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112 Cards in this Set

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What is Achondroplasia?
**AD trait

=Failure of longitudinal bone growth --> SHORT LIMBS

NOW, membranous ossification is NOT affected = skull, facial bones, and axial skeleton are NORMAL

**Head + trunk are normal in size, BUT limbs are much shorter than normal.
What is Osteoarthritis?
**Mechanical wear-and-tear of joints leads to DESTRUCTION of articular cartilage PLUS:

1) Subchondral bone formation
2) Sclerosis
3) Osteophytes
4) Heberden's nodes (DIP)
5) Bouchard's nodes (PIP)
6) Eburnation
=change in exposed subchondral bone in which it is converted to a dense substance w/ a smooth surface like ivory
Clinical Presentation:
**Common in OLDER patients.

=Pain in weight-bearing joints after USE (i.e. at the end of the day) --> IMPROVING w/ rest

**NO SYSTEMIC SYMPTOMS!
Rheumatoid Arthritis
**AUTOIMMUNE, INFLAMMATORY disorder affecting synovial joints
=PANNUS formation in joints--MCP + PIP

**Females > Males

**80% have POSITIVE RHEUMATOID FACTOR (anti-IgG Ab)
Classic Presentation:
1) Morning Stiffness improving w/ use

2) Symmetric joint involvement

3) Boutonniere Deformity, Swan-Neck Deformity, and Z-thumb Deformity

4) Systemic symptoms
=fever
=fatigue
=pleuritis
=pericarditis
Physical Exam Findings:
1) Subcutaneous rhematoid nodules

2) Ulnar deviation

3) Subluxation
=partial dislocation due to deformation of the joint space

COLOR IMAGE 56
Osteoporosis
=Reduction of BONE MASS in spite of normal bone mineralization

**Affects WHITES > Blacks > Asians
Type I
**Postmenopausal
=Increased bone reabsorption due to DECREASED estrogen levels

**Estrogen replacement is controversial as prophylaxis (=side effects)
Type II
**Senile Osteoporosis
=Affects men AND women > 70 y.o.
What is the major threat of Osteoporosis?
**FRACTURE

1) Vertebral Crush Fractures --> seen mainly in TYPE I
=Acute back pain
=Loss of height
=Kyphosis

2) Distal radius (=Colles') fractures --> Type II

3) Vertebral wedge fractures --> Type II
What can you give patients if they have REALLY severe cases of osteoporosis?
=Bisphosphonates OR pulsatile PTH
What is Osteopetrosis?
="Marble Bone Disease"

**Failure of normal bone resorption --> thickened, dense bones

**Bone defect is due to abnormal function of OSTEOCLASTS
=Serum calcium, phosphate, and alkaline phosphatase are NORMAL
What is Osteomalacia/Rickets?
**Defective mineralization of osteoid --> SOFT BONES

=Vitamin D deficiency in adults --> DECREASED Ca levels --> INCREASED secretion of PTH + Increased serum phosphate

**REVERSIBLE when vitamin D is replaced.

**In childhood--vitamin D deficiency causes rickets.
What is Osteitis Fibrosa Cystica?
**Caused by HYPERPARATHYROIDISM

=characterized by "BROWN TUMORS" --> cystic spaces lined by osteoclasts, filled w/ fibrous stroma + sometimes blood
Lab Results:
=HIGH serum Ca
=LOW serum phosphorus
=HIGH alkaline phosphatase
What is Paget's Disease?
="Osteitis Deformans"

**Abnormal bone architecture caused by an increase in BOTH osteoblastic + osteoclastic activity
Lab Results:
=Serum Ca, phosphorus, and PTH levels are normal

=Serum ALKALINE PHOSPHATASE is ELEVATED
What is Polyostotic Fibrous Dysplasia?
**Bone is replaced by fibroblasts, collagen + irregular bony trabeculae
=affects MANY bones

**A specific form of it is called "Albright's Syndrome"
What is Albright's Syndrome?
**Form of polystotic fibrous dysplasia in which there are MULTIPLE UNILATERAL bone lesions associated w/:

