AUTOIMMUNE DISEASE Flashcards
(37 cards)
autoimmune ds
immune response to self antigens
autoimmune disease v. immunodeficiency
AUTOIMMUNE DS
- immune response to self antigens
- in context of genetic predisposition resulting in ds expression (organ or system)
IMMUNODEF
- primary is direct consequence of structural or functional issue in immune network
- secondary conseq of alteration in immune status resulting in manifestation of infectious ds
systemic- connective tissue
systemic lupus erythematosus
- organs involved
- patho
- multisystem: inflamm to skin, joints, kidney, cardiovasc system, nervous system, lung and hemopoeitic cells
- production of antibodies to a variety of nuclear and cytoplasmic antigens
- antibodies to ds DNA
systemic lupus erythematosus
- SS
- dx/labs
SS
- BUTTERFLY RASH, pleural effusions, heart problems, lupus nephritis, arthritis, raynauds
Dx
- rash, arthritis, oral ulcers (SIGNS)
- proteinuria, cytopenia, pos anti-ds DNA, pos anti phospholipid antibody, pos anti-Sm antibody, false pos syph, abnorm ANA
systemic lupus erythematosus
- ANA
ANA is 95% pos in lupus patients, BUT does not show specificity
- can still be negative for an actual autoimmune disease
best dx with combo of findings and markers to confirm this ds
systemic- connective tissue
sjogren syndrome
- MC in?
- organs involved
- MC women>men
- autoimmune excrinopathy involves lacrimal glands, salivary glands, pancreas
- primary or assoc w other autoimmune ds
sjogren syndrome
- SS
- dx/labs
SS:
- sicca syndrome (dry eyes), dry throat/skin/nose, dry mouth + inc tooth decay
- additional SS: recurrent bronchitis, joint pain, digestive problems, muscle pain, vaginal dryness, neuro issues
Dx:
- pos ANA, pos SS-A v. SS-B, pos rheumatoid factor
DRYNESS wherever there should be MOISTURE
sjorgen syndrome
- tests/labs
- test for sicca, dry eye schirmer test (capillary action/moisture in lower eyelid)
- bx salivary gland
- complication: 2.5% develop non hodgkin lymphoma
systemic- connective
scleroderma
- organs involved
excessive and widespread deposition of collagen in many organ systems
- involves skin, GI tract, kidney (renal ischemia), lung and muscles
3 main characteristics:
- tissue fibrosis, prolif and occlusive vasculopathy of small BVs, specific autoimmune response assoc with distinc autoatinbody profile
systemic- connective
scleroderma
- subtypes
- Dx/labs
CREST
- Calcinosis: calcium deposits in skin/joint spaces
- raynauds: spasm of BVs in response to cold or stress
- Esoph dysfunc: acid reflux, dec motility of esophagus
- Sclerodactyly: thickening and tightening of skin on fingers and hands
- Telangiectasias: dilation of capillaries, causes red marks on skin surface
Dx
- pos ANA, antibody Scl-70
systemic- connective
inflamm muscle ds
- 3 types and commonly assoc SS
- dermatomyositis: heliotrope rash, shawl sign, gottron papules, complement vasculopathy
- polymyositis: T cell mediated muscle injury
- inclusion body myositis
inflamm muscle ds
- criteria needed
- labs
3/5 needed for Dx
- proximal muscle weakness
- muscle pain and tenderness on palpation
- EKG abnorm
- elevated serum/plasma conc of muscle enzymes
- muscle bx with cellular inflamm
labs
- pos ANA, anti-Jo
systemic- connective
mixed connective tissue ds
- SS
- Dx/labs
SS:
- arthralgias, myalgias, fatigue, raynauds
- pulm: interstitial pneumonitis, pulm HTN, interstitial fibrosis, diaphragm and esoph dysfunc
dx:
- clin features, pos ANA, anti-U1 RNP
- combined features of SLE, sclerosis, and polymyositis
systemic- connective
rheumatoid arthritis
- affects what
- MC in?
- SS
- Dx/labs
- affects synovial joints often starting as a synovitis
- MC young women
SS:
- 3/7 criteria with morning stiffness
- hand deformities due to destruction of synovial joint spaces/degraded cartilage
Dx/labs:
- inc serum rheumatoid factor, anti-CCP
systemic- connective
amyloidosis
- organs invovled
-
amyloid proteins deposit extracellularly, cause waxy appearance to infiltrated organs
- multiorgan involvement
- factor X coagulopathy
amyloidosis
- SS
- Dx/labs
SS:
- fatigue/dizzy, SOB, proteinuria, BP changes, wt loss, stiff heart, kidney issues
Dx
- congo red stain on microscopy
- abdominal fat pad bx (checking for amyloid deposits)
systemic- connective
cryoglobulinemia
- SS
- Dx/labs
immunoglobulins in serum that precipitate/clump at temps below 37 C–> resolves w/ warmth
SS
- immune complex vasculitis,a rthritis, neuropathy, renal involvement
Dx/labs
- wintrobe tube collects cryoglobulin, reported as cryocrit
ds of immune system
x linked agammaglobulinemia (XLA)
absence of b lymphocytes in males
- results in recurrent infections (inc susceptibility)
- XLA stops B cell maturation in bone marrow (pre B cell to immature B cell stopped)
ds of immune system
common variable immunodeficiency
- SS and labs affected
ADULT males and females
- B cells are dysfunctional
- result in recurrent infx, immune hemolytic anemia, neutropenia, pernicious anemia, B cell lymphoma
SS
- chronic cough, swollen LN, ear pain, N/V/D, wt loss
ds of immune system
hyper IgM syndrome
- labs
B cells cant undergo antibody class switching
- low IgG and IgA
- normal or high IgM
- normal B cells
ds of immune system
selective IgA deficiency
- SS
- Dx/labs
MC primary immunodef syndrome
SS:
- Some asymp, others have allergies, autoimmune ds, recurrent infxs, GI ds
Dx/labs:
- IgA<5 w/normal IgG and IgM
ds of immune system
DiGeorge syndrome
- SS
- labs
chromosome 22 deletion
catch 22:
- cardiac abnorm, abnorm facies, thymic hypoplasia, cleft palate, hypocalcemia
- t cell abnorm, B cell dysfunc, parathyroid abnorm
ds of immune system
severe combined immunodef syndrome (SCID)
- tx and Dx
cellular and humoral immunity defects
(defect in development of T+B cells)
- bubble babies–> very susceptible to infx
- Tx: BM transplant
- Dx: neonate heel prick test
complement system
protects against pathogens and non self antigens
- fights against infx by cytokine release, stim of phagocytes, inflamm, cell killing attack
