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FA complete review part 2 Pathology II

What is the pathogenesis of Osteoarthritis? Mechanical- wear and tear destroys articular cartilage leading to inflammation with inadequate repair
What cell mediate the pathogenesis of Osteoarthritis? Chondrocytes
What is the role of Chondrocytes in Osteoarthritis pathogenesis? Mediate degradation an inadequate repair of the articular cartilage
Is the pathogenesis of Osteoarthritis, mechanical or autoimmune? Mechanical
What are common predisposing factors of Osteoarthritis? Age, female, obesity, and joint trauma
What is the clinical presentation of Osteoarthritis? 1. Pain in weight-bearing joints after use, improving with rest 2. Asymmetric joint involvement 3. Knee cartilage loss beings mediall 4. NO SYSTEMIC symptoms
Which condition, rheumatoid arthritis or osteoarthritis, presents with "bowlegged" deformity? Osteoarthritis
Joint involvement in osteoarthritis, is symmetric or asymmetric? Asymmetric
True or False. Osteoarthritis present with systemic symptoms. FALSE. Osteoarthritis has NO systemic symptoms
What are the joint finding of osteoarthritis? 1. Osteophytes, 2. Joint space narrowing, 3. Subchondral sclerosis ans cysts 4. Synovial fluid noninflammatory 5. Involves DIP, PIP, and 1st CMC
Is the MCP involved in Osteoarthritis? No
Which joint involvement in osteoarthritis produces the Heberden nodes? DIP
The "nodes" of joint deformities are involved in Osteoarthritis or Rheumatoid arthritis? Osteoarthritis
Which are joint deformities caused by DIP and PIP in osteoarthritis? Heberden nodes (DIP) and Bouchard nodes (PIP)
Besides the PIP and DIP, which other joint is involved in Osteoarthritis? 1st CMC
It the arthritic condition does NOT involve the MCP, it most likely is, osteoarthritis or RA? Osteoarthritis
List of treatment options for Osteoarthritis: Acetaminophen, NSAIDs, intra-articular glucocorticoids
What is the pathogenesis of Rheumatoid arthritis? Autoimmune- inflammation induces formation of pannus which erodes articular cartilage and bone
What is Pannus? Proliferative granulation tissue
What common articular condition is associated with pannus? Rheumatoid arthritis
What are risk factors of Rheumatoid arthritis? Female, HLA-DR4, smoking, (+) rheumatoid factor, anti-cyclic citrullinated peptide antibody
What is most specific antibody for Rheumatoid arthritis? Anti-cyclic citrullinated peptide antibody
What is the clinical presentation of Rheumatoid arthritis? Pain, swelling, and morning stiffness lasting > 1 hour, improving with use. Symmetric joint involvement and, Systemic symptoms such as fever, weight loss, and fatigue Extraarticular manifestations are common
Which RA or Osteoarthritis presents with symmetrical joint involvement? Rheumatoid arthritis
Which, RA or osteoarthritis, is presented with systemic symptoms? Rheumatoid arthritis
How is the pain in joint in RA in the morning? Morning stiffness lasting > 1 hour, but improves with use
What are the extra-articular manifestation presented in RA? 1. Rheumatoid nodules in subcutaneous tissue and lung 2. Interstitial lung disease 3. Pleuritis 4. Pericarditis 5. Anemia of chronic disease 6 .Neutropenia + splenomegaly 7. AA amyloidosis 8. Sjogren syndrome 9. Scleritis 10. Carpal tunnel syndrome
What are some joint findings in Rheumatoid arthritis? Erosions, juxta-articular osteopenia, soft tissue swelling, subchondral cysts, joint space narrowing
What are the classical joint deformities in RA? 1. Cervical subluxation 2. Ulnar finger deviation 3. Swan neck, 4 Boutonniere
Which joints are involved in Rheumatoid arthritis? MCP, PIP, and wrist
Which joint is involved in RA but not in Osteoarthritis? DIP or 1st CMC
Articular condition with synovial fluid inflammatory Rheumatoid arthritis
What are the lung conditions associated with Rheumatoid arthritis? Pneumoconiosis leading to Caplan syndrome
What are the treatment options for Rheumatoid arthritis? NSAIDs, glucocorticoids, disease-modifying agents, and biologic agents.
