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Flashcards in MSI Deck (85):
1

Macula Adherens

Desmosomes attached to keratin

2

Zonula Dherens

Cadherins homotypic

3

ACL/PCL

Named for their location on the tibia
-ACL is anterior drawer test. Normally prevents anteior displacement of the tibia on the femur
-PCL is posterior drawer prevents posterior movement of the tibia on the femur

4

Achondroplasia

-AD FGFR3 activating mutation that inhibits chondrocyte proliferation in the epiphysis leading to disorganized epiphysis
-Impaired endochondral ossification and shortening of long bones. Other bones are fine
-Usually associated with advanced paternal age

5

Osteoporosis

Normal labs and caused by an age related increase in clastic activity and a decreae in blastic activity
-IL-1 and 6 cause decrease clasts and estrogen increases protegrin
-One type is in post menopausla women and can respond to SERMs like raloxifen
-Type 2 is in everybody
-Treat with vitamin D, Ca, excercise, bisphosphonates, SERM
-Avoid corticosteroids
-Thinned trabechulae is histologic halmark
-Can also give pulsatile PTH that will increase the activity of blasts (RANK-L is expressed by blasts in response to PTH adn RANK is on clasts)

6

Osteopetrosis

-AR dysfucntion in Carbonic Anhydrase 2 which leads to an inability to generate acidic environment for clasts to degrade bone
-Results in an increase in ALP activity and a decrease in Ca
-Sclerotic thickened bone that is easy to break
-Can present as hearing and neural impingment
-Can be cured with a BM transplant that regenerates calsts
-Can also get a myelophtisic process that causes pancytopmenia and EMH

7

Osteomalacia and Rickets

Caused by a decrease in vitamin D that impairs Ca and P metabolism
-Low Ca and P with icnreased ALP and PTH
-There will be unmineralized osteoid (collagen)
-Rickets is short stature, bowing of legs and head, ribs that have beads
-Malacia is weak bendy bones that are prone to fracutre
-Deficent dietary or malabsorption

8

Pagets

-Cause is unknown, but there is an initial increae in blastic activity that degrades bone that is then followed by an increae in clastic activity that increases bone lay down
-Leads to a disorganized lamellar structure histologically and enlarging or changing bones grossly
-Increase in ALP with all other lab values normal. The priamry defect is with the clasts and does not involve Ca metabolism
-Increaed risk of osteosarcoma, especially in the older population
-Can also have high output heart failure because of AV malformations.

9

Osteotits Fibrosis Cystica

-Increase in PTH leads to subperiosteal resporption of bone that is then replaced by fibroblasts and collagen
-Can be seen secondary to primary PTH or renal disease
-Labs are variable, but 1 is increase in PTH and ALP and Ca with a decrease in phosphate

10

McCune Albright

-Hereditery increase in GPCR signalling that elads to overactivation of a numbr of hormone pathways, most often PTH
-Fibrosa cystica, unilateral cafe au lait spot and possible precocious puberty
-Shortened 4th and 5th digits is classic

11

Labs

-Osteoporosis all normal
-Osteopetrosis: Increase in ALP and decrease in Ca
-Pagets: Increase in ALP isolated
-Osteomalacia: increase in PTH and ALP decrease in Ca and P
-Osteotitis: Decrease in P increase in PTH, ALP and Ca

12

Giant Cell Tumor

Osteoclastic benign tumor most commonly located in the epiphysis
-Will have osteoclastic markers, CD68, will be of monocyte lineage
-Will have a soap bubble appearance on X Ray
-Multinucleated giant cells and spindle cells

13

Osteoma

-Will improve with NSAIDs

14

Osteochondroma

-Exophytic process from epiphyseal plate leads to chondrocyte cap
-Generally occurs in young men
-Pain is relieved by NSAIDs

15

Osteosarcoma

-Malignant tumor of the metaphysis
-Classically appears as Codmans triangle with a sunburst pattern
-Is osteoblastic in origin
-Asslocated with Rb, Pagets, Radiation

16

Ewings Sarcoma

-Small blue cell neuroendocrine tumor in the diaphysis of long bones, small blue cells
-translocation of 11;22
-Onion skin appearance that can take up the entire bone laterally
-Highly malignant, but generally responsive to radiation

17

Chondrosarcoma

-Tumor of cartilage cells that leads to a radiolucent image in the diaphysis
-Generally occurs in older males
-Meddullary cavity

18

Avascular Necrosis

-Necrosis of scaphoid following fracture
-Pathologic fracture of femur head without osteoporosis
Associated with alcohol and steroids
-Sickle cell is aslo a common cause (dactylitis)

19

OA

Chronic wear and tear inflammation that leads to breakdown of collagen covering of bone, generally in large weight bearing jounts. Cartillage is normally type 2 collagen and PG
-DIP is often involved (not in RA, but MCP is spared)
-Osteophytes
-Treat with NSAIDS adn intrajoint steroids

