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102 Cards in this Set
- Front
- Back
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rheumatoid factor
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autoantibody against Fc portion of the autologous IgG
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most RF is what
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IgM
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anti nuclear antibodies
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antibodies directed against non-organ specific nuclear antigens
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ANA are present where
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in 5-15% of normal individuals in low titer
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ANAs are in high titer when
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connective tissue disease
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chronis SLE is characterized by what
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relapses and remissions affecting skin, joints, kidney, heart and serosal membranes
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SLE is more common and severe in what population
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american black women
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what is pathogenesis of SLE
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failure of regulatory mechanisms to maintain self tolerance
ANAs directed against several nuclear antigens |
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what are the four categories of SLE
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antibodies to DNA, to histones, to non histone proteins bound to RNA, to nuclear antigens
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what things are virtually diagnostic of SLE
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antibodies to double stranded DNA and the Sm antigen
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high titers of DS DNA ANA is associated with what
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active renal disease
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low risk for what is anti-SS-B is present
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nephritis
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what are the non-genetic factors affecting SLE
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drugs, UV light, sex hormones
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most visceral lesions of SLE are due to what
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deposition of immune complexes type 3 hypersensitivity
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autoantibodies to platelets, red cells, and white cells in SLE are mediated by what
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type 2 hypersensitivity
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what is morphology of skin in SLE
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liquefactive degeneration of dermal/epidermal jnct
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immunoflourescence of skin in SLE shows what
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deposition of immunoglobulin and complement at the dermal/epidermal jnct
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what percent of case by light microscopy show renal involvement in SLE
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60-70%
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with EM and immunoflourescence is used what percent of SLE have renal involvement
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almost all cases
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WHO class 1 of lupus nephritis
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no change rare
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WHO class 2 of lupus nephritis
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mesangial GN 20%
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WHO class 3 of lupus nephritis
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focal proliferative GN 20%
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WHO class 4 of lupus nephritis
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diffuse proliferative GN 40-50%
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WHO class 5 of lupus nephritis
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membranous GN 15%
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most common class of lupus nephritis
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class 4 diffuse proliferative GN
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what is mildest of lupus nephritis
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mesangial glomerulonephritis
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mesangial glomerulonephritis
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granular mesangial deposits of immunoglobulin and complement
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focal proliferative glomerulonephritis affects what
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50% of glomeruli and only portions of each glomerulus
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what infiltration with focal proliferative glomerulonephritis
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infiltration with neutrophils and occasionally fibrinoid deposits and thrombi
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what is clinical presentation of focal proliferative glomerulonephritis
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hematuria and proteinuria
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diffuse proliferative glomerulonephritis
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proliferation of endothelial mesangial and sometimes epithelial cells, some crescents and subendothelial deposits on EM
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what part of glomeruli is involved in diffuse proliferative glomerulonephritis
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most of it
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what is clinical presentation of diffuse proliferative glomerulonephritis
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micro or gross hematuria, proteinuria, HTN
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membranous glomerulonephritis
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widespread thickening of capillary walls
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what is clinical presentation of membranous glomerulonephritis
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severe proteinuria and nephrotic syndrome
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wire loop lesion
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extensive subendothelial deposits create a peculiar thickening of the capillary wall
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what microscope are wire loop lesions seen with
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light microscope
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what WHO classes can wire loop lesion be seen in
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Class 4
and also Class 3 and 5 |
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what do wire loop lesions indicate
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active disease and poor prognosis
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what is mechanism for all types of lupus nephritis
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immune complex deposition
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all types of lupus nephritis show what
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granular immunoglobulin in mesangium alone or along basement membrane
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what is the pathology of joints in SLE
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nonerosive synovitis with little deformity
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what are the CNS symptoms of SLE ascribed to
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acute vasculitis
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what is the cardiovascular pathology assoc with SLE
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pericarditis
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what valves are involved in SLE
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mitral and aortic valves
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libman-sacks endocarditis
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nonbacterial verrucous endocarditis
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what is morphology of spleen with SLE
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enlarged with capsular thickening, plasma cells in the pulp
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what is morphology of lungs with SLE
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pleuritis and pleural effusions
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what are the criteria for diagnosis of SLE
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malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal disorder, neurologic disorder, hematologic disorder, immunologic disorder, antinuclear antibody
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what is necessary for diagnosis of SLE
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must have 4 or more of criteria
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what is typical presentation of SLE
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young women with butterfly rash, fever, pain but no deformity in one or more joints, pleuritic chest pain, and photosensitivity
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what is 5 yr survival with SLE
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90%
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what is 10yr survival with SLE
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80%
