Musculoskeletal/Connective tissue
Terms
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- what type of ossification is not affected in achondroplasia?
- membranous - skull, facial bones, and axial skeleton are normal
- Heberden's nodes (DIP) and Bouchard's nodes (PIP)
- osteoarthritis
- pain in weight-bearing joints after use that improves with rest
- osteoarthritis
- destruction of articular cartilage, subchondral bone formation, sclerosis, osteophytes, and eburnation are associated with what?
- osteoarthritis
- what are joint mice? what are they associated with?
- osteophytes that fracture and float into synovial fluid along with fragments of separated cartilage - seen in osteoarthritis
- pannus formation in joints, especially MCP, PIP
- rheumatoid arthritis
- morning stiffness improving with use; symmetric joint involvement, and systemic symptoms (fever, fatigue, pleuritis, pericarditis)
- rheumatoid arthritis
- Rx for severe cases of osteoporosis?
- bisphosphonates or pulsatile PTH
- type I osteoporosis?
- postmenopausal - increased bone resorption due to decreased estrogen levels
- type II osteoporosis?
- senile osteoporosis - affects men and women over 70
- what types of fractures are associated with type I osteoporosis?
- vertebral crush fractures - acute back pain, loss of height, kyphosis
- what type of fractures are associated with type II osteoporosis?
- distal radius (Colles') fractures, vertebral wedge fractures
- in this disease there is a failure of normal bone resorption that leads to thickened, dense bones
- osteopetrosis (marble bone disease)
- in this disease osteoclasts function abnormally
- osteopetrosis
- what level of alk phos is osteopetrosis associated with?
- normal
- what causes osteitis fibrosa cystica?
- hyperparathyroidism
- this disease is characterized by 'brown tumors', high serum calcium, low serum phosphorus, and high alk phos
- osteitis fibrosa cystica
- these are cystic spaces lined by osteoclasts, filled with fibrous stroma and sometimes blood
- brown tumors (seen in osteitis fibrosa cystica)
- in this disease there is abnormal bone architecture caused by an increase in both osteoblastic and osteoclastic activity
- Paget's disease (osteitis deformans)
- what level of alk phos is Paget's disease associated with?
- increased (normal serum calcium, phosphorus, adn PTH)
- in this disease, bone is replaced by fibroblasts, collagen, and irregular bony trabeculae
- polyostotic fibrous dysplasia
- Albright's syndrome is a form of what?
- polyostotic fibrous dysplasia
- multiple unilateral bone lesions, unilateral ppigmented skin lesions, precocious puberty
- Albright's syndrome
- pain and stiffness in shoulders and hips, often with fever, malaise and weight loss; doesn't cause muscular weakness
- polymyalgia rheumatica
- what is polymyalgia rheumatica associated with?
- temporal (giant cell) arteritis; occurs in patients over 50
- is ESR increased or decreased in polymyalgia rheumatica?
- increased
- progressive proximal muscle weakness caused by CD8+ T cell-induced injury to myofibers
- polymyositis
- muscle biopsy with evidence of inflammation is diagnostic for what?
- polymyositis
- increased CK, increased aldolase, and positive ANA, anti-Jo-1
- polymyositis/dermatomyositis
- Raynaud's, arthralgias, myalgias, fatigue, esophageal hypomotility
- mixed CT disease
- antibodies to U1RNP?
- mixed connective tissue disease
- what is the classic triad of Sjogren's syndrome?
- xerophthalmia, xerostomia, arthritis
- patients with Sjogren's syndrome are at increased risk for what?
- B cell lymphoma
- what is enlarged in Sjogren's syndrome?
- parotids
- what patients does Sjogren's primarily affect?
- females between 40-60
- autoantibodies to ribonucleoprotein antigens, SS-A (Ro), SS-B (La)
- Sjogren's
- fever, fatigue, weight loss, nonbacterial verrucous endocarditis, hilar adenopathy, and Raynaud's
- SLE
- what are the kidney findings in SLE?
- wire loop lesions in kidney with immune complex deposition (with nephrotic syndrome)
- what part of the SI does celiac sprue tend to affect?
- jejunem
- what skin finding is celiac sprue associated with?
- dermatitis herpetiformis
- what malignancy is celiac sprue most strongly affiliated with?
- T cell lymphoma
- blunting of villi and lymphocytes in the lamina propria are suggestive of what?
- celiac sprue
- what type of crystals precipitate in gout?
- monosodium urate
- what type of diuretics is gout associated with?
- thiazide
- needle shaped negatively birefringent
- gout
- with what type of crystals is pseudogout associated?
- calcium pyrophosphate
- rhomboid crystals that are weakly positively birefringent
- pseudogout
- what type of joints does pseudogout usually affect?
- large joints, especially the knee
- what is the treatment for pseudogout?
- none
- pulmonary hemorrhages leading to hemoptysis, renal lesions leading to hematuria, anemia, crescentic glomerulonephritis
- Goodpasture's syndrome
- what type of hypersensitivity reaction does Goodpasture's represent?
- type II
- anti-glomerular basement membranes produce what type of staining on immunofluorescence?
- linear
- what population does Goodpasture's usually affect?
- men 20-40
- what level is elevated in sarcoidosis?
- ACE
- immune-mediated, widespread noncaseating granulomas common in black females
- sarcoidosis
- what do the epithelial granulomas contain in sarcoidosis?
- microscopic Schaumann and asteriod bodies
- why is hypercalcemia associated with sarcoidosis?
- elevated conversion of vitamin D to its active form in epitheliod macrophages
- restrictive lung disease, bilateral hilar lymphadenopathy, erythema nodosum, Bell's palsy are associated with what?