20

RA

-Systemic type 3 hypersensitivity immune response
-CCP is more specific, but RA is also likely
-IgM to Fc IgG is RA
-Deposition in joints and inflammation elads to pannus formation and deviation
-Fibrnioid necrosis below teh skin is a common complaint (Rheumatoid nodules)
-Systemic illness that can cause pleuritis, pericarditis, amyloidosis (AA)
-Treat with DMARDs (MTX, Steroids, Sulfasalazine)
-HLADR4
-Invovment of PIP and MCP with sparing of DIP

21

Juvenile RA

-Generally presents as a mores systemic illness and can present with fevers
-OFten get associated uveitis
-Systemic is predominant feature

22

Sjogrenna

-Lymphocytic infiltrate into exocrine glands
-Salivary leads to xerostomia and lacrimal to xeroopthalmia
-Leads to infections and cavities
-Also causes arthritis
-RA is commonly positive
-Ribonucleptide ssRA and ssLA are more specific. Can cross placenta and cause neonatal heart block
-Tx: steroids and symptoms
-Risk of marginal zone lymphoma from lyphoid aggregates
-Can also treat with muscarinic agonist to increase eyeflow
-Can also see cryoglobulins as you can in RA
-Associated with HLADR/Q

23

Alkaptonuria

-Defect in homogentisic oxidase that metabolizes tyrosine
-Leads to arthritis, black urine, and heart disease

24

Gout

-MSU preco[itates in joints that are then phagocytosed by nneutrohils leading to inflammatin
-Most commonly big toe
-Due to increase in purine breakdown
-HGPRT in lesch nyhan
-Inhibitors of xanthine oxidase are treatment (fuboxistat and allopurinoll
-Causes can be destruction of leukemia cells and decreased excretion
-Decreased excretion from competition for organic anion transporter (lactate and alcohol)
-Thiazides also cause gout
-Tophus formation possible

25

Pseudogout

Larger joints with calcium pyrophosphate crystals
-Basophilic on stain (like psammoma bodies)
-Usually knees
-Hypercalcemia is a risk factor

26

Osteonecrosis

Infarction of marrow space due to alcoholism, sickle cell, and high dose steroids

27

Seronegative Spondyloarthropathies

-are all RF negative
-Associated with HLA B27 (MHC1)
-IBD

28

Psoriatic Arthritis

-Occurs in fewer than 1/3 of patients
-Pencil in cup deformity always involving DIP and has pencil in cup appearance

29

Ankylosing spondylitis

Pannus and fusion of the sacro illiac joints
-Leads to restrictive lung diseas due to kyphosis
-Uveitis can cause blindness
-Aoritis can cause regurg

30

Reactive arthritis

Urethritis, Conjunctivitis, and arhtritis that is sometimes accompanied by a rash

31

Osteomyleitis

-Hematogenous spread
-A Aureus
-Salmonella
-Psuedomonas
TB

32

Ankylosis Spndylitis

-Fusion of sacroiliac joints
-Aortitis can cause death
-Uveitis can cause blindness

33

Infectous Arthritis

Gonorrhea
-S Aureus, Lyme, pastuerulla

34

SLE

-Type 3 hypersensitvity to post apoptotic nuclear fragments
-Defect in early compliment leads to inabilty to clear via C3b
-Deposition in tissue throughout the body
-Most common presenting symtoms are arthritis and macular facial rash
-Basal cell degeneration and dermal atrophy
-Also discid rash
-Pericarditis and pleuritis are possible
-Liebman sacks aeseptic endocarditis
-Antiphospholipids give false positive VDRL and also lead to hypercoagulable state that commonly causes abortions
-Mucocytis
-Neurologic symptoms
-Renal disease is the most common cause of death
Membranous leads to nephrotic syndrome early in disease course
-Diffuse proliferative gives rise to nephritis syndrome late in course and is common cause of death
-ANA and Anti-smith/anti DSDNA
-Hilar adenopathy and raynauds are common
-Hemolytic anemia

35

Drug induced lupus

-Antihistone antibodies
-Commonly seen with hydralazline and procaimamide

36

Sarcoidosis

-Noncasseating granulomas
-Most common is restrictive lunf disease with bilateral hilar adenopathy
-Can effect any organ
-Commonly effects GI and Eyes, may lead to blindness
-Eryhthema nodosum is common skin complaint (subq fat, commonly on shins)
-Increased ACE expression in lung tissue
-Also elevated vitamin D levels leads to incrase in Ca levels
-Bells Palsy
-Ca depsoits are shumman bodies and asteroid bodies are classic

37

Polymyalgia Rheumatica

-Inflammation in joints and bones that involves multiple
-Older patients with massively elevated ESR, commonly seen with temporal arteritis
-Normal CK
-Treat with steroids