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what are CODs with SLE
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renal failure, infections, diffuse CNS disease
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what are the lupus variants
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chronic discoid lupus erythematous, subacute cutaneous lupus erythematosus, drug-induced lupus erythematosus
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what is predominantly invovled in lupus variants
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skin mild if any systemic involvement
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lupus variants are less frequently positive for what
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ANA or LE cell positive
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what are skin lesions with chronic discoid lupus
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scaly erythematous plaques and atrophic scars
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in chronic discoid lupus you rarely have antibodies to what
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double stranded DNA
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what are the type of skin lesions with subacute cutaneous lupus erythematosus
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widespread but superficial and nonscarring lesions
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subacute cutaneous lupus erythematosus has a strong assoc with what
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antibodies to SS-A and HLA-DR3 genotype
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what is drug induced lupus
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lupus-like symptoms develop in patients recieving certain drugs
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drug induced lupus is usually associated with what
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development of ANAs
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drug induced lupus has a high frequency of what
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antihistone antibodies
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drug induced lupus is associated with what
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HLA-DR4
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most common drugs involved in drug induced lupus
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hydralazine, procainamide, isoniazide, D-penicillamine
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sjogren syndrome
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a clinicopathologic entity characterized by dry eyes and dry mouth resulting from immunologically mediated destruction of the lacrimal and salivary glands
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what is predominate in the infiltrate in lacrimal and salivary glands
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activated CD4 helper cells
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ANA and RF are present in what percent in sjogren
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ANA 50-80%
RF 75% |
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what are the antibodies directed against in sjogrens
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ribonucleoprotein antigens
SS-A and SS-B |
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which patients with sjogrens are more likely to have extraglandular manifestations
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those with high titers of antibody to SS-A
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what is the morphology of sjogrens
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periductular lymphocytic infiltrate, acinar atrophy, fibrosis, hyalinization, and fatty replacement of gland
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what are clinical features of sjogrens
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dry, burning eyes and dry mouth, trouble swallowing, problems with oral hygiene, enlargement of salivary glands
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what are people at increased risk for
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40 fold higher risk of developing a non-hodgkins lymphoma
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what are the two categories of scleroderma
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diffuse scleroderma
localized scleroderma: CREST |
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what is pathogenesis of scleroderma
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fibrosis is felt to be secondary to abnormal activation of the immune system
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what is morphology of scleroderma
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diffuse sclerotic atrophy of the skin beginning in the fingers
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what is the morphology of scleroderma in the GI tract
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progressive atrophy and collagenous fibrous replacement of the muscularis
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what is the morphology of scleroderma in the musculoskeletal system
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inflammation of synovium early fibrosis later
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renal abnormalities occur in how may patients with scleroderma
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2/3
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scleroderma in the kidneys is restricted to what vessels
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vessels 150-500mm in diameter
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what percent of patients with scleroderma and kidney involvement have HTN and malignant HTN
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HTN=30%
malignant HTN=20% |
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how often are lungs involved in scleroderma
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>50%
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scleroderma may manifest as what in the lungs
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pulmonary HTN and interstitial fibrosis
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what is the morphology of scleroderma in the heart
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pericarditis with effusion and myocardial fibrosis
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what percent of scleroderma has ANA
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50-80%
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what two ANAs are unique to systemic scleroderma
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antibodies against DNA topoisomerase 1
anticentromere antibody |
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what percent of patients with CREST havd anticentromere antibody
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96%
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what is clinical course of systemic sclerosis
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raynaud phenomenon, dysphagia, abdominal pain, intestinal obstruction, respiratory difficulty
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what are CODs with systemic sclerosis
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heart failure, pulmonary insufficiency, renal failure
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CREST
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C-Calcinosis
R-Raynauds Phenomenon E-Esophageal dysphagia S-Sclerodactyly T-Telangectasia |
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mixed connective tissue disease
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describes a disease with clinical features suggestive of SLE, polymyositis, and scleroderma
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mixed connective tissue disease serologically
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high titers of antibodies to RNP particle containing U1 RNP
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what is the classic rash of dermatomyositis
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lilac or heliotrope discoloration of the upper eyelids with periorbital edema
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rash of dermatomyositis may be accompanied by what
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scaly erythematous lesions or dusky red patches over the knuckles, elbows, or knees
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what is characteristics of muscle weakness of dermatomyositis
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slow onset, bilaterally symmetric, proximal muscles affected first fine movement affected late
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1/3 of patients with dermatomyositis have this
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dysphagia from oropharyngeal and esophageal involvement
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adults with dermatomyositis have an increased risk of what
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developing visceral cancers 6-45%
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what is pathogenesis of dermatomyositis
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capillaries principle target, microvasculature is attacked by immunoglobulin and complement
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polymyositis
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symmetric proximal muscle weakness, direct injury to myofibers by CD8 T cells
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sarcolemma in polymyositis has increased expression of what
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HLA class 1 molecules
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there is a slight increased risk of what with polymyositis
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developing visceral cancers
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