What are common Disease-Modifying agents used in treatment of Rheumatoid arthritis? Methotrexate, sulfasalazine, hydroxychloroquine, and Leflunomide.
What is Gout? Acute inflammatory monoarthritis caused by precipitation of monosodium urate crystal in joints.
What risk factors associated with developing of Gout? Male sex, hypertension, obesity, diabetes, and dyslipidemia
What is the strongest risk factor for Gout? Hyperuricemia
Acute inflammatory monoarthritis caused by precipitation of monosodium urate crystals in joints. Dx? Gout
What type of crystals are seen in joints of Gout patients? Monosodium urate crystals
What are the two causes of hyperuricemia? 1. Underexcretion of uric acid 2. Overproduction of uric acid
Which cause of hyperuricemia, underexcretion or overproduction of uric acid, is most common in causation? Underexcretion of uric acid
What are some condition that lead to the underexcretion of uric acid leading to Gout? Idiopathic but potentiated by renal failure
What type of diuretic are often associated with precipitating gout by underexcretion of uric acid? Thiazide diuretics
What conditions cause the overproduction of uric acid leading to Gout? Lesch-Nyhan syndrome, PRPP excess, increase cell turnover, and von Gierke disease
Description of Gout crystals Needle shaped and (-) birefringence under polarized light
Crystal are needle-shaped and with a negative birefringent. Dx? Gout
How are the crystals of Gout under polarized light? Yellow under parallel light, and blue under perpendicular light
Needle-shaped crystal (-) birefringence under polarized light Gout
How is the birefringent of Gout crystals? Negative
What is the shape of crystal in gout? Needle-shaped
What is a common condition that causes a elevated cell turnover? Tumor lysis syndrome
What type or hyperuricemia is produced by Tumor lysis syndrome, that later develops into Gout? Overproduction of uric acid
Is the joint affection in Gout, asymmetric or symmetric? Asymmetric
Clinical description of affected joint in Gout? Joint is swollen, red, and painful
What is the classical manifestation of Gout? Painful MTP joint of big toe (podagra)
What is Tophus formation associated with? Gout
Where in Gout is Tophus formation visible? External ear, olecranon bursa, or Achilles tendon
What are events or actions that precipitate Gout? 1. After large meal with foods rich in purines (red meat, seafood), 2. Trauma 3. Dehydration 4. Diuresis 5. Alcohol consumption
How does alcohol consumption precipitate acute Gout attacks? Alcohol metabolites compete for same excretion sites in kidney as uric acid lead to decrease uric acid secretion and subsequent buildup in blood.
What are medications are used for acute flare of gout? NSAIDs, glucocorticoids and colchicine
What are the chronic (preventive) medications of Gout? Xanthine oxidase inhibitors
What are tow commonly used xanthine oxidase inhibitors used in gout? Allopurinol and Febuxostat
MOA of Allopurinol and Febuxostat Xanthine oxidase inhibitors
What was the old nomenclature for Calcium pyrophosphate deposition disease? Pseudogout
What is the pathogenesis of Pseudogout? Deposition of calcium pyrophosphate crystals within the joint space.
What are some associations causative of Calcium Pyrophosphate deposition disease (Pseudogout)? Hemochromatosis, hyperparathyroidism, and joint trauma
What is the most commonly involved joint in Pseudogout? Knee
Pain and swelling with acute inflammation and/or chronic degeneration (pseudo-osteoarthritis). Clinical presentation of Pseudogout
What is the key feature of x-ray of Calcium Pyrophosphate deposition disease? Chondrocalcinosis
What is chondrocalcinosis? Cartilage calcification
How are the crystals in Calcium Pyrophosphate deposition disease? Rhomboid and weakly (+) birefringent under polarized light
What is the treatment for Pseudogout? NSAIDs, colchicine, and glucocorticoids
What medication is often used as prophylaxis for Pseudogout? Colchicine
Rhomboid calcium crystal in the knee joint. Dx? Pseudogout
Birefringent of crystal in Pseudogout Weakly (+) birefringent
What is Systemic juvenile idiopathic arthritis? Childhood arthritis seen in < 12 year olds.