1) Endocrine Abnormalities
=Precocious puberty

2) Unilateral pigmented skin lesions
What is Polymyalgia Rheumatica?
**Pain + stiffness in shoulders and hips OFTEN w/ fever, malaise, and weight loss

=does NOT cause muscle weakness

**Occurs in patients > 50 y.o.

**Associated w/ Temporal (GIANT CELL) Arteritis
=can suspect polymyalgia rheumatica via an ELEVATED ESR
How would you treat Polymyalgia Rheumatica?
Prednisone
What is Polymyositis?
=Progressive proximal muscle weakness caused by CD8+ T-cell-induced injury to myofibers

**Muscle biopsy w/ evidence of inflammation is diagnostic.
What is Dermatomyositis?
**Similar to polymyositis BUT also involves a SKIN RASH + an increased risk of MALIGNANCY
What do labs for polymyositis/dermatomyositis show?
1) Elevated CK

2) Elevated ALDOLASE

3) Positive ANA

4) Anti-Jo-1
What is mixed connective tissue disease?
1) Raynaud's Phenomenon
=vasospastic disorder causing discoloration of fingers/toes

2) Arthralgias and myalgias

3) Fatigue

4) Esophageal hypomotility
Etiology:
AUTOIMMUNE DISEASE

**Antibodies to U1-RNP
What is the classic triad of Sjogren's Syndrome?
1) Xerophthalmia
=dry eyes, conjunctivitis

2) Xerostomia
=dry mouth, dysphagia

3) Arthritis
=i.e. associated w/ RHEUMATOID ARTHRITIS --> most people will be RF +

=i.e. immune cells attack and destroy exocrine glands that produce tears and saliva

**Also may have parotid enlargement.
Who does it predominantely affect?
FEMALES between 40 and 60 y.o.
Risks of Sjogren's:
**Increased risk of B-cell lymphoma
Laboratory Results:
1) Autoantibodies to ribonucleoprotein antigens

2) Anti-nuclear Abs (=ANAs)
= SS-A (Ro)
= SS-B (La) --> more specific
What is SICCA SYNDROME?
**The constellation of clinical SYMPTOMS relating to the actual salivary gland destruction:

=dry eyes
=dry mouth
=nasal + vaginal dryness
=chronic bronchitis
=reflux esophagitis

**NOW, when you COMBINE these symptoms w/ a CT disorder (=usually RA) --> you get SJOGREN'S SYNDROME
What population do you normally find SLE in?
**90% female between 14 and 45 (=reproductive age)

**Most common + severe in BLACK females
Symptoms of SLE:
DOPAMINe RASH

1) Discoid Rash

2) Oral ulcers

3) Photosensitivity

4) Arthritis
=NOT usually bone-destructive

5) Malar rash
=BUTTERFLY WING rash

4) Immunoglobulins
=anti-dsDNA
=anti-Sm
=antiphospholipid ab

5) Neurologic disorders

6) Renal disorders

7) Antinuclear antibody

8) Serositis
=pleuritis
=pericarditis

9) Hematologic disorders
Other Symptoms:
=Fever

=Fatigue

=Weight loss

=Nonbacterial verrucous endocarditis --> Liebmann Sacks

=Hilar adenopathy

=Raynaud's phenomenon
What specifically is going on in the kidney?
**Membranous glomerulonephritis with WIRE LOOP lesions in the kidney:
=due to immune complex deposition on the BM

=Causes NEPHROTIC SYNDROME --> kidneys damaged + so leak protein into urine = proteinuria

**Death is often from RENAL FAILURE as well as INFECTIONS.
Lab Tests Will Detect the Presence of:
1) ANA
=sensitive but NOT specific for SLE

2) anti-dsDNA
=Abs to double-stranded DNA
=VERY specific --> POOR prognosis

3) Anti-Smith (anti-Sm) Abs
=VERY specific but NOT prognostic

4) Antihistone Abs
=seen in DRUG-INDUCED lupus

**Patients might also have false positives on syphilis tests (RPR/VDRL)
=due to antiphospholipid antibodies

COLOR IMAGE 52
What is Celiac Sprue?
**Autoimmune-mediated intolerance of GLIADIN (=wheat) --> leading to STEATORRHEA

