Path Ch 6 Immune Diseases

Front 1

16 yr female with severe pain and swelling of right thigh. Recurrent episodes of pain in knees, ankles, writs, low grade fever, anorexia, rash on face.

Back 1

systemic lupus erythematous, sunburn, rheumatoid arthritis, tumor

Front 2

lupus is typical in which patients

Back 2

female 20-40s

Front 3

clinical presentations of SLE

Back 3

IM DAMN SHARP: butterfly rash, fever, pain, pleuritic chest pain, photosensitivity

Front 4

ex of Type II antibody mediated diseases

Back 4

hemolytic, pernicious anemia, thrombocytopenic purpura, vaculitis, goodpasture, acute rheumatic fever, myasthenia gravis, Graves, diabetes

Front 5

mechanism of disease for autoimmune hemolytic anemia (Type II)

Back 5

opsonization and phagocytosis of red cells

Front 6

mechanism of disease for autoimmune thrombocytopenic purpura

Back 6

opsonization and phagocytosis of platelets

Front 7

mechanism of disease for pemphigus vulgaris

Back 7

antibody mediated activation of proteases, disruption of intercellular adhesions

Front 8

mechanism of disease for vasculitis

Back 8

nutrophil degranulation and inflammation

Front 9

mechanism of disease for goodpastures

Back 9

complement and Fc receptor mediated inflammation

Front 10

distal interphalangeal joint pain and swelling leads to

Back 10

osteoarthritis

Front 11

proximal interphalangeal joint pain and swelling leads to

Back 11

rheumatoid arthritis

Front 12

immune complex deposition within glomerulus can lead to

Back 12

nephrotic (glomerulopathy) and nephritic presentations(mesangial, focal proleferative glomerulonephritis). Vasculitis, pyelonephritis

Front 13

what is the fundamental defect in SLE?

Back 13

self tolerance loss. CD4 T cell dep B cell hypersensitivity. Type II(cell surface) III(multiple organs)

Front 14

What is the significance of antibodies against the phospholipid-b2-glycoprotein complex

Back 14

bind to cardiolipin antigen. Used in syphillis, SLE pts can have false + for syphillis. Antigens associated with hypercoaguable states

Front 15

most common causes of death in SLE?

Back 15

kidney failure, recurrent infections-immunosuppresant drugs. CAD

Front 16

if serum protein electrophoresis shows polyclonal hypergammaglobulinemia, what does the pt have

Back 16

SLE

Front 17

if serum protein electrophoresis shows monoclonal hypergammaglobulinemia, what does the pt have

Back 17

multiple myeloma

Front 18

lymphocytic infiltrate at dermal-epidermal junction and loss of basal cells in skin is characteristic of

Back 18

SLE

Front 19

if renal glomerular immunofluorescence staining for IgG shows linear pattern of staining with anti-glomerular BM antibodies, pt has

Back 19

goodpastures syndrome

Front 20

38 yr old female with dry mouth, mild conjunctivities,sore eyes, mild bilateral pain in hands, wrists, kness.

Back 20

Sjogrens.

Front 21

sjogrens is typical in which pts? What do they present with

Back 21

50-60 yr old women with keratoconjunctivitis(dry eyes) dry mouth, enlarged parotid gland, inflamed/fissured tongue, dental caries, ulcerations of nose, mouth

Front 22

extraglandular disease seen in 1/3 pts with sjogrens include

Back 22

synovitis, diffuse pulmonary fibrosis, peripheral nephropathy, --automminue disease also(RA)

Front 23

what autoantibodies are present in someone with Sjogrens

Back 23

RF, anti-Ro, anti-La(SS-A, SS-B)

Front 24

what is Mikulicz syndrome?

Back 24

destruction of both lacrimal and salivary glands. Nonspecific-present in sarcoidosis

Front 25

mononuclear cell infiltrate and ductal epithelial hyperplasia indicates

Back 25

Sjogrens.

Front 26

sjogrens has a 44x increased risk for

Back 26

B cell lymphoma

Front 27

55 yr female has polyneuropathy, Raynauds phenomenon, swelling to forearms, face, trunk. Ulcer on finger. Hard skin on hand, forearms, face

Back 27

scleroderma

Front 28

what autoantibodies are present for scleroderma

Back 28

ANA, anti-topoisomerase

Front 29

a pt with scleroderma presents with

Back 29

thickening of skin, Raynaud phenomena, esophageal fibrosis, resp insufficiency-pulmonary fibrosis, GI-malabsorption, abdominal pain, obstruction. Hypertension-narrowing of intrarenal arteries

Front 30

what damage to GI tract is present with scleroderma?

Back 30

ischemic damage to neurons with smooth m. atrophy, fibrosis. GE reflux, Barrett esophagus, dysphagia, malaborption

Front 31

difference between diffuse and limited scleroderma?

Back 31

diffuse-widespread skin involvement, early visceral involvement. Anti-topoisomerase. LIMITED-CREST-skin involv to face, fingers, forearms. Better prognosis. Anti-centromere

Front 32

what is CREST syndrome?

