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70 Cards in this Set

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what are two types of OA?
Primary- unknown cause
secondary: traumatic causes, inflammatory conditions, metabolic disorders, chronic non-inflammatory joint disease.
55 year old female presents to you with pain in her hips and knees. On examination they are not tender and she explains that it only hurts when she tries to walk or in the middle of the night. She has no fever, no chills and no sputum. Dx and what would you expect upon further examination.
Osteoarthritis.

on further evaluation- at 90 degrees of flexion of the affected joint, the pt starts to feel pain or you will feel a locking.
what are the 3 pathological changes found in OA? at what stage does the pt start to suffer from pain?
1) compression of catilage and detachment of cartilage fragments floating in the synovial fluid (joint mice)
2) increased subchondral bone density and eburnation (ivory-like appearance of bone)- sclerotic
3) cystic change in the subchondral bone and stimulation of new bone (osteophytes)

stage 2 is when the pt will start to suffer from pain.
osteophytes around the small joints of the fingers include two nodes, what are their names and where are they located?
-Bouchard's nodes- around PIP
-Heberden's nodes- around DIP
Pts with what genetic variation are usually RF positive and has an association w/ RA?
HLA-DR4
40 year old woman comes to your office complaining of morning stiffness in both of her hands for the past 2 months. She said that it feels better when she take a really hot shower. She doesn't have a fever, suffer from night sweat, or have productive sputum. Dx
RA- a chronic inflammatory disorder of synovial membranes

20-50 yo women, may have HLA-DR4, RF+.
what is the pathogenesis of RA?
1) acute inflammatory reaction of synovial membrane (synovitis) with acute inflammatory infiltrate (neutrophils) and edema (hyperplasia and hypertrophy of synovial cells).
2) chronic inflammation: Granulation tissue (pannus) w/ cellular infiltrates (lymphs and plasma cells) causes erosion of bone and cartilage--> ostechondral deformity and subcutaneous rheumatoid nodules.
end result: scarring, contracture and deformity.
what is the early sign of RA? what can you expect 10-15 years down the line without tx?
early: wrist and hand swelling, fatigue, weight loss.
late: ulnar deviation, swan neck or boutonier defornity
what are the 5 systemic manifestations of RA?
1) pleural and pericardial effusions
2) anemia of chronic dz
3) vasculitis (endothelium of BV)
4) neurologic abnormalitis (meninges)
5) secondary reactive amyloidosis (B2 amyloid deposition)
how do you differentiate still's disease from RA?
Still's is in children and it involves the large joints like elbow and shoulder. You will see generalized lymphadenitis, hepatosplenomegaly.

in RA- they are adults and it involves the small joints like in the hand.
a 14 year old girl presents with pain in her elbow for 2 months. On examination you notice that she has lymphadenopathy and hepatosplenomegaly. Her temperature is 101 degrees fahrenheit. Dx
STill's dz

Female predominance, pathologic changes similar to RA + lymphadenopathy and hepatosplenomegaly + acute onset marked by fever.
what is the genetic haplotype associated with JRA?
HLA-DRB1
50 year old woman presents with RA, dry eye w/ conjunctivitis and dry mouth w/ problems swallowing. She also has painful intercourse. dx
Sicca Syndrome

RA + xerophthalmia + xerostoma + painful coitus = sicca because it involves dryness of other parts of the body besides the eye and mouth.
45 year old female presents with RAm xerophthalmia, xerostomia and parotid enlargement. what are you most worried about?
she has an increased risk of B-cell lymphoma.

she has Sjogren syndrome.
what 2 ribonucleoproteins are characteristic of Sjogren syndrome?
anti-Ro (SS-A) and anti-LA (SS-B)
45 year old female present with RA and fatigue. You do a cbc and find that she has low neutrophils. Upon physical exam you notice a palpable spleen. dx
Felty syndrome.

