Joint/muscle pathologies etc. Flashcards
What collects in the joints in someone who has gout?
Monosodium urate crystals
Which is more common, the overproduction or uric acid or underexcretion?
Undersecretion of uric acid
Causes of undersecretion of uric acid
mostly idiopathic, but potentiated by renal failure
Causes of overproduction of uric acid
Lesch-Nyhan syndrome, PRPP excess, increased cell turnover
Findings under polarized light of joint fluid from someone with gout
Needle shaped crystals with - bifringence
-yellow under parallel light, blue under perpendicular light
Treatment of acute gout attack
NSAIDs, glucocorticoids, colchicine
Treatment of chronic gout attack
Xanthine oxidase inhibitors
What is calcium pyrophosphate deposition disease AKA
pseudogout
What deposits in the joints of someone with calcium pyrophosphate deposition disease?
calcium pyrophosphate
Most affected joint in calcium pyrophosphate deposition disease?
Knee
What will you see on xray of someone with calcium pyrophosphate deposition disease?
chondrocalcinosis
What do the crystals in calcium pyrophosphate deposition disease look like?
rhomboid and weakly + birefringent (blue when parallel to light)
acute treatment of calcium pyrophosphate deposition disease
NSAIDs, colchicine, glucocorticoids
Prophylaxis of calcium pyrophosphate deposition disease
colchicine
What is the presentation of systemic juvenile idiopathic arthritis?
Daily spikign fevers, salmon-pink macular rash, arthritis, leukocytosis, thrombocytosis, anemia,
Treatment of systemic juvenile idiopathic arthritis?
NSAIDs, steroids, methotrexate, TNF inhibitors
Sjogren syndrome pathophys
autoimmune disorder characterized by desctruction of exocrine glands by lymphocytes
Clinical findings in sjogrens syndrome
Inflammtory joint pain
Keratoconjunctivitis sicca (decreased tear production and corneal damage)
Xerostomia
Bilateral parotid enlargement
What will you find in the blood of someone with Sjogrens?
ANA antibodies, rheumatoid factor, and antiribonucleoprotein antibodies: SS-A, SS-B
In what condition other than Sjogrens may someone e have positive anti-SSA and anti-SSB antibodies?
SLE
Complications of sjogrens
dental caries
MALT (may present as parotid enlargement)
What confirms the diagnosis of sjogrens syndrome?
lymphocytic sialadenitis on labial salivary gland biopsy
What are the four types of seronegative spondyloarthritis?
Psoriatic arthritis
Ankylosing spondylitis
Inflammatory bowel disease
Reactive arthritis
What are seronegative spondyloarthritis’s?
arthritis without rheumatoid factor (no igG antibody)
what HLA type are seronegative spondyloarthritis’s associated with?
HLA-B27
What do the seronegative spondyloarthritis’s have in common as presentation ?
inflammatory back pain that improves with exercise, peripheral arthritis, enthesitis and dactylitis, uveitis
What may you see on XRAY of someone with psoriatic arthritis?
Pencil in cup deformity
What is the presentation of someone with ankylosing spondylitis?
Arthritis with symmetric involvement of spine and SI joints which leads to joint fusion
-also uveitis, aortic regurgitation and ankylosis
What may be seen on XRAY of someone with ankylosing spondylitis?
Bamboo spine
Complication of ankylosing spondylitis?
Restrictive lung disease
Explain the two different types of seronegative spondyloarthritis seen in IBD patients
Type I: <5 joints, large joints, worse w/ flares
Type II: >5 joints, small, independent
What is the triad seen in reactive arthritis?
conjunctivitis, urethritis, arthritis
What is SLE?
A relapsing and remitting autoimmune disease
Pathophys of lupus?
organ damage primarily due to type III hypersensitivity (partially type II)
-associated with early complement protein deficiency leading to decreased clearance of immune complexes
How does lupus classically present?
rash, joint pain, fever, in female of reproductive age (especially african-american or Hispanic women)
What type of endocarditis is associated with SLE?
