SLE Overview

what does PML stand for?

Progressive Multifocal Leukoencephalopathy

what does SLE stand for?

Systemic Lupus Erythematous

what does xeropthalmia mean?

dry eyes

what does photophobia mean?

sensitivity of the eyes to light

what does dermatitis mean?

skin inflammation

what is a marlar rash?

“butterfly rash” on the face

what does autoimmune mean?

immune response against ones healthy body

How is Lupus defined?

it is an autoimmune disease-causing inflammation throughout the body

what is SLE?

targets tissues and skin along with organs throughout the body - has relapsing and remitting pattern of disease activity

which type of Lupus is the most common (70% of all lupus cases) and most severe?

SLE

what can SLE progress to?

lupus nephritis

what does CLE stand for?

cutaneous lupus erythematosus

what is CLE?

effects the skin causing rashes and lesions

which medications trigger drug-induced lupus erythematosus?

isoniazid, hydralazine, procainamide, quinidine, TNFai, minocycline, methyldopa

what is the most likely cause of neonatal lupus?

maternal antibodies (very rare)

why can SLE be difficult to diagnose?

has non-specific symptoms and these symptoms can vary from mild to severe

T/F: SLE is not curable

true

what is the possible life-threatening complication of SLE?

antiphospholipid syndrome

what is lupus nephritis (LN)?

severe progression of SLE that specifically attacks the kidneys

what are the broad etiologies of SLE?

complex pathways make finding etiologies difficult, environmental causes, genetic causes, hormonal links, and unknown causes

what are the major environmental contributors towards SLE?

UV radiation, silica dust, smoking, infections (Epstein bar), air pollution, pesticides, heavy metal exposure (lead and cadmium)

How do genetics play a role in SLE development?

Is best described by variation on the component 4 (C4) gene on chromosome 6 within the MHC complex, identical twins have a 25-50% change of both having SLE, epigenetics still have role to play

T/F: 90% of SLE patients are male

false

which females have the highest rates of SLE?

those of premenopausal age

estrogen promotes which type of immune responses?

type 1

T/F: estrogen acts to help stimulate B-cell maturation

true

what is prolactin’s role in the immune system?

plays a key role by promoting cytokine production and helps activate CD8+ and T-cells

T/F: alterations of sex hormones are proposed to have change of disease activity (SLE) with main effect seen in pregnant patients

true

who is more commonly diagnosed with SLE

women of child baring age account for 8.83 patients per 100,000 while men of the same age was 1.53 patients per 100,000

African American and Hispanic women are 3 times as likely to be diagnosed with SLE than Caucasian women

what is the patho of SLE?

autoreactive B cells are allowed to mature, soluble factors allow for proliferation and survival such as B cell activating factor (BAFF), T-cells are over activated and an elevated CD4:CD8 ratio is seen — from here the body will mount an immune response causing inflammation and damage to tissues and organs that it considers foreign

what is pre-clinical SLE?

increased B and T cell activation; non-specific symptoms are experienced

what is clinical SLE?

more inflammatory disease is seen due to active antibody recognition; symptoms become more specific to SLE

what occurs when there is organ involvement in SLE?

Major organ systems are adversely affected. Lupus nephritis, neurological disorders, pulmonary effects (HTN), and antiphospholipid syndrome can occur

what is the timeline for SLE?

Patients can have non-specific symptoms for an undisclosed amount of time. Patients will have relapsing and remitting disease pattern which can be aggravated with triggers.

Patients may then enter the clinical phase where the flares and symptoms become more debilitating

Organ involvement is the final stage (many pts will not get to this point)

T/F: once there is organ involvement in SLE, it can be life-threatening

true

what are the non-specific symptoms that appear first in SLE?

arthritis, photosensitivity, dry eyes, retinal changes

what are the more specific signs of SLE and are typically seen in the clinical phase?

sun exposure, infecmalar rash and oral ulcers

what are triggers of SLE?

sun exposure, infection, medications, smoking, chemical exposures, hormonal changes

how is SLE diagnosed?

antinuclear antibodies >/= 1:80 AND >/= 10 points of criteria with at least 1 clinical criteria

which test looks for antibodies that attack healthy cells in SLE?

antinuclear antibodies

T/F: antinuclear antibodies only show the presence of an autoimmune disease

true

T/F: a small percent ~10% of SLE patients will have a negative ANA result

true

what is the best test to exclude SLE?

antinuclear antibodies

what test looks at the antibodies that specifically attack the bodies double stranded DNA causing organ damage in SLE?

anti-dsDNA

T/F: anti-dsDNA is much more specific for SLE diagnosis than antinuclear antibodies

true

which test looks at antibodies that target proteins in the nucleolus of the cell that help keep its structure?

anti-smith

what is the most specific lab finding of SLE patients?

anti-smith

what are C3 and C4?

compliment proteins that are crucial for immune response

T/F: disease activity can be assessed based on C3 and C4 levels

true

what does low C3 and C4 mean?

active disease since C3 and C4 are sent to areas where inflammation is occurring causing low blood levels

T/F: C3 and C4 are specific to SLE

false

what are the self-care options for SLE?

diet rich in antioxidants (fruit) can support immune health and foods rich in anti-inflammatory properties can help with joint pain (walnuts)

exercise can improve CV health, strengthen bones and muscles

stress reduction can help in decreasing flare-ups

vaccines are important to help decrease preventable illness especially for patients using immunosuppressive therapies

med adherence is key to help control symptoms and flares

what are the short term goals for SLE?

improve quality of life, decrease the number of flares, and minimize treatment toxicities

what are the long-term goals of SLE?

slow disease progression, keep disease in remission, and decrease morbidity risk

facts regarding lupus nephritis:

inflammation causes kidney dysfunction or kidney failure

specific nephroprotective RAAS system inhibitors are used to help protect the kidneys from damage d/t inflammation

diagnosis of LN is done via urine tests for protein in the urine, blood tests for kidney function, and a kidney biopsy to confirm

T/F: active disease at the time of conception correlates to increased disease flares during pregnancy

true

what are the most recommended agents for pregnant patients with SLE?

hydroxychloroquine, azathioprine, and topical agents (non-fluorinated)

which medications used for SLE are safe for pre-conception, during pregnancy, and during lactation?

hydroxychloroquine sulfasalazine, azathioprine, cyclosporine, tacrolimus, and prednisone (if dose is < 20 mg/day)

facts regarding antiphospholipid syndrome (APS):

increase in clotting risk d/t antiphospholipid antibodies that is caused by the body attacking blood cells and lining blood vessels

higher risk in pregnancy

anticoagulation is needed to prevent clots that can cause more damage