High RF titers, positive ANA, positive P-ANCA. increased incidence of infection and malignant lymphoma.
what gene is most commonly associated w/pts who are positive for seronegative arthritis?
HLA-B27
what are the four types of seronegative arthritis?
Ankylosing spondyloarthritis
reiter's syndrome
Psoriatic arthritis
arthritis associated w/ inflammatory bowl dz
33 year old male presents to your office complaining of low back pain that is stiffest in the morning, but gets better as the day progresses. He also complaings of frequent red eyes and blurry vision. You do a RF Test, which comes up negative. dx, what would you expect to see on histopathology?
Ankylosing spondyloarthritis (Rheuamtoid spondylitis or marie-strumpell's dz)- strong assoc w/ HLA-B27

histopathology: chronic synovitis + destruction of articular cartilage and bony ankylosis esp SI and apophyseal joints.
what is reiter syndrome?
an asymmetric polyarthritis that affects mainly the LE assoc w/ conjunctivitis or iritis and nongonococcal urethritis w/ or w/o mucocutaneous lesions
19 year old male pt presents to your office complaining of low back pain along with ulcers in his mouth and on his penis. He says he had the stomach flu a couple weeks ago and since then he started having all of these sxs. dx and what else would you expect to find on examination?
Reiter syndrome

post-infection w/ chlamydia or shigella, salmonella, yersinia and campylobacter in GI infections

expect: arthritis, low back pain, enthesopathy (achilles tendinitis, plantar fasciitis), sausage digits and ulcers in all mucus areas.
what is psoriatic arthritis?
a chronic inflammatory disease involving axial and peripheral joints, usually asymmetric, seronegative for RF
what type of lesions are strongly associated with the development of psoriatic arthritis in the DIP joints?
Psoriatic nail lesions
what are the four inflammatory cytokines?
IL-1, 6, 8 and TNF alpha
45 year old asian man presents to your office complaining of a painful mass on his big toe. He says that the pain comes and goes and usually follows a drinking binge. He tells you that everyone in his family has the same problem, but they never do anything about it. DX and explain other techniques to confirm your dx
dx: primary gout

dx: blood analysis for hyperurecemia + urate crystals and neurtophils in synovial fluid analysis OR
finding tophaceous deposits (biopsy) and the presence of urate crystals which have negative birefringence and needle shape under polarized light.
On a photomicrograph you see an aggregate of dissolved urate crystal is surrounded by reactive fibroblasts, mononuclear inflammatory cells and giant cells. DX
primary gout. This is pathognomonic for primary gout.
what is the cause of secondary gout? what are two independent risk factors for secondary gout?
Hematopoietic malignancies--> leukemia, multiple myeloma, and myeloproliferative syndromes.

independent RF: HTN and obesity
what is lesch-nyhan syndrome?
x-linked deficiency of hypoxanthine guanine phosphorylbosyl transverase (HGPRT) enzyme leading to hyperurecemia w/ severe neurologic manifestations including self-mutilation.
65 year old female presents to your office complaining of knee pain. SHe says that the pain only comes after big family occasions like Christmas or Thanksgiving. You aspirate the synovial fluid of the knee and look at it under microscope and find slightly positive crystals that are basophilic and rhomboid in shape. DX
Pseudogout

why? 1) pseudogout affects large joints (knee is involved in 50% of cases) and 2) pseudogout crystals are weakly positive and basophilic and rhomboid in shape.
22 year old female comes in complaining of fever and severe knee pain. Upon examination you notice that her knee is red, hot and very tender. You do a blood culture and it comes back positive for an organism. You aspirate the knee and it produces purulent synovial fluid. dx and name the pathogen.
Gonococcal arthritis- septic joint stage.

Caused by Neisseria Gonorrheae.
pts may also present w/ papular or petechial rash with tenosynovitis. age group 16-40
In nongonococcal arthritis
____ is the main causative agent in older children and adults
_____ arthritis predominates in children under 2 yoa
_____ may infect sickle cell dz pts at any age
1) S. aureus
2) H. influ
3) Salmonella
a 1 year old suddenly develops acute pain, hot and swollen knee joint w/ restricted range of motion along w/ fever, leukocytosis and elevated ESR. Dx, name the pathogen and tx. what else should you be considering?
dx: nongonococcal arthritis
pathogen: H influenza
Tx: urgent w/ parenteral antibiotics and aspiration or surgical drainage.

