Week 5 tuesday Flashcards
2 factors lead to autoimmune:
- Inheritance of susceptible gene
2. Environmental triggers
Infection can cause autoimmune by:
- Upregulate expression of costimulators on APC
- Molecular mimicry
- Cause polyclonal activation
Mechanism of SLE:
- System lesion from antibodies on dsDNA and smith antigens form immune complex (type 3)
- Antibodies against RBC, WBC and platelet. Opsonize and lead to cytopenia (type 2)
Neutrophil extracellulr trap in response to inflammation. release nuclear antigens
Problems with anti-phospholipid antibodies in SLE
Hypercoagulation, leading to clot and ischemia
Clinical skin features of SLE
- Erythema in light exposed areas
2. immune complex on dermoepidermal junction
Clinical kidney features in SLE
Nephritis due to immune complex deposition in glomeruli
Clinical joint features in SLE
NON erosive and NON deforming small joint involvement
Clinical hematologic system in SLE
Pericarditis, endocarditis, atherosclerosis
SOAP BRAIN MD
SEROSITIS ORAL ULCERS ARTHRITIS PHOTOSENSITIVITY BLOOD CELLS RENAL, RAYNAUDS ANA IMMUNOLOGIC NEUROPSYCH MALAR RASH DISCOID RASH
Mechanism of RA
Systemic inflammatory disorder against joints, producing NONSUPPORATIVE proliferative and inflammatory synovities that leads to DESTRUCTION of articular cartilage and ankylosis
Major genetic susceptibility
Best test for RA
NOT rheumatoid factor
Test the ACCP = antibody cyclic citrullinated proteins
Pathologic findings seen in RA joints
T and B cells form a PANNUS (mass of inflamed synovium) that grows over joint cartialge and leads to inflammation and destruction.
RA nodule pathology
Area of central fibrinoid necrosis surrounded by a PALISADE OF MACROPHAGES and scattered chronic inflammatory cells
Clinical presentation of Sjogren syndrome
DRY eyes and DRY mouth
Mostly in middle ages woman
Pathology of sjogren syndrome and antibodies found
Autoimmune demediated destruction of LACRIMAL and SALIVARY glands
Often a secondary syndrome from other autoimmune. RA most common
Antibodies to ribonucleoproteins SS-A and SS-B are measured with LIP BIOPSY
Neoplasm associated with Sjogren syndrome
LYMPHOMA (typically marginal zone)
CREST syndrome
Can develop from limited scleroerma
Calcinosis Raynauds phenomenom Esophageal dysmotility Sclerodactylyl Telangiectasia
Diffuse vs Limited scleroderma
Diffuse: WIDESPREAD skin involvement at onset, RAPID progression to visceral
Limited: Skin involvement CONFINED to fingers, forearms and face. Follow by LATE visceral involvement. Can lead to CREST
Systemic sclerosis pathology
Unknown
Widespread damage to small blood vessels and progressive interstitial perivascular firbrosis of skin and organs. Patho findings are secondary to ischemic damage and fibrosis to organs
MAY have antibodies to SCL-70 (DNA TOPO 1 )
CREST may have anticentromere antibody
Most common cause of death from Systemic sclerosis
Respiratory failure from interstitial fibrosis
Clinical presentation of dermatomyositis vs myositis
dermatomyositis: muscle weakness and skin rash (discolor eye lids, red patches on knuckles, elbows and knees)
Myositis: Same, but WITHOUT skin involvement
Lab findings and treatment for dermatomyositis and myositis
Elevated creatine kinase
Treat with immunosuppressives
Antibody for Mixed connective tissue disease
Overlap of features with distinctive ANTI-U1-RNP antibody
CATU / centromere antibodies found in:
CREST syndrome
SS-A AND SS-B antibodies support:
Sjogren syndrome
Jo 1 antibodies support
POLYMYOSITIS
Scl 70 antibodies supports:
scleroderma
Sm antibodies and dsDNA antibodies support
LUPUS
primary vs secondary immunodefiency
primary: congenital or genetic
secondary: cancer, infection chemo, etc
Primary mainly found in INFANTS 6mo-2yr
X-linked agammaglobulinemia (brutons)
failure of bcell precursors
X LINKED GENE FOR cytoplasmic tyrosine kinase
recurrent bacterial infections. Males > females
variable immunodefiency
failures of bcells to differentiate. both sexes equally. later onset of symptoms
Digeorge syndrome
failure of 3 and 4 pouch to develop = no thymus.
Loss of tcells
congenital heart and vessel problems
facial abnormalities
Gene problem with digeorge syndrome
Sporadic deletion of chromosome 22q11
CD40 ligand xlinked recessive mutation
Hyper IgM syndrome. Can not swtich to other Ig. Only IgM found
Defect in gamma chain of cytokine receptor
SCID
autosomoal recessive
Defiency in enzyme adenosine deaminase
Treat with hemato cell transplant
Loss of spleen and risk of infections?
Encapsulated bacteria, esp streptocossus pneumoniae
recieve vaccinations for s pneumoniae, h influenzae and n meningtidis
Lead inhibits:
aminolevulinate dehydratase (ALA)
Ferrochelatase
Both reduce heme production as a result
Lead poisoning urine results
elevated protoporphyrin and ALA
elevated erythrocyte protoporphyrin found early in both kids and adults