Libman-Sacks Endocarditis
What is libman-sacks endocarditis?
nonbacterial thrombi on mitral or aoric valve that may be present on either side of the valve
What is lupus nephritis?
glomerular deposition of DNA-anti-DNA immune complexes that leads to nephrotic or nephritic syndrome - most commonly diffuse proliferative
What is an anti-SSA + pregnant women at risk of?
the newborn developing neonatal lupus
Presentation of neonatal lupus?
congenital heart block, periorbital/diffuse rash, transaminitis, and cytopenias
What is a sensitive antibody for lupus?
ANA
What are some specific antibodies for lupus?
Sensitive - ANA
Specific - anti-dsDNA and anti-SM
What is mixed connective tissue disease?
Disease with features of SLE, systemic sclerosis, and or polymyositis
What antibody is associated with mixed connective tissue disease?
anti-U1 RNP antibodies
How does antiphospholipid syndrome present?
venous/arterial thrombosis and reccurrent fetal loss
What lab findings are present in antiphospholipid syndrome?
lupus anticoagulant, anticardiolipin, anti-B2 glycoprotein I antibodies
Treatment of antiphospholipid syndrome ?
systemic anticoagulation
antiphospholipid syndrome may occur secondarily to what?
SLE
Presentation of polymyalgia rheumatica?
Bilateral pain and stiffness in proximal muscles with fever, malaise and weight loss. NO muscle weakness.
What is polymyalgia rheumatica associated with?
giant cell arteritis
Findings in polymyalgia rheumatica?
increased ESR, Increased CRP, normal CK
Treatment for polymyalgia rheumatica?
corticosteroids (rapid response)
Fibramyolgia presentation
Chronic widespread musculoskeletal pain associated wiht tender points, stiffness, paresthesias, poor sleep, cognitive disturance. More common in women 20-50 years old.
Treatment of fibromyalgia
Regular exercise, antidepressants, neuropathic pain agents
Antibodies found in polymyositis/dermatomyositis?
+ANA, anti-Jo-1, anti-SRP, anti-Mi-2
polymyositis presentation
progressive symmetric proximal muscle weakness.
Usually involves shoulders.
pathophys of polymyositis
Endomysial inflammation with CD8+ T cells.
dermatomyositis presentation
Similar to polymyositis plus Gottron papules, photodistributed facial erythema, shawl and face rash., darkening and thickening of fingers tips.
dermatomyositis pathophys
perimysial inflammation and atrophy with CD4+ cells
pathophys of myasthenia gravis
autoantibodies to post-synaptic Ach receptors
presentation of myasthenia gravis
Ptosis, diplopia, weakness (may lead to dyspnea if respiratory muscles involved, or dysphagia if bublar muscle involvement).
Worsen with muscle use.
What is myasthenia gravis associated wtih?
thymoma, thymic hyperplasia
Treatment of myasthenia gravis?
AchE inhibitors - pyridostigmine
Tensilon test use
Determines whether a patient presenting with worsening symptoms of myasthenia gravis has an insufficient dose or is having a cholinergic crisis.
Tensilon test findings
Administer edrophonium (a short acting AchE inhibitor). If symptoms improve, patient needs higher dose. If symptoms gett worse, patient needs lower dose.
Pathophys of lambert-eaton syndrome
autoantibodies to presynaptic Ca channels – decreased Ach release
Presentation of lambert eaton syndrome
proximal muscle weakness, autonomic symptoms (dry mouth, impotence). Improves with muscle use.
what is lambert-eastion syndrome associated with?
small cell lung cancer
Treatment of Raynaud phenomonen ?
Ca channel blockers
Scleroderma pathophys
Autoimmunity activates fibroblasts –> collagen deposition
Diffuse scleroderma presentation
widespread skin involvment, rapid progression, early visceral involvment.
Antibodies seen in diffuse scleroderma?
anti-scl-70
anti-RNA polymerase
someone with anti-RNA polyermase is at increased risk for what?
renal crisis
Limited scleroderma pathophys
skin involvement confined to fingers and face. Associated with CREST syndrome.
What is CREST syndrome?
Calcinosis cutis, anti-centromere antibody, raynaud phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia.
Which antibody may be seen in limited scleroderma?
Anti-centromere antibody
