Sicca symptoms
dry
What is keratoconjunctivitis sicca
dry eyes
what is xerostomia
dry mouth
what is polydipsia
extreme thirst
patient has positive Anti-Ro/SS - A antibodies and Anti-La/SS-B antibodies what is their likely disease
Sjogrens Syndrome
What is Schirmer’s Test
measures tear production
What other test is used for Sjogrens syndrome
Salivary gland biopsy
What is the treatment for Sjogrens syndrome
Primary symptomatic management for dryness
What are the pharmacologic options for Sjogrens
Pilocarpine (oral) - increases exocrine gland secretion
Cevimeline - increases saliva production
what is a patient with Sjogrens syndrome at greater risk for
lymphoma
