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What are two things that characterize sarcoidosis?
widespread non-caseating granulomas, elevated ACE levels.
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What does GRAIN stand for?
Gammaglobulinemia, Rheumatoid arthritis, ACE elevation, Interstitial fibrosis, Non-caseating granulomas -- (sarcoidosis).
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What is the treatment for sarcoidosis?
Steroids.
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Polymyositis?
Symmetrical proximal muscle weakness by CD8+ T-cell injury, most often shoulders, muscle inflammation diagnostic.
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Dermatomyositis?
Polymyositis also involving malar and heliotrope rashes, gottron's papules, has increased risk of malignancy.
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What characterizes Scleroderma (Progressive systemic sclerosis)?
Excessive fibrosis and collagen deposition systemically, commonly in skin (derma), involves renal, pulmonary, CV, and GI systems.
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What are the two major types of Scleroderma?
Diffuse scleroderma and CREST syndrome.
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What is Diffuse scleroderma, which antibody is associated?
Widespread skin involvement, early visceral involvement, anti-Scl-70 antibody.
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CREST syndrome and antibody?
Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly, Telangienctasia -- antiCentromere antibody, most benign course.
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What are verrucae?
warts, soft, tan-colored, cauliflower-like lesions -- epidermal hyperplasia, hyperkeratosis, koilocytosis.
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Urticaria?
Hives, wheals, pruritic, common, mast cell degranulation (hypersensitivity type II).
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Atopic dermatitis (eczema)?
Pruritic eruption, skin flexures, associated with other atopic diseases (asthma, allergic rhinitis).
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Contact dermatitis (allergy)?
Type IV hypersensitivity on site of exposure to allergen.
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Psoriasis?
Autoimmune, silvery, scaly papules and plaques, knees and elbows (extensor), acanthosis w/parakeratotic scaling, increased spinosum, decreased granulosum, nail pitting and psoriatic arthritis -- auspitz sign: bleeding spots when scales scraped off.
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What is parakeratosis?
The nuclei remain in keratinocytes of stratum corneum.
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Seborrheic keratosis?
Flat, greasy squamous epithelial proliferation with keratin filled cysts, head, trunk, extremeties, benign.
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What is the sign of Leser-Trelat?
Sudden appearance of multiple seborrheic keratosis may indicate an underlying malignancy.
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What are 4 characteristics of Osteogenesis imperfecta?
AD, Defetective collagen 1 synthesis, blue sclera, deafness.
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How do you treat osteogenesis imperfecta?
bisphosphonates (increase bone mineralization).
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What is the main mutation in achondroplasia?
FGFR3 (constituvely active), AD.
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What are the two types of osteopetrosis?
AR (severe), AD (less severe).
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Pathogenesis osteopetrosis?
Osteoclast deficiency, carbonic anhydrase II deficiency, "too much bone" (cortical).
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Clinical findings of osteopetrosis?
Anemia, pathologic fractures, cranial nerve compression (visual and hearing loss), normal levels of ALP, serum calcium, phosphate.
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What is the most common site and organism in osteomyelitis?
Metaphysis (tibia and fibula), staph aureus.
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What is the common organism in sickle cell patients?
Salmonella paratyphi.
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Pott's disease?
Tuberculosus osteomyelitis, hematogenous spread to vertebral column and intervertebral disc.
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What is the sequestrum, involucrum?
Sequestrum: area of neutrophilic necrosis, involucrum: reactive bone formation around sequestrum.
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When do you see pseudomonal aeruginosa osteomyelitis?
Puncture of foot through rubber footwear.
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What is osteoporosis?
Decreased bone mass, primarily trabecular bone.
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What are the two classifications of osteoporosis?
Primary: most common, idiopathic in children, postmenopausal, senile -- Secondary: underlying disease, drugs hypogonadism, malnutrition, space travel.
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Pathogenesis of postmenopausal osteoporosis?
Estrogen deficiency -> increased osteoclast resorption, decreased osteoblast bone formation.
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What are the three most common bone fractures in osteoclast?
Femoral neck, colles' fracture (distal radius), vertebral compression fracture.
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What is Mccune albright syndrome?
Polyostotic fibrous dysplasia, cafe au lait, precocious puberty.
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What is the most common bone affected in fibrous dysplasia?
Ribs.
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Clinical findings of fibrous dysplasia?
Pain overlying bone, swelling of bone.
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What is the most common malignancy of bone?
Metastasis.
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what is the most common primary site of metatastic bone tumor?
Brest cancer, prostate in men, then: lung, kidney.
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Most common primary bone tumor?