38

Fibromyalgia

female in middle age that has musculoskeletal compaints
-Treat with SSRI

39

Polymyositis

-CD8 driven endomysial inflammation
-A component of mixed connective tissue disease
-Elevated CK and Anti-Jo-1 antibody
-Treat with steroids
-Morning stiffness that most commonly effects the shoulders
-On differential for RA

40

Dermatomyositis

-CD4 driven TH2 inflammation of epimysial muscle and face/hands
-Heliotrope rash that involves area around eyes and sometimes looks like lupus rash
-Mechanics hands
-Commonly associated with a visceral malignancy and may be a paraneoplastic syndome
-Anti-Jo-1 antibodies to tRNA synthase
-Treat with steroids

41

Inclusion body byositis

Similiar presentation with muscle pain and an elvetated CK
-Incluision bodies will be seen and does not respond to steroids
-Commonly seen in elderly

42

MG

Anitbody to AchR leads to muscle weakness
-Most commonly in small muscles and occurs at the end of the day and is worse with movement
-Thymoma is almost always found
-Treat with neostygmine and pyridostigmine

43

Lambert Eaton

-Ab to Ca Channels that are V gated
-Leads to muscle weakness in proximal msucles commonly that gets better with exercise
-Small Cell lung paraneoplastic

44

Myositis Ossificans

-Fibroblasts turn into osteoblasts
-Most commonly seen around sites of trauma, but can occur genetically

45

Scleroderma

-Vascular injury that leads to TH2 mediated activation of fibroblasts that leads to collagen and PG depostion in tissues
-Most noticible in the skin that leads to thickened skin that is not wrinkled
-Can occur in any organ
-Lungs is the most common cause of death leading to a restrictive disease picture
-Heart can also be invlolved
-Kidney can also be involved, interlobular artery, change is myxoid
-Can be diffuse that commonly has visceral involvment, characterized by anti scl-70 antibody to topoisomerase
-CREST is characterized by anti centromere antibody
-Calcinosis, Esophageal dysmotility, Raynauds, sclerodactyly, tel;angectasia. Minimal sclerosis of internal organs, therefore more benign presentation

46

Mixed connective tissue

-Has facets of SLE, Scelroderma, and Polymyositis
-U1 riboneucleotide positive

47

Parakaratosis

Epidermal cells retain nuclei into stratum granulosum layer
-Seen along with acanthosis in psoriasis

48

Albinism

-Can be due to decrease in melanocyte number (s-100)
-Or decrease melanin prouction from tyrosine

49

Vitiligo

Autoimmune destruciton of melanocytes in a particular pattern, seen in

50

Melasma

-Change in color due to elevated estrogens (OCP) or pregnancy

51

Psoriasis

Increase gworht of epidermis and cells retain theri nuclei
-Acanthosis and parakeratosis
-If scraped, ther ewill be pinpoint hemorrhages at the point of dermal papilae entry
-Salmon coloerd plaques
-Can treat with UVA sunlight and soralen to destory cells
-Nuclei still present in stratum corenum
-Increase in spinosum and decrease in granularum, more immature cells in spinosum

52

Seborheic Keratosis

-Lipid filled, stuck on lesions that are benign
-Can occur all at once in leser trelaut sign which signals visceral malignancy like pancreatic cancer
-Keratin cyst with keratin horns

53

Pemphigus Vulgaris

-IgG antibodies to desmoglein 3 which leads to acanthlolysis and classic reticular pattern
-Will break easily and can be deadly, treat like burns
-Will involve mucous membranes
-Macula Adherens (Desmosomes) intercellular attachments to keratin

54

Bullous Pemphigoud

-Ab to hemidesmososes at D/E junction leads to blisters that don't rupture easily and not acanthlysis
-Does not involve mucosa and is less severe

55

Dermatitis Herpetiformis

-IgA antibodies to gliadin that lead to accumulation at derrmal papipllae
-Pruritic vesicualr lesions on extensor surfaces
-Celiac is HLA DR2/8

56

Eryhtema Multiforme

-Many causes including indcetion (HSV adn mycoplasma) drugs (beta lactams and sulfa) and cancer
-Leads to a irregular targetoid lesion and epithelial disruption
-There is no consistent morpholgy (Multiforme)
-Does not involve mucous membrans and is a precursosr to stevens johnsons

57

Stevens Johnsons

-Continution of EM, usually due to drugs (Sulfa)
-Widespread CD8 mediated necrosis and destruction at D/E junction leading to massiv sheding of skin
-Involves mucous membranes
-If greater than 30% of body involved then is TEN

58

Acanthosis Nigricans

-IGF mediated increase in epithelial proloferation and also collagen deposition leads to a velvety thick skin
-Usually bilateral and commonly located at armpits
-Sign of inuslin resistance or visceral malignancy