- sarcoidosis
- patients with what disorder have decreased sensitivity and often anergy to skin test antigens?
- sarcoidosis
- chronic inflammatory disease of spine and SI joints -> ankylosis, uveitis, and aortic regurgitation
- ankylosing spondylitis
- this disorder is characterized by excessive fibrosis and collagen deposition throughout the body
- scleroderma (progressive systemic sclerosis)
- widespread skin involvement, rapid progression, early visceral involvement
- diffuse scleroderma
- CREST syndrome
- calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia
- idiopathic, segmental, thrombosing vasculitis of intermediate and small peripheral arteries and veins; seen in heavy smokers
- Buerger's disease
- intermittent claudication, superficial nodular phlebitis, and cold sensitivity, severe pain in affected part (may lead to gangrene) are characteristic of what?
- Buerger's disease
- what is the treatment for Buerger's disease?
- quit smoking
- what type of arteries does Takayasu's arteritis affect?
- medium and large arteries
- granulomatous thickening of aortic arch and/or proximal great vessels; elevated ESR
- Takayasu's arteritis ("pulseless disease")
- who does Takayasu's arteritis primarily affect?
- Asian females under 40
- what are the symptoms of Takayasu's arteritis?
-
fever, arthritis, night sweats, myalgia, skin nodules, ocular disturbances, weak pulses in upper extremities:
FAN MY SKIN On Wednesday - this is the most common vasculitis that affects medium and small arteries, usually branches of the carotid
- temporal/giant cell arteritis
- unilateral headache, jaw claudication, impaired vision (occlusion of ophthalmic artery, which can lead to blindness)
- temporal/giant cell arteritis
- half of patients with temporal arteritis have what?
- systemic involvement and polymyalgia rheumatica (proximal muscle pain, periarticular pain)
- what is elevated in temporal arteritis? what is the treatment?
- elevated ESR; steroids are Rx
- necrotizing immune complex inflammation of medium-sized muscular arteries, typically involving renal and visceral vessels
- polyarteritis nodosa
- approximately 30% of patients with polyarteritis nodosa are seropositive for what?
- Hep B
- multiple aneurysms and constrictions are seen on angiogram in what disease?
- polyarteritis nodosa
- what is the treatment for polyarteritis nodosa?
- corticosteroids, cylophosphamide
- what is the triad associated with Wegener's granulomatosis?
- necrotizing vasculitis, necrotizing granulomas in the lung and upper airway, and necrotizing glomerulonephritis
- perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis, cough, dyspnea, hemoptysis, and hematuria are associated with what disease?
- Wegener's granulomatosis
- what is a strong marker of Wegener's granulomatosis?
- C-ANCA
- what might be seen on chest x ray in Wegener's?
- large nodular densities
- what is the treatment for Wegener's?
- cyclophosphamide and corticosteroids
- this vasculitide is like Wegener's but lacks granulomas and is associated with P- or C-ANCA
- microscopic polyangitis
- this is a vasculitis limited to the kidney
- primary pauci-immune crescentic glomerulonephritis
- granulomatous vasculitis with eosinophilia that involves lungs, heart, skin, kidneys, nervous system; often seen in atopic patients
- Churg-Strauss syndrome
- this is an acute, self-limiting disease of infants/kids; acute necrotizing vasculitis of small/medium sized vessels; fever, congested conjunctiva, changes in lips/oral mucosa, lymphadenitis
- Kawasaki disease/ mucocutaneous lymph node syndrome
- what potentially serious outcome can result from Kawakaki disease?
- coronary aneurysms
- what is the most common form of childhood systemic vasculitis?
- Henoch-Schonlein purpura
- skin rash, arthralgia, intestinal hemorrhage, abdominal pain, and melena in kid?
- Henoch-Schonlein purpura
- where do giant cell bone tumors most commonly occur? in what age group?
- epiphyseal end of long bones - 20-40 years old
- this is a locally aggressive benign tumor often around the distal femur, proximal tibial region
- giant cell tumor
- what type of bone tumor has a 'soap bubble' or 'double bubble' appearance on x-ray?
- giant cell tumor
- spindle-shaped cells with multinucleated giant cells found in what type of benign bone tumor?
- giant cell tumor
- what is the most common benign bone tumor?
- osteochondroma (exostosis)
- this type of bone tumor is usually found in men <25 and commonly originates from long metaphysis
- osteochondroma
- this is a benign cartilaginous neoplasm found in intramedullary bone, usually in the distal extremities
- endochondroma
- what is the most common malignant tumor of bone?
- osteosarcoma
- this malignant bone tumor's peak incidence is in men 10-20 years old and is commonly found in the metaphysis of long bones
- osteosarcoma
- name some predisposing factors for osteosarcoma
- Paget's disease of bone, bone infarcts, radiation, and familial retinoblastoma
- Codman's traingle or sunburst pattern on x-ray
- osteosarcoma
- this is an anaplastic small blue cell tumor of bone most common in boys <15
- Ewing's sarcoma
- what is the characteristic appearance of Ewing's sarcoma?
- onion skin
- this malignant bone tumor is aggressive with early mets but is responsive to chemo; commonly appears in diaphysis of long bones, pelvis, scapula, and ribs
- Ewing's sarcoma
- what is the chromosomal translocation associated with Ewing's sarcoma?
- 11; 22
- this is a malignant cartilaginous tumor most common in men 30-60
- chondrosarcoma
- where are chondrosarcomas usually located? what can they originate from?
- pelvis, spine, scapula, humerus, tibia, or femur; may be primary or from osteochondroma (rare)