-multi-system autoimmune disorder of unknown cause

-characterized by immune complex formation and resulting inflammation

-influenced by hormonal, genetic, immunological, and possibly environmental factors

-immune complex formation and abnormalities in the complement system support immunologic origin 

-90% of cases are female in childbearing years 

-can occur at any age, but most commonly 20's, 30's and 40's

-African americans are most susceptible 

-individuals with famhx of auto-immunity

-identical twins, children of SLE patients

-any organ system can be involved 

-can be slowly progressive disease or severe and rapidly progressive, life-threatening 

-lupus nephritis, lupus cerebritis, pulm. hemorrhage, and small vessel vasculitis of brain or mesentery are most severe complications 

-pregnancy can cause severe disease flares

-need 4/11:

-malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal disorder, neurologic disorder, hematologic disorder, immunologic disorder, antinuclear antibody (ANA)

SOAP BRAIN MDrash

-usually non-erosive, inflammatory

-big joints and hands

X-ray: no erosions, osteopenia, no joint destruction (which is seen in RA)

-Red rash: photoexposed regions such as face, arms, chest, back

-Discoid: scalp, ears, extensor surfaces

-Subacute cutaneous on trunk and extremities

-Malar rash: "butterfly rash"

**any type of rash can be seen in SLE 

-99% of patients with SLE test positive for ANA when a sensitive assay is used 

-used as diagnostic criteria 

-no actual treatment

-aim is to reduce inflammation, suppress immune system, closely monitor possible manifestations of SLE 

-meds include immuno-suppressants and glucocorticoids, need to monitor for toxicities

-anti-malarial drugs may be effective in some pts. 

-patient education and prophylaxis are key 

-routine immunizations

-inflammation and destruction of lacrimal and salivary glands 

-results in xerophthalmia (dry eyes) and xerostomia (dry mouth)

-can also exhibit ILD, vasculitis, and lymphoma 

-affects women more commonly but can affect men as well

-found in all ages, races, ethnicities

-secondary Sjogren's = these sx. along with diagnosis of SLE, RA, scleroderma, or primary biliary cirrhosis 

-primary = no underlying immunologic disorder is present 

-dry, irritated eyes; corneal abrasions

-dry mouth; oral sores; dysphagia, GERD

-dyspareunia 

-pancreatic malabsorption

**first two, by far most common and annoying manifestations 

-association w/ increased prevalence of lymphoma

-usually B-cell types

-need to be aware of this

-look for changes in swelling, constitutional symptoms, or resistant treatment patterns 

-many different diagnostic criteria out there; but commonly:

1. subjective and objective dry eyes and mouth

2. presence of at least 1 of the following 4 autoantibodies: ANA, RA, anti-Ro (SS-A), anti-La (SS-B)

3. exclusion of underlying diseases that may mimic this

-biopsy may help to confirm presence of CD4 T cells 

-Dry eyes: artificial tears, eyeglasses/goggles, punctal occlusion 

-Dry mouth: artificial saliva, fluoride treatments, avoiding candies

-Dyspareunia: vaginal lubricant

Systemic tx: pilocarpine or cevimeline

-may also consider NSAIDS, anti-malarial, steroids, immunosuppressive agents 

-multi-system disorder characterized by auto-immunity and immune cell activation; also called systemic sclerosis

-characterized also by vascular damage and dysfunction (Raynaud's) 

-cutaneous and visceral fibrosis 

-"scleroderma" means thick skin, in which shiny, thickened skin can be seen in patients 

-more common in women, 3-4 x

-onset <30 is unusual, and incidence rises slowly between 40's and 70's

-familial occurrence is unusual, but more commonly, other autoimmune disease will be seen in other family members 

3 aspects:

1. metabolic defect in fibroblast metabolism leading to overproduction of collagen and other matrix proteins --> dermal and internal organ fibrosis

2. vascular lesion: damage and dysfunction (intimal proliferation, luminal narrowing, Raynaud's)

3. immune cell activation and autoimmunity 

4. autoimmunity: ANA, centromere, SCL-70

(CREST) syndrome

-cutaneous calcinosis: limited to fingers, toes, face

-Raynaud's (95-100% of cases); ischemia, digital ulcers

-esophageal dysmotility/hypomotility 

-sclerodactyly: thickening and tightening of skin

-telangiectasia: dilated blood vessels, can occur on hands, face, chest 

-anticentromere antibodies 

-skin induration is widespread: extremities, trunk, face

-Raynaud's 

-GI involvement: hypomotility and malabsorption

-ILD: MAJOR cause of death (~30-40%)

-renal involvement (~30%)

-pulm. HTN

-cardiac fibrosis

-myositis

-SCL-70 antibodies 

-usually directed at specific organs involved

-Ca2+ channel blockers

-ACE inhibitors

-prostacyclin and anticoagulant therapy

-anti-fibrotic agents

-antibiotics for infections

-PPI's

-clinical diagnosis suggested by presence of Raynaud's, esophageal reflux, and sclerodactyly 

-presence of telangiectasia and ANA antibodies suggest risk of developing scleroderma

-ANA is NOT specific for this diagnosis

-distinguishing between limited and diffuse may be difficult