What is the clinical presentation Systemic Juvenile Idiopathic arthritis? 1. Daily spiking fevers 2. Salmon-pink macular rash 3. Uveítis 4. Arthritis (2+ joints)
What are accompanying lab results in Systemic Juvenile idiopathic arthritis? Leukocytosis, Thrombocytosis, Anemia Increased ESR, Increased CRP
What are the treatment options of Juvenile arthritis? NSAIDs, steroids, methotrexate, TNF inhibitors
What is the key cutaneous (skin) feature of Juvenile Arthritis? Salmon-pink macular rash
A 9 year old complains of joint pain, and PE is (+) for a salmon-pink macular rash. Dx? Systemic Juvenile Idiopathic Arthritis
What is Sjogren syndrome? Autoimmune disorder characterized by destruction of exocrine glands by lymphocytic infiltrates.
Which exocrine glands are commonly destroyed n Sjogren's syndrome? Lacrimal and salivary glands
Autoimmune disorder that is featured by the destruction of lacrimal and salivary glands by lymphocytic infiltrates, most commonly in women. Dx? Sjogren syndrome
What is the epidemiology of Sjogren's syndrome? Women of 40-60 years old
What are the findings in Sjogren syndrome? 1. Inflammatory joint pain 2. Keratoconjunctivitis sicca 3. Xerostomia 4. Presence of several antibodies
What is Keratoconjunctivitis sicca? Decreased tear production and subsequent corneal damage
What is Xerostomía? Decreased saliva production
What autoimmune disorder is associated with Xerostomia? Sjogren syndrome
What occular (eye) condition is associated with Sjogren syndrome? Keratoconjunctivitis sicca
What are the antibodies associated with Sjogren syndrome? - Antinuclear antibodies - Rheumatoid factor - Anti-ribonucleoprotein antibodies - Bilateral parotid enlargement
What are the anti-ribonucleoprotein antibodies associated with Sjogren syndrome? SS-A (anti-Ro) and/or SS-B (anti-La)
Other than Sjogren syndrome, which other condition has (+) anti-SSA and anti-SSB? SLE
What is the risk of (+) anti-SSA in pregnant women with SLE? Congenital heart block in the newborn
What is a common primary or secondary syndrome assocaited with other automimmune disorders such as RA, SLE, and systemic sclerosis? Sjogren syndrome
What are common complications of Sjogren syndrome? Dental caries, mucosoa-associated lumphoid tissue (MALT) lymphoma, .
What is the key finding of Sjogren syndrome in a biopsy? Focal lymphocytic sialadenitis on labial salivary gland
Focal lymphocytic sialadenitis on labial salivary gland biopsy. Dx? Sjogren syndrome
What are the common pathogens that cause Septic arthritis? S. aureus, Streptococcus, and Neisseria gonorrhoeae.
How is the synovial fluid in Septic arthritis? Purulent with WBC > 50, 000/mm3
What is the common triad of Gonococcal arthritis? Polyarthralgia, tenosynovitis (hand), and dermatitis.