**Associated w/ people of northern european descecnt
Findings:
**Tends to affect the JEJUNUM (=small bowel)

1) Blunting of villi
2) Lymphocytes in the lamina propria
3) Abnormal D-xylose test

**Can be associated w/ dermatitis herpetiformis
=itchy cutaneous condition linked to the transglutaminase enzyme in the skin
Complication/Risk:
10-15% lead to MALIGNANCY
=usually T-cell lymphoma
What is Gout?
**Precipitation of monosodium urate crystals into joints due to HYPERURICEMIA
What can cause hyperuricemia?
1) Lesch-Nyhan Syndrome

2) PRPP Excess

3) Decreased excretion of uric acid

4) Glucose-6-Phosphate Deficiency

5) Associated w/ use of thiazide diuretics
=bc they competitively inhibit the secretion of uric acid
What is Lesch-Nyhan Syndrome?
=build-up of uric acid in all body fluids
=causes gout, poor muscle control, + moderate MR
=kiddos have self-mutilating behaviors--i.e. try to bite off their fingers/lips
Classic Presentation:
**More common in MEN

**Assymetric joint distribution

**Painful MTP joint in the big toe (=podagra --> gout of the foot)

**Tophus formation
=often on external ear OR Achille's tendon
When do "attacks" of gout usually occur?
**Acute attacks tend to occur after alcohol consumption OR a large meal

COLOR PLATE 54
What do the urate crystals look like?
=Needle shaped + NEGATIVELY birefringement
Treatment of Gout:
1) Allopurinol

2) Probenecid

3) Colchicine

4) NSAIDs
What is PSEUDOgout?
**Caused by deposition of calcium pyrophosphate crystals w/i the joint space.

**Usually affects LARGE joints
=classically the KNEE

**Occurs in people >50 y.o. --> BOTH sexes affected equally

**NO treatment
What do the crystals look like?
**Basophilic, RHOMBOID crystals that are WEAKLY POSITIVELY BIFRINGENT

=i.e. as opposed to the negatively birefringent needle-shaped crystals in gout
What is Goodpasture's Syndrome?
**Type II Hypersensitivity Reaction
=autoimmune disease where Abs attack specific antigens (i.e. Goodpasture's antigens) in the glomeruli of the KIDNEY and ALVEOLI of lungs

**Most common in MEN 20-40 y.o.
Findings:
**Most patients present w/ BOTH lung and kidney disease, but they can present w/ one alone:

1) Pulmonary hemorrhages --> hemoptysis

2) Renal lesions --> hematuria, anemia, + crescentic glomerulonephritis (i.e. rapidly-progressive glomerulonephritis)

**Anti-glomerular basement membrane Abs (Anti-GBM Abs) produce LINEAR STAINING on immunoflorescence.
What is Sarcoidosis?
**Characterized by immune-mediated, widespread NONCASEATING granulomas + elevated serum ACE levels

=granulomas most often appear in the LUNGS and LYMPH NODES

**Common in BLACK FEMALES
SO...Symptoms of Sarcoidosis
"GRAIN"

=Gammaglobulinemia
=RA
=ACE increase
=Interstitial fibrosis
=Noncaseating granulomas
Associated with:
1) Restrictive lung disease

2) Bilateral hilar lymphadenopathy

3) Erythema nodosum

4) Bell's Palsy

5) Epithelial granulomas containing microscopic Schaumann + asteroid bodies

6) Uveoparotitis

7) Hypercalcemia
=due to elevated conversion of vitamin D to its active form in epitheliod macrophages

**IMAGE 104
Seronegative Spondyloarthropathies
**Arthritis WITHOUT RF
=i.e. NO anti-IgG antibody

**Strong association w/ HLA-B27
=i.e. the gene that codes for HLA MHC I

**Occurs more often in MALES

**Includes:
=Ankylosing Spondylitis
=Reiter's Syndrome
Ankylosing Spondylitis
**Chronic inflammatory disease of the spine + sacroiliac joints --> results in:

1) Ankylosis (=stiff spine)
2) Uveitis
3) Aortic regurgitation
Reiter's Syndrome
CLASSIC TRIAD:

1) Urethritis
2) Conjunctivitis + anterior uveitis
3) Arthritis

**Can't SEE (=anterior uveitis/conjunctivits), can't PEE (=urethritis), can't climb a TREE (=arthritis)

**Occurs post-GI OR CHLAMYDIA infections.
Scleroderma
="Progressive Systemic Sclerosis" (PSS)

**Excessive fibrosis + collagen deposition throughout the body--mainly skin

**Commonly involves sclerosis of the SKIN but can also involve the CV + GI systems and the KIDNEY

**75% female

**2 Major Categories:
=Diffuse Scleroderma
=CREST Syndrome
What is Diffuse Scleroderma?
**Widespread skin involvement and EARLY visceral involvement

**Rapid progression
=bc this is the more generalized, systemic type--can be fatal due to heart, kidney, lung, or intestinal damage!

**Associated w/ anti-Scl-70 antibody
What is the CREST Syndrome?
**CREST

=Calcinosis
=Raynaud's phonomenon
=Esophageal dysmotility
=Sclerodactyly
=Telangiectasia (i.e. small enlarged blood vessels near surface of skin)

**Limited skin involvement--often CONFINED to fingers + face

**More benign clinical course

**Associated w/ ANTICENTROMERE ANTIBODY

COLOR PLATE 53
Dermatitis
**Group of inflammatory pruritic skin disorders

Etiology:
=Allergy (usually Type IV hypersensitivity)
=Chemical injury
=Infection
Atopic Dermatitis
**"eczema"**

=Pruritic eruption--commonly on FLEXOR surfaces

**Often associated w/ other atopic diseases
=asthma
=allergic rhinitis
Allergic Contact Dermatitis
**Type IV hypersensitivity reaction that follows exposure to allergen:
=poison ivy/oak
=nickel
=rubber
=chemicals

**Lesions occur at SITE of contact
Psoriasis
**Epidermal hyperplasia (=acanthosis) w/ parakeratotic scaling (i.e. nuclei still in stratum corneum) especially on the KNEES and ELBOWS

**"Red patches w/ silvery scales"**
Findings in Psoriasis:
1) Increased stratum spinosum
2) DECREASED stratum granulosum

COLOR IMAGE 65

**Auspitz sign
=punctate bleeding sites when psoriasis scales are scraped off
Dermatitis herpetiformis
**Pruritic papules and vesicles

**Associated w/ CELIAC DISEASE
Lichen planus
**Pruritic, purple, polygonal papules

=infiltrate of lymphocytes at dermoepidermal junction
Erythema multiforme
**Associated w/ infections, drugs, cancers, and autoimmune disease

**Presents w/ multiple types of lesions including:
=macules
=papules
=vesicles
=target lesions (i.e. red papules w/ a pale central area)
What is a major form of Erythema multiforme?
**Stevens-Johnson Syndrome

**Characterized by:
=high fever
=bulla formation and necrosis
=ulceration of the skin
=high mortality rate
Seborrheic keratosis
**Flat, pigmented squamous epithelial proliferation w/ keratin-filled cysts (=HORN CYSTS)

**Benign--lesions look like they were "pasted on"
Actinic keratosis
**CAUSED BY SUN EXPOSURE
=small, rough erythematous or brownish papules

**PREMALIGNANT lesion
=risk of progressing to squamous cell carcinoma

**Risk is proportional to epithelial dysplasia
Keloid
**Tumor of CT elements of dermis that causes raised, thickened scars

**Follows trauma to the skin, especially in African Americans
Bullous pemphigoid
**Autoimmune disorder w/ IgG antibody against EPIDERMAL BASEMENT MEMBRANE
=i.e. shows linear immunoflorescence

