Immune diseases Flashcards
(26 cards)
SLE
Type III hypersensitivity (immune complex)
- antigen: DNA, nucleoproteins
- clinical manifestation: nephritis, arthritis, vasculitis
Post-streptococcal glomerulonephritis
Type III hypersensitivity (immune complex)
- antigen: strep cell wall antigen released into circulation after strep infection, bound by antibody to form immune complex that is deposited into small blood vessels of kidney
- clinical manifestation: nephritis
Systemic immune complex disease
Type III hypersensitivity (immune complex)
aka acute serum sickness
seen in the past following vaccination with horse serum, systemic immune complex disease caused by antigen excess
clinical manifestation: arthritis, rash, fevers
Arthus reaction
Type III hypersensitivity (immune complex)
local severe acute immune complex mediated vasculitis associated with necrosis
circulating antibodies present in a patient react with injected antigen (immunization)
edema, hemorrhage, frank ulceration of skin at injection site
Polyarteritis nodosa
Type III hypersensitivity (immune complex)
antigen: hepatitis surface antigen
clinical manifestation: vasculitis
Reactive arthritis
Type III hypersensitivity (immune complex)
antigen: yersinia bacterial antigen
clinical manifestation: acute arthritis
TB skin reaction
Delayed type (type IV) hypersensitivity - if prior sensitization to TB, red induration at injection site with hyperemia from local vascular dilation
Contact dermatitis
Delayed type (type IV) hypersensitivity
Type I diabetes mellitus
Delayed type (type IV) hypersensitivity against pancreatic B cells
Multiple sclerosis
Delayed type (type IV) hypersensitivity against myelin basic protein
IPEX
immune dysregulation, polyendocrinopathy, enteropathy, X linked
caused by mutation in Foxp3, the transcription factor needed for production of regulatory T cells
Autoimmune Lymphoproliferative Syndrome
Lupus-like disorder caused by Fas mutation; failure of clonal deletion by activation induced cell death via death receptor pathway so autoreactive cells escape deleation
Autoimmune polyendocrinopathy
AIRE mutation
Rheumatic heart disease
Autoimmune disease brought on by molecular mimicry following Group A Streptococcal infection: Streptococcal antigens mimick self antigens in the heart, ntibodies “cross-react” and damage heart
Systemic Lupus Erythematosis
- multi-system autoimmune disease
- antibodies are anti-nuclear: anti-DNA, anti-histone, anti-non-histone protein bound to RNA, anti-nucleolar
- affects joints, kidneys, serosa
- must have 4 of 11 major criteria present to dx (malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal disorder, neurologic disorder, hematologic disorder, immunologic disorder, antinuclear antibodies)
- clinical manifestations: common= hematologic disorder, arthritis, malar rash, fever, fatigue, weight loss, renal disease, CNS issues, pleuritis, pericarditis, myalgia, Raynaud
- pathophysiology: deposition of immune complexes in kidneys, joints, blood vessels- Type III hypersensitivity and hematologic manifestations due to opsonization of blood elements- type II hypersensitivity
- granular pattern of antibodies in glomeruli
Antiphospholipid antibody syndrome
diagnosis in individuals with significant antiphospholipid antibodies, thrombotic complications, but without SLE
Goodpasture Syndrome
- The anti-glomerular basement membrane (GBM) antibodies attack the kidneys and lungs, although, generalized symptoms like malaise, weight loss, fatigue, fever and chills are also common, as are joint aches and pains
- Linear pattern of immunoflourescence in glomeruli- “wire loop lesions”
- Type II (antibody mediated) hypersensitivity
Drug induced lupus erythematosus
Lupus like syndrome induced by drugs (hydralazine, procainamide)
Anti-histone antibodies (no dsDNA Ab)
May have no symptoms, just positive Ab titer
Arthralgia, fever, serositis (no renal or CNS involvement)
Sjogren Syndrome
- autoimmune destruction of lacrimal and salivary glands leading to keratoconjunctivitis sicca and xerostomia
- histology: T cell infiltrate, fibrosis and destruction of glandular parenchyma, loss of secretions; biopsy of lip is required to confirm diagnosis
- pathology: antibodies to RNP antigens SS-A Ro and SS-B La
- more common in older women, increased risk of lymphoma
Mukulicz syndrome
Refers to lacrimal and salivary gland enlargement due to any cause (with or wihtout Sjogren syndrome)
Systemic slcoerosis
- can be diffuse with widespread skin involvement and early visceral involvement, or limited with limited skin involvement/ late visceral involvement/ possible CREST
- associated with antibody anti Scl-70 against DNA topoisomerase I
- fibrosis due to increased secretion of TGF-B with chronic inflammation
GI- dysphagia, GERD, MAS
Kidney- HTN, possibly malignant
Lungs- pulmonary HTN, interstitial fibrosis
CREST
- form of limited scleroderma
- calcinosis, Raynaud, esophageal dysmotility, sclerodactyly, telangectasias
- associated with anticentromere antibodies
Churg- Strauss syndrome
Autoimmune condition that causes inflammation of small and medium-sized blood vessels (vasculitis) in persons with a history of airway allergic hypersensitivity (atopy)
Associated with pANCA
Microscopic polyangiitis
Autoimmune disease characterized by a systemic, pauci-immune, necrotizing, small-vessel vasculitis without clinical or pathological evidence of necrotizing granulomatous inflammation
Associated with pANCA