59

Actinic Keratosis

-Crusty scaly plaque that is a precursor to SCC
-Associated with UVB exposure
-UVA is more energy and causes burns
-UVB is lower energy and causes thymidine dimers

60

Eryhtema Nodosum

-Small nodules of subq necrosis of fat, commonly presenting in skin
-Associated with all kinds of granulomatous reactions
-TB, Sarcoid, Fungal

61

Lichen Planus

-Purple Pruritic papules,polygonal commonly presenting onshings, but can present anywehre
-Highly associated with Hep C infection
-Sawtooth at DE junction
-Lymphocytic infiltrate
-Commonly invovles oral mucosa
-Also see dystrophic naiils

62

Pitarysis Rosea

-Herald patch that is often misdiagnosed as RIngworm
-Followed later by christmas tree distribution extensive rash
-Do not treat and goes away on its own
-Commonly occurs following a URI

63

Sunburn

Burns and tanning are commonly associated with UVA light and UVB light is thymidine dimers
-Can lead to infections and impetigo

64

Impetigo

Infection of the epidermis, msot commonly by S Aureus and S Pyogenes
-S Aurues may cause bullae

65

Cellulitis

Infection of the dermis
-Pyogenes and aureus

66

Necrotizing fascitis

-S Pyogenes
-Clostridium perfringens - gas gangrene

67

Scalded skin synddomre

S Aureus exotoxin to desomsomes leads to acanthllyssis and skin shedding
-Different from TEN because doesn't involve the DE junction just the
-Involves the stratum granulosum

68

Hairy Oral Leukoplakia

Associated with EBV occurs on the lateral tongue in immunocompromised individuals

69

Basal Cell Carcinoma

-Upper lip
-Pearly white plaques with ulcerations and rolled edges
-Histologically will show nests of cells with pallisading nuclei
-Locally aggressive but rarely metastasize
-5-FU is a common topical treatment

70

SCC

-Kertin pearls, associated with UVB expsore
-Commonly forms out of actinic keratosis
-Scaly red, gross appearing
-Can be keratoacanthoma that has an ulcerated keratin filled pit
-Local invasion and may get to nodes, but mets are rare
-Can also occur at sites of chronic inflammation such as draining sinuses
-Increased risk with arsenic exposure and immunosupresion

71

Melanoma

-S-100 neural crest cells
-ABCDE
-BRAF kinase activtin mutaiton
-Spread is a function of depth
-Treat with vemerafanib

72

AA pathway

-AA goes to lipoxygenase that leads to leukotrienes
-LTB4 is NO tactic
-LTBC-E are vasodilation, leakage and bronchoconstrictin
-COX pathway
-1: TXA2 and PGE, 2: PGI and Pain
-E2 important for renal flow, gut flow and uterine contraction

73

Glucocoriticouds

Lipocortin inhibits clevage of AA
-Also causes hypocalcemia and death to blasts and increase in clast activity leading to osteoporosis. Some effect is blurred by PTH and hypocalcemia (decrease absorption in gut)

74

Aspirin

-Irreversible acetylation of COX
-Low dose is antiplatelt med is anitpain and fever, and high dose is antiinflammatory
-Reyes

75

NSAIDs

Reversible Cox inhibitors
-Ulcers
-Intersitial Nephritis

76

Celecoxib

Specific for reversible cox 2
-Has minimal GI effect but also dose not decreae platelts

77

Acetomenaphin

Does not effect GI and effects cox in CNS
-Does not cause Reye's syndrome
-Causes toxic oxygen radical mediated necorsis of hepatocytes in response to overdose.
-N acetlycystein will regenerate glutathione to get rid of it
-NAPQI matabolite

78

BisP

-Pyrophosphate analog that binds to hydroxyappetite and decrease ability to resororb
-Pagets, osteoporosis, hyperclacemia
-Can cause corrosive esophagitits and osteonecrosis of the jaw

79

Caliptriol

Vitamin D analog that can be used for psoriasis

80

Allopurinol, Febuxostat

Inhibits Xanthine oxidase to decrease production of uric acid
-Can be used in gout and tumor lysis syndrome
-Will increase toxic concentration of 6MP
-Can also decrease rate of gout excretion
-

81

Probenacid

-Prevents Uric acid reabsoprtion in PCT
-Also prevents peniclin secretinon in PCT

82

Colchicine

-Stabalizes MT and prevents degranulation and chemotaxis of neutrophils
-Causes GI upset

83

Etanercept

-Decoy IgG receptor for TNF alpha

84

Adalimnuab, infliximab

TNF Ab
-RA, ankylosing, psoriasis, crohns

85

Uztezumimab

Ab to IL-12 and 23 that decreases differntiation into TH1 cells
-Used to treat psoriasis