**Similar but LESS SEVERE than pemphigus vulgaris --> i.e. affects skin but SPARES oral mucosa

COLOR IMAGE 64
Pemphigus vulgaris
**Potentially fatal autoimmune skin disorder
=intradermal bullae involving the oral mucosa

**SO, skin basically sloughs off!
Findings in Pemphigus vulgaris:
1) Acantholysis
=breakdown of epithelial cell-to-cell junctions

2) IgG antibody against epidermal cell surface
=immunoflorescence throughout epidermis

COLOR IMAGE 63
Squamous Cell Carcinoma
**Very common

**Associated with:
=Excessive SUN exposure
=Arsenic exposure

**LOCALLY invasive but rarely metastasizes

**Precursor = actinic keratosis
Where does SCC commonly appear?
Hands and Face
Histopathology?
KERATIN PEARLS

(COLOR IMAGE 60)
Basal Cell Carcinoma
**Most common in SUN-EXPOSED areas of the body

**Locally invasive BUT almost NEVER metastasizes
GROSS and HISTOLOGIC apperance:
GROSS
=pearly papules
=COLOR IMAGE 62

HISTOLOGICALLY
="palisading" nuclei
Melanoma
**Common tumor w/ significant risk of metastasis
=DEPTH of tumor correlates w/ risk of metastasis

**Precursor lesion = DYSPLASTIC NEVI

**Associated w/ SUNLIGHT EXPOSURE
=fair-skinned people are at an increased risk
Primary Bone Tumors
BENIGN
=Giant Cell Tumor
=Osteochondroma
=Enchondroma

MALIGNANT
=Osteosarcoma
=Ewing's Sarcoma
=Chondrosarcoma
Giant Cell Tumor
**Occurs most commonly at the epiphyseal end of long bones
=LOCALLY AGGRESSIVE benign tumor often around the distal femur/proximal tibial region

**Peak incidence 20-40 y.o.
Appearance on X-Ray:
=characteristic "DOUBLE BUBBLE" or "SOAP BUBBLE" appearance on X-ray
Histology:
Spindle-shaped cells + MULTINUCLEATED GIANT CELLS
Osteochondroma
(=exostosis)
**MOST COMMON BENIGN BONE TUMOR
=usually MEN <25 y.o.

**Commonly originates from long metaphysis

**Malignant transformation to chondrosarcoma is RARE
Enchondroma
**Benign cartilaginous neoplasm found in intramedullary bone

**Usually DISTAL EXTREMITIES (vs. chondrosarcoma)
Osteosarcoma
(=osteogenic carcinoma)
**MOST COMMON MALIGNANT TUMOR OF BONE

**Peak incidence in men 10-20 y.o.

**Commonly found in the metaphysis of LONG BONES
Predisposing Factors:
1) Paget's disease of bone
2) Bone infarcts
3) Radiation
4) Familial retinoblastoma
X-ray Findings:
CODMAN'S TRIANGLE

=due to elevation of the periosteum
Ewing's Sarcoma
**Anaplastic small blue cell malignant tumor

**MOST COMMON in boys <15
=associated w/ an 11:22 translocation

**Extremely aggressive w/ early metastasis BUT responsive to chemo.

**Commonly appears in the diaphysis of long bones, pelvis, scapula, and ribs
Appearance of Bone:
**characteristic "ONION SKINNING" appearance
Chondrosarcoma
**Malignant cartilaginous tumor

**Usually found in MEN age 30-60

**Usually located in the:
=pelvis
=spine
=scapula
=humerus
=tibia
=femur

**May be of PRIMARY origin OR from an OSTEOCHONDROMA
What is Buerger's Disease?
**Also known as "thromboangiitis obliterans"

=Idiopathic, segmental, thombosing vasculitis (inflammation + thrombosis) of intermediate and small peripheral arteries and veins--usually of the feet and hands

**Seen in HEAVY SMOKERS
Findings:
1) Intermittent claudication
2) Superficial nodular phlebitis
3) Cold sensitivity
=Raynaud's phenomenon
4) Severe pain in affected part