Back 32

calcinosis, Raynauds, esophageal dysmotility, sclerodactyly(tapered fingers), telangiectatia(cap dilation-swelling)

Front 33

etiology of Scleroderma?

Back 33

abnormal T cell CD4 activation-release of cytokines. Fibroblasts produce collagen, endothelial cell injury-fibrosis, vascular instability, ischemia to multiple organs

Front 34

leading cause of mortality of ppl with scleroderma? What can it also cause?

Back 34

pulmonary fibrosis. Pulmonary hypertension, cor pulmonale(R heart failure)

Front 35

62 yr man with orthopnea, nocturia, bulky, greasy stools. MI. highBUN, creatinine, protein in urine. Rouleaux formation, lymphocytosis

Back 35

prostate, CHF, atherosclerosis, AMYLOIDOSIS

Front 36

proteinaceous substance deposited extracellular in many organs in a variety of clinical settings.

Back 36

amyloid

Front 37

group of disease with similar protein deposits in tissue. Primary and generalized(multiple myeloma), Alzheimer, chronic inflammation, hereditary

Back 37

amyloidosis

Front 38

serum monoclonal IgM and Bence Jones proteinuria indicates

Back 38

amyloid deposition. (multiple myeloma)

Front 39

what stain is amyloid seen in? what color does it turn with polarized light?

Back 39

Congo red stain. Turns apple green

Front 40

frequently associated with multiple myeloma or other monoclonal B-cell proliferation and often involves the heart, GI tract, peripheral nerves, kidney, and tongue (AL fibril protein).

Back 40

primary amyloidosis

Front 41

follows a chronic inflammatory disorder and the deposits are of the AA fibril protein.

Back 41

secondary amyloidosis

Front 42

What features on the above patient's rectal biopsy were likely diagnostic for amyloidosis?

Back 42

amorphous, extracellular, eosinophilic material deposited in mucosa when stained with Congo red demonstrated apple green birefringence

Front 43

lymphoplasmacytoid cell malignancy of unknown cause that presents with adenopathy, hepatosplenomegaly, neurologic symptoms (peripheral neuropathy) and hyperviscosity.

Back 43

What is Waldenstrom macroglobulinemia?

Front 44

amyloidosis-what does heart look like

Back 44

heart shows glistening tan waxy deposits and myocardium is thickened, firm, and rubbery

Front 45

23 yr homosexual man is weak, cant speak normally. Fever, dry cough, dyspnea. Lost a lot of weight, cutaneous patches, plaques and nodules on back and legs. Disorented to time and place

Back 45

AIDS. Kaposi sarcoma--patches, plaques, nodules

Front 46

primary groups of adults at risk for AIDS

Back 46

homosexual, bisexual men, IV drug abusers, hemophiliacs, recipients of blood

Front 47

2 main targets of HIV include what systems?

Back 47

immune system, CNS

Front 48

major components of the HIV life cycle include

Back 48

infection of cells via CD4 molecule, integration of provirus into host, viral replication, production and release of new virus

Front 49

typical pt with AIDS in the US presents with

Back 49

fever, weight loss, diarrhea, gneralized lymphadenopathy, multiple infections, neurologic SX, secondary neoplasms

Front 50

most common secondary neoplasm in AIDs

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Kaposi sarcoma

Front 51

most common lymphomas in AIDS

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systemic lymphoma

Front 52

most common extranodal site of involvement in AIDS

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CNS

Front 53

common infections in AIDS

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pneumocyctosis, cryptococcosis, mycobacteriosis, CMV, HSV

Front 54

pts with HIV infection with CD4 T lymph <200 or had oral candidiasis should receive

Back 54

chemoprophylaxis against Pneumocystic jiroveci pneumonia (PCP). TMP-SMZ

Front 55

most important causes of morbidity and mortality in AIDS

Back 55

TB, PCP(pneumocystic carini pneumonia)

Front 56

39 yr man progressive muscle weakness, recurrent facial edema esp lips, eyelids. Difficulty swallowing, rash on upper torso and back

Back 56

dermatomyositis

Front 57

how does juvenile dermatomyositis differ from adult onset form?

Back 57

higher incidence of vasculitis within GI tract leading to mucosal ulceration, hemorrhage, perforation and abdominal pain; also calcinosis more common in juvenile form (1/3 of patients). In contrast to adult form, there is no increased risk of malignancy in childhood.

Front 58

Type III Immune complex hypersensitivity diseases

Back 58

SLE, poststreptococcal glomerulonephritis, reactive arthritis, serum sickness, Arthus rxn

Front 59

Type IV cell mediated diseases

Back 59

Type I DM, MS, RA, Crohn, peripheral neuropathy-Guillain Barre, contact dermatitis

Front 60

what autoantibodies indicate SLE

Back 60

anti-dsDNA, ANA

Front 61

what autoantibodies indicate drug induced LE

Back 61

ANA, antihistone

Front 62

what autoantibodies indicate inflammatory myopathies

Back 62

Jo-1

Front 63

primary immunodeficiencies

Back 63

agammaglobulinemia, isolated IgA, Digeorge, SCIDS, wiskott aldridge, complement def

Front 64

secondary immunodeficiencies

Back 64

HIV, cancer