Multiple myeloma, osteogenic sarcoma, chondrosarcoma, Ewing's sarcoma.
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Osteochondroma?
Most common benign bone tumor, metaphysis of distal femur, outgrowth of bone (exostosis - includes medulla) capped by cartilage, loss of function mutation ext1, ext2, 10-30 y/o males.
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Enchondroma?
20-50 y/o, medullary location, small tubular bones in hands and feet, multiple enchondroma: risk of chondrosarcoma.
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Osteoid Osteoma?
10-20 y/o males, less than 2cm diameter, cortex of proximal femur, nocturnal pain responds to aspirin, radiolucent focus surrounded by sclerotic bone.
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Osteoma?
Any age, associated with Gardner's syndrome, new piece of bone grows on another piece in skull.
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Osteoblastoma?
Similar to osteoid osteoma, larger than 2cm, vertebral column, does not respond to aspirin.
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Giant cell tumor (osteoclastoma)?
Benign, 20-40 y/o females, epiphyseal distal femur, locally aggressive, multinucleated cells, "soap bubble" appearances.
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Osteosarcoma?
Malignant, 10-25 y/o male, codmann's triangle, metaphysis of distal femur, proximal tibia, very aggressive.
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Risk factors for osteosarcoma?
Familial retinoblastoma, paget's disease, radiation.
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Ewing's sarcoma?
Malignant, males 10-20 y/o, anaplastic blue cell tumor, overexpression of myc2 (11;22 translocation), diaphysis of long bones, pelvis, scapula, ribs, "onion peel" appearance, early metastasis, responsive to chemotherapy.
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Chondrosarcoma?
30-60 y/o male, pain and swelling, arise denovo within medullary cavity, pelvis, spine, scapula, not distal extremities, resistant to chemotherapy, metastasizes to lungs.
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Which crystals are negatively birefringent, what do they look like?
Monosodium Urate, needle-shaped (monoclinic) -- Gout.
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Calcium pyrophosphate crystals?
Pseudogout, rhomboid crystals, positive birefringence.
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What are mucin clots for?
Evaluate joint viscosity, amount of hyaluronic acid.
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What does poor mucin clot formation reflect?
Decreased hyaluronic acid, joint inflammation.
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What are noninflammatory joint disorders?
Osteoarthritis, neuropathic joint.
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Inflammatory joint disorders?
RA, gout.
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Septic arthritis?
Most common: gonococcemia, lyme disease.
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Hemorrhage joint disorders?
Trauma, hemophilia A and B.
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What does arthritis include?
Pain, joint swelling, tenderness, warmth.
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What disorders have characteristic morning stiffness (>30min)?
RA, polymyalgia rheumatica, SLE.
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What is the most common disabling joint disease?
Osteoarthritis.
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What are some secondary causes of osteoarthritis?
Legg-Calve-perthes, osteochondritis dissecans, obesity, trauma, neuropathic joint, meniscus injuries, hemochromatosis.
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What is Legg-Calve-Perthes disease?
Avascular necrosis of femoral head in children.
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Common sites of OA?
Femoral head, knee, cervical/lumbar vertebrae, hands.
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What collagen makes up articular cartilage?
Type II, and proteoglycans.
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What is the most common complaint of OA?
Pain, pain, pain.
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What joints are involved in OA of hands?
DIP's - heberden's node, PIP's - bouchard's nodes.
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What are some treatments for OA?
Heat, decreased weight bearing (cane), ROM exercise, analgesics, joint replacement.
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What is Charcot's joint?
Neuropathic arthropathy, non-inflammatory-- loss of proprioception, deep sensation -> leads to recurrent trauma.
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What are three causes of Charcot's joint?
DM (tarsometatarsal), syringomyelia (shoulder, elbow, wrist joints), Tabes dorsalis (hip, knee, ankle joints).
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What is syringomyelia?
Cyst in spinal cord.
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What is tabes dorsalis?
Demyelination of propioceptive neurons due to untreated syphilis infection.
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RA is associated with what gene?
HLA-DR4.
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What is rheumatoid factor?
IgM antibody against Fc portion of IgG.
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What cytokine is activated by immune complexes, who do they call in turn?
C5a, chemotactic for neutrophils and other leukocytes --> chronic synovitis and pannus formation.
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What is pannus?
Granulation tissue formed within synovial tissue by fibroblasts and inflammatory cells.
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What can fibrosis repair cause?
Ankylosis of joint.
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What are some clinical findings of RA?
Symmetric involvement of hand joints: MCP, PIP.
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Two deformities seen with RA?
Swan neck, boutonniere.