**May lead to GANGRENE

**Treatment = QUIT smoking!
Takayasu's Arteritis
**Known as "pulseless disease"

=granulomatous thickening of the aortic arch and/or proximal great vessels (i.e. medium + large arteries)

**Associated w/ an elevated ESR

**Primarily affects ASIAN FEMALES < 40 y.o.
Symptoms:
**"FAN MY SKIN On Wednesday"

=Fever
=Arthritis
=Night sweats
=MYalgia
=SKIN nodules
=Ocular disturbances
=Weak pulses in upper extremities
Temporal (GIANT CELL) Arteritis
**Most common vasculitis that affects MEDIUM and SMALL arteries --> usually branches of the CAROTID ARTERY

=Focal, granulomatous

**ESR MARKEDLY elevated

**Affects elderly females

**GIVE STEROIDS ASAP to prevent BLINDNESS!
Findings:
1) Unilateral headache

2) Jaw claudication

3) Impaired vision
=occlusion of the opthalmic artery --> can lead to blindness

4) 1/2 of patients have SYSTEMIC involvement + Polymyalgia Rheumatica
=proximal muscle pain + periarticular pain
Polyarteritis nodosa
**Characterized by necrotizing immune complex inflammation of MEDIUM-SIZED MUSCULAR arteries

=typically involves RENAL and VISCERAL vessels

**Lesions are of different ages!
Symptoms:
1) Fever

2) Weight loss

3) Malaise

4) Abdominal pain + melena

5) Headache

6) Myalgia

7) HTN

8) Neurologic dysfunction

9) Cutaneous eruptions
Findings:
**Hepatitis B seropositivity in 30% of patients!

**Multiple aneurysms and constrictions on arteriogram

**NOT associated w/ ANCA (anti-neutrophil cytoplasmic antibody)
Treatment:
Corticosteroids, cyclophosphamide
Wegener's Granulomatosis
**Characterized by a TRIAD of:

1) Focal necrotizing vasculitis

2) Necrotizing granulomas in the lung + upper airway

3) Necrotizing glomerulonephritis
Symptoms:
1) Perforation of the nasal septum

2) Chronic sinusitis

3) Otitis media

4) Mastoiditis

5) Cough, dyspnea, hemoptysis

6) Hematuria

**i.e. form of vasculitis that affects mainly the LUNGS and KIDNEYS
Findings:
**C-ANCA**
=strong marker of disease

**Chest X-ray may reveal LARGE NODULAR DENSITIES

**In urine:
=hematuria
=red cell casts
Treatment:
Cyclophosphamide and corticosteroids
Microscopic polyangiitis
**Like Wegener's but LACKS granulomas
=can be P (=perinuclear; usually) or C-ANCA
Primary pauci-immune crescenteric glomerulonephritis
Vasculitis limited to KIDNEY
=usually P-ANCA
Churg-Strauss Syndrome
**Granulomatous vasculitis w/ EOSINOPHILIA
=usually P-ANCA

**Involves:
=Lungs (mainly)
=Heart
=Skin
=Kidneys
=Nerves

**Often seen in atopic patients
=i.e. begins as a worsening of allergies OR new onset allergies --> progresses into ACUTE ASTHMA --> symptoms involving various organ systems
Kawasaki Disease
**Acute/self-limiting disease of infants/kids

=ACUTE NECROTIZING vasculitis of small/medium sized vessels
Symptoms:
=Fever

=Congested conjunctiva (red eyes)

=Changes in lips/oral mucosa (red cracked lips, red oral mucosa + "strawberry tongue")

=Lymphadenitis

**May develop CORONARY ANEURYSMS
Henoch-Schonlein Purpura
**Most common form of childhood SYSTEMIC VASCULITIS
=usually preceded by an URTI
=characterized by prominent tissue deposition of IgA-containing immune complexes esp. in the skin and kidney

Symptoms:
=purpuric skin rash
=arthralgia
=intestinal hemorrhage
=abdominal pain
=melena