Systemic Autoimmune Disease Flashcards
What does ANA mean?
Anti-Nuclear Antibody
An immune complex disease is what type of immune injury?
Type 3
Type 3 immune injuries hit which organs (2)?
Vessels
Kidneys
Type 2 immune injury is involved in what type of reactions? And what do they cause
Complement mediated that result in localized tissue infiltrates and damage
Type 4 immune injury present what kind of infiltrates?
Lymphocytic
Healing and regeneration requires what two steps?
Organization of fibrinoid deposits
Interstitial fibrosis
In SLE what happens to B cells to produce pathogenesis in SLE?
Activation of multiple clones of B cells
Possibly due to loss of normal suppressor T cell mechanisms
Most characteristic formulation of Antibodies in SLE?
Autoantibodies to double stranded DNA
What causes loss of normal hematological elements in SLE?
Autoantibodies that go after RBCs, neutrophils, and platelets
What test is used to show deposition of antibodies in basement membrane of the skin in SLE?
Immunofluorescence techniques (positive band test) Normal people can show these too so beware
What causes heart problems in SLE?
Antibodies to hyaluronic acid
What is specificity rule for deposition of immune complexes in the kidney and small blood vessels of the spleen?
Regardless of the specificity of the antibody immune complexes here will lead to type 3 mediated lesions.
What causes the tissue damage in SLE?
Combination of Type 2 (cytotoxic) and type 3 (immune complex mechanisms)
Lesions in SLE result in what involving complement?
Fixation of the complement
Fixation of complement in SLE results in what?
Low total complement level (hypocomplementemia)
SLE primarily affect whom?
Women of reproductive age
Most frequent manifestation of SLE?
Arthritis and arthralgia
Attacks self limited and DO NOT lead to chronic inflammatory destruction of joint with pannus formation
Progression of SLE?
Episodic and remitting attacks, gets more frequent, increases in number of systems involved, organ failure
Death in SLE results normally from?
Renal/cardiac failure
Characteristic rash is where in SLE?
Malar rash across nose and cheeks
What does every or nearly every patient with SLE test positive for?
ANA, but not diagnostic
What is diagnostic of SLE?
Antibodies to double stranded DNA
What ANA staining pattern would show binding to double stranded DNA?
Rim or peripheral staining patter
Progressive renal failure in SLE is heralded by what?
Proteinuria
Decrease in creatinine clearance
What are hematoxylin bodies?
Inclusion bodies of phagocytes formed by phagocytosis of leukocytes damaged by ANAs
What leads to reactive skin lesions from deposited immune complexes in SLE?
UV light
Four types of Renal disease patterns in SLE?
Mesangial (Mildest)
Focal Proliferative
Diffuse Proliferative (Worst)
Membranous
What results in Onion skin concentric rings of fibrin deposition around small arterioles
Repeated injury resulting from immune complex deposition the walls of small blood vessels in the spleen
What is Libman-Sacks endocarditis in SLE?
1-3 mm warty deposits on either surface of any of the heart valves
What is anti-phospholipid syndrome?
Anti-phospholipid or anti-cardiolipin antibodies
In anti-phopholipid syndrome in SLE how do the antibodies work?
In vitro they interfere with coagulation
In vivo they promote coagulation
In chronic discoid lupus what is the characteristic lesion
Disc shaped scaling lesions usually above neck
Are discoid lesions diagnostic of Chronic discoid lupus?
No, identical lesions can appear in systemic lupus
Drug induced lupus is what?
Immune hypersensitivity to drug (Isoniazid, Penicillamine, and Procainamide)
What resolves Drug induced lupus?
Remove use of the drug
Sjogren’s syndrome destroys what structures?
Lacrimal and salivary glands
What is Sicca syndrome associated with Sjogren?
Dry eyes, dry mouth, difficulty swallowing
What causes the decrease of tears and saliva in Sjogrens?
Lymphocytic infiltration and fibrosis of lacrimal and salivary glands
Which autoantibodies are most important in Sjogren’s?
Anti-ribonucelar protein antibodies SS-A and SS-B
High SS-A associated with extra glandular manifestations
Important clinical feature of Sjogren’s?
Mikulicz’s syndrome: lacrimal and salivary gland enlargement of any cause
Sjogren’s involvement in B-Cells?
Pseudolymphoma, 40 fold increase in risk of developing lymphoid malignancies
What do you do to diagnose for Sjogren?
Biopsy the lip to examine minor salivary glands
What is Progressive Systemic Sclerosis (Scleroderma)?
Excessive fibrosis through body, but skin is primary manifestation
What are the two major patterns of Scleroderma?
Diffuse with early visceral involvement
Localized with limited skin confined to fingers, forearms, and face with late visceral involvement
What is CREST?
Mnemonic for symptoms of Scleroderma: Calcinosis Raynaud's Esophageal dysmotility Sclerodactyly Telangiasctasia
Scleroderma associated with what?
Deposition of collagen, cause unknown
Distinctive feature of Scleroderma?
Cutaneous involvement involving hands face
Raynauds precedes other symptoms
Progression of Scleroderma?
Fibrosis of dermis -> Increased compact collagen -> Thinning of epidermis -> Focal calcifications -> Loss of blood supply to fingers may cause autoamputation
Diagnostic feature for Scleroderma?
Anti-RNP (Ribonucleoprotein)
SS-A, SS-B
Most ominous manifestation of renal involvement in Scleroderma?
Malignant hypertension with subsequent renal failure