since its monoarticular arthritis, immediately consider septic arthritis until proven otherwise.
what joint is most affected in nongonococcal arthritis of IVDA?
sternoclavicular.
what is a consequence of spreading of TB to weight bearing joints?
Tuberculous arthritis which can result in severe destruction w/ fibrous ankylosis and obliteration of the joint space. The affected synovium may erode articular and bone along the joint margins.
how do you diagnose lyme dz?
IgM serum antibodies (anti-IgM) to B. Burgdorferi
what is hypertrophic osteoarthropathy?
it's a reaction d/t a systemic disorder (chronic lung dz, congenital cyanotic heart dz, liver cirrhosis and IBD)
what is frequently associated with hypertrophic osteoarthropathy?
periostitis at the distal end of radius and ulnar as well as clubbing of fingers.
where are ganglion cysts located?
arising in the tendon sheath or joint capsule of the wrist joint.
what is synovial sarcoma? what is the cause? where does it originate in?
a highly malignant soft tissue tumor that occurs d/t chromosomal translocation (T(x;18). mostly originates in the tissue adjacent to a joint rather than in a joint cavity.
24 year old male presents complaining of a painless, deep-seated mass that has been around for several years, but continues to get larger. Upon palpation, you try and move the nodule and you cannot. The pt doesn't suffer from a fever or have any sputum production. dx
Synovial sarcoma
on histology, you notice a biphasic growth pattern; Both epithelial and spindle cells. dx
synovial sarcoma.
what is villonodular synovitis?
a term for several closely related benign neoplasms (not inflammatory) that develop in the synovial lining of joints, tendon sheaths, and bursae.

pts presents w/ pain, locking and recurrent swelling
On examination of a pts synovium you notice that the synovial membrane is brown w/ fingerlike processes. Dx and tx
pigmented villonodular synovitis: this is d/t increased proliferation resulting in wrinkling and excessive synovial fluid every couple of weeks.

tx: removal of the whole synovial membrane.
what does giant cell tumor of tendon sheath manifests as?
a solitary, slow growing, painless mass that freqently involves the tendon sheaths along the wrists and fingers.
what is the most common mesenchymal neoplasm of the hand?
giant cell tumor of tendon sheath.
on histology, you notice a biphasic growth pattern; Both epithelial and spindle cells. dx
synovial sarcoma.
what is villonodular synovitis?
a term for several closely related benign neoplasms (not inflammatory) that develop in the synovial lining of joints, tendon sheaths, and bursae.

pts presents w/ pain, locking and recurrent swelling
On examination of a pts synovium you notice that the synovial membrane is brown w/ fingerlike processes. Dx and tx
pigmented villonodular synovitis: this is d/t increased proliferation resulting in wrinkling and excessive synovial fluid every couple of weeks.

tx: removal of the whole synovial membrane.
what does giant cell tumor of tendon sheath manifests as?
a solitary, slow growing, painless mass that freqently involves the tendon sheaths along the wrists and fingers.
what is the most common mesenchymal neoplasm of the hand? give an example
giant cell tumor of tendon sheath. EG: de Quervain's dz
histological sample presents cells grown in a solid nodular aggregate that may be atatched to th synovium by a pedicle. dx
Giant cell tumor of tendon sheath
How do you grade a tumor? how does the site of the tumor relate to the prognosis?
grade- based on degree of differentiation
site- tumors arising in superficial locations have a better prognosis than deep-seated lesions.
what is the most common soft tissue tumor of adulthood?
Lipoma
what are the pathologic changes associated with conventional lipoma? where is it usually located? what would see histologically?
well-encapsulated mass of mature adipocytes which varies in size. It arises in the subcutis of proximal extermities and trunk- mid adulthood
histo: mature white fat cells.
where are liposarcomas located?
in proximal extremities and retroperitoneum as deep soft tissues inbetween muscle fibers/ groups.
what are the three types of liposarcoma? what is the prognosis for each of them?
1) well differentiated variant- only lipocytes, no lipoblast--> good prognosis
2) Myxoid variant- intermediate malignant behavior- lipocytes> lipoblast
3) round cell and pleomorphic variants: aggressive and frequently metastasize: lipoblast> lipocytes
what are the pathological changes of liposarcoma? what chromosomal abnormality occurs in most cases of myxoid and round cell variant?
Path: Lipoblasts are common in the fetus, but abnormal in adults are present and indicative of fatty differentiation mimicking fetal fat cells and contain round clear cytoplasmic vacuoles of lipid scalloping the nucleus.