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How does RA affect lung?
Intertitial fibrosis, effusions, chronic pleuritis.
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Hematologic effects of RA?
Anemia of chronic disease (ACD), AIHA, Felty's syndrome.
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What is Felty's syndrome?
Autoimmune neutropenia and splenomegaly with RA.
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What is AIHA?
Autoimmune hemolytic anemia.
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What part of spine does RA affect?
Cervical spine -- subluxation of atlantoaxial joint, cord/vertebral artery compression.
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What is Caplan syndrome?
Rheumatoid nodules in lung plus pneumoconiosis.
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Where will you find Rheumatoid nodules?
Extensor forearm, lungs -- fibrinoid necrosis in center of nodules, correlates with very high RF.
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How does RA affect CV system?
Pericarditis, aortitis, vasculitis.
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What is a baker's cyst?
Outpouching of synovial sack in popliteal fossa - due to increased intra-articular pressure.
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Lab findings in RA?
positive ANA (sensitive not specific), positive RF, normal to increased serum C3, decreased synovial C3, increased serum total protein.
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Treatment of RA?
Movement of joints (swimming), initially with NSAIDS, Methotrexate, gold cpds, corticosteroids, cyclosporine, TNF-a blockers(if DMARD innefective).
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Which is the most commonly used DMARD?
Methotrexate.
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What are the characteristics of sjogren's syndrome?
Xerostomia, xeropthalmia, RA -- sicca syndrome -- female dominant disorder, autoimmune.
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Lab findings of sjogren's?
ANA, RF, Anti-SS-A Ro, Anti-SS-B La antibodies.
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What confirms sjogren's?
Lip biopsy to show lymphoid destruction of minor salivary glands.
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Treatment for sjogren's?
Artificial tears, pilocarpine, cevimeline.
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Lab results of Juvenile RA (JRA)?
Negative RF (seronegative).
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What are three types of JRA?
Polyarticular, pauciarticular, still's disease.
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What is still's disease?
JRA presents as infectious disease - fever, rash, polyarthritis.
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What complication is associated with pauciarticular JRA?
Uveitis (potential for blindness).
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Pathology of primary gout?
Inborn error of purine metabolism (HGPRT deficiency in lesch-nyhan).
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Secondary causes?
More common, underexcretion of uric acid (lead poisoning, alcoholism, red meat, beer) -- overproduction of uric acid (treating leukemia, psoriasis).
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Clinical conditions associated with gout?
Urate nephropathy, renal stones, hypertension, artery disease, Pb poisoning.
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Most commonly affected joint in gout?
Podagra: first metatarsalphalangeal joint.
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What initiates attack in acute gout?
Free uric acid crystals ->activate synovial cells ->leukocytes -> C5a -> neutrophils, phagocytose uric acid crystals, repeat.
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Lab finings in acute gout?
Hyperuricemia (>7mg/dl men, >6 women), absolute neutrophilic leukocytosis.
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What is confirmatory in acute gout?
Aspiration showing negative birefringent crystals.
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What is a tophus?
MSU deposits in soft tissue around the joint.
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Treatment of acute gout?
Diet modification, NSAIDS, colchicine, corticosteroids if intolerant to other rx.l
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Treatment of chronic gout?
Normalize serum uric acid: uricosuric agents for underexcretors (<700mg), allopurinol for overproducer (>900mg).
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What is a uricosuric agent?
Probenecid.
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How does allopurinol work?
Xanthine oxidase inhibitor - Hypoxanthine -> xanthine.
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What is CPPD?
Calcium pyrophosphate deposition -- pseudogout.
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What is the most common variant of CPPD?
CPPD OA variant: knee most common joint, chondrocalcinosis present.
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What are some characteristics of seronegative spondyloarthropathies?
RF negative, HLA-B27, male, sacroiliitis, spondilytis.
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What are the types of spondyloarthropathies?
Ankylosing spondylitis, Reiter's syndrome, psoriatic, enteropathic.
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How does Ankylosing spondylitis usually start?
Bilateral sacroiliitis with morning stiffness, leads to bamboo spine, kyphosis leads to nonpulmonary restrictive lung disease.
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What are some associations with AS?
aortitis, uveitis.
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What is the Schober test?
Evaluates degree of restriction to forward bending.
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What is Reiter's syndrome?
Chlamydia trachomatis infection leading to urethritis, conjunctivitis, arthritis.
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What is a diagnostic sign for Reiter's syndrome?
Achilles tendon periostitis.
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What has sausage shaped DIP joints and "pencil cup deformities"?
Psoriatic arthritis.
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