chromosomal abnormality: t(12;16)
35 year old male presents with a solitary, rapidly growing painful mass on the volar aspect of his forearm. He said he was in a car accident a month or so ago and that same surface slammed up against the dashboard. Upon palpation you notice that the mass doesn't move very much and seems to be deeply planted. You do a biopsy and find plump immature appearing fibroblasts arranged randomly w/ variable size and shape and abundant mitotic figures. Dx and tx
dx: Nodular fasciitis
tx: rush to remove it to make sure it is not one of the more malignant tumors. (must exclude fibromatosis and spindle cell sarcoma).
A 16 year old football quarterback comes into your office complaining of a painless, hard, well-demarcated mass on his femur. He says that he got hit in that location at a football game about a month ago and there was a lot of swelling and pain off and on over the following weeks. You do an xray and you see soft tissue fullness, and patchy radiodensities in the periphery. dx and how do you distinguish this from something else?
Myositis ossificans

must be distinguished from extraskeletal osteosarcoma by the presence of metaplastic bone.
A 23 year old white male from Minnesota presents to your office complaining of a stuck ring finger. He said that he finger has been getting closer and closer to his palm over the past couple of weeks, but now he can't move it out of this position. DX
Dupuytren contracture --superficial fibromatoses (palmar variant)

two other kinds: plantar fibromatosis, penile fibromatosis (peyronie dz)
what is the histopathology of desmoid tumors?
aggressive fibroblastic proliferations form plump fibroblasts and arranged in broad sweeping fascicles that infiltrate to adjacent tissue.
Desmoid tumors are borderline between _______ ___ tumors and ____ _______ fibrosarcomas
nonaggresive fibrous and low-grade
what are the three types of desmoids? which one has is associated w/ pregnancy and which one is associated with Gardner syndrome?
1) extra-abdominal: musculature of shoulder, chest wall, neck and thigh
2) abdominal: women during or after pregnancy as a musculoaponeurotic structure of anterior abdominal wall
3) intra-abdominal desmoids: mesentery or pelvi walls-- Gardener syndrome- also called familial adenomatous polyposis.
what type of mutation is found in desmoids? how do you cure? what population is generally affected?
Mutations in the APC or Beta catenin genes
cruable by adequate excision, chemo, or irradiation, tamoxifen (high recurrent rate after excision)
pop: teens to mid thirties
what is a fibrosarcoma?
unencapsulated, infiltrative, soft, fish-flesh masses w/ areas of hemorrhage and necrosis commonly located retroperitoneum.
ON a histological slide you see spindled cells growing in a herringbone fashion, architectural disarray, pleomorphism, frequent mitoses and areas of necrosis. dx
fibrosarcoma.
35 year old female presents with a painless, slow growing nodule. You notice that the nodule is <1 cm in size and is located somewhere in the dermis or subcutaneous layer of skin. ON histology you notice spindle cells arranged in a storiform pattern w/ foam cells, hemosiderin deposits, multinucleated giant cells and hyperplasia. Dx and tx
Dx: dermatofibroma (benign fibrous histiocytoma)
tx: simple excision
what areas of the body do malignant fibrous histiocytoma affect? what can it resemble? tx?
areas: musculature of proximal extremities and retroperitoneum
resemble: pleomorphic variants of liposarcoma, leiomyosarcoma and rhabdomyosarcoma.

tx: resection otherwise it is aggressive and recurrent. Metastatic in 30-50%
which variant of malignant fibrous histiocytoma is not metastic in 30-50% of its pts?
angiomatoid variant.
on histology you see considerable cytologic pleomorphism, presence of bizarre multinucleate cells and storiform architecture. dx
malignant fibrous histiocytomas.

--> fibroblastic NOT histiocytic.