STI that presents as either purulent arthritis (knee) with a triad of polyarthritis, tenosynovitis, and dermatitis. Dx? Gonococcal arthritis
Which part is Gonococcal arthritis tenosynovitis present? Hand
What type of arthritic conditions are without rheumatoid factor? Seronegative Spondyloarthtirtis
What is the MHC class serotype is associated Seronegative spondyloarthritis? MHC class I
List of Seronegative Spondyloarthritis: 1. Psoriatic arthritis 2. Ankylosing spondylitis 3. Inflammatory bowel disease 4. Reactive arthritis
What are general clinical signs of Seronegative spondyloarthritis? 1. Inflammatory back pain 2. Peripheral arthritis 3. Enthesitis 4. Dactylitis 5. Uveitis
What is Enthesitis? Inflamed insertion sites of tendons
What is a common way to refer to Dactylitis? "sausage fingers"
What is the HLA association with all Seronegative spondyloarthritis? HLA-B27
Description of inflammatory back pain associated with HLA-B27 arthritic conditions Morning stiffness, that improves with exercise
What are common associations of Psoriatic arthritis? Skin psoriasis and nail lesions
What are clinical signs of Psoriatic arthritis? - Asymmetric and patchy involvement. - Dactylitis and "pencil-in-cup" deformity of DIP on x-ray
What is a classic finding of DIP x-ray in Psoriatic arthritis? "pencil-in-cup" deformity of DIP
"Pencil-in-cup" deformity of DIP on x-ray. Dx? Psoriatic arthrtitis
Which arthritic condition is characterized by symmetric involvement of spine and sacroiliac joints? Ankylosing spondylitis
What are the results of the joint inflammation in Ankylosing spondylitis? Ankylosis (joint fusion), uveitis, and aortic regurgitation
Which heart condition is associated with Ankylosing spondylitis? Aortic regurgitation
Bamboo spine. Dx? Ankylosing spondylitis
What is a possible respiratory complication in Ankylosing spondylitis? Costovertebral and costosternal ankylosis may cause restrictive lung disease
What is the cause of a respiratory lung disease in Ankylosing spondylitis? Costovertebral and costosternal ankylosis
Which inflammatory bowel diseases are associated with Spondyloarthritis? Crohn disease and Ulcerative colitis
What was the former name of Reactive arthritis? Reiter syndrome
What is the classic triad of Reactive arthritis? 1. Conjunctivitis 2. Urethritis 3. Arthritis
A person complains of conjunctivitis, inability to urinate, and peripheral arthritis. Dx? Reactive arthritis
What are the pathogens associated with causing Reactive arthritis? Shigella, Yersinia, Chlamydia, Campylobacter, and Salmonella
Systemic Lupus Erythematosus (SLE) is a : Systemic, remitting, and relapsing autoimmune disease
What causes organ damage in SLE? Primarily due to a type III hypersensitivity reaction and, to a lesser degree, a type II hypersensitivity reaction.
What is the cause of a decrease in clearance of immune complexes in SLE? Deficiency of early complement proteins (C1q, C4, and C2)
What is the classic clinical presentation of SLE? Rash, joint pain, and fever in a female of reproductive age
Which ethnicities are more common to develop SLE? African-American and Hispanic descent
Which are the early complement proteins associated with the pathogenesis of SLE? C1q, C4 and, C2
What is Libman-Sacks Endocarditis? Nonbacterial, verrucous thrombi usually on mitral or aortic valve and can be present on either surface of the valve
What subtype of endocarditis is associated with SLE? Libman-Sacks Endocarditis
SLE endocarditis (name) Libman-Sacks Endocarditis
Glomerular deposition of DNA-anti-DNA immune complexes. Lupus nephritis
What is Lupus nephritis cause? Glomerular deposition of DNA-anti-DNA immune complexes
What is the common and severe type of Lupus nephritis? Diffuse proliferative
What are the common causes of death in SLE? 1. Renal disease 2. Infections 3. Cardiovascular disease
What is the MCC of death in SLE? Renal disease
In a anti-SSA (+) pregnant woman ---> Increased risk of newborn developing neonatal lupus
What are the clinical features of Neonatal lupus? Congenital heart block, Periorbital/diffuse rash, Transaminitis and, Cytopenias
What is the common mnemonic used to summarize SLE? RASH OR PAIN
RASH OR PAIN: Rash (malar or discoid) Arthritis (nonerosive) Serositis Hematologic disorders Oral/nasopharyngeal ulcers Renal disease Photosensitivity Antinuclear antibodies Immunologic disorder Neurological disorders
What is the type of rash seen in SLE? Malar or discoid rash
How is the arthritis in SLE described as? Nonerosive
What are the anti-antibodies associated with SLE immunologic disorders? anti-dsDNA, anti-Sm, and antiphospholipid
Mixed connective tissue disease has features of: SLE, Systemic sclerosis, and/or polymyositis
What is the associated antibody of Mixed CT disease? anti-U1 RNP antibodies
(+) anti-U1 RNP antibodies. Dx? Mixed connective tissue disease
Antiphospholipid syndrome is: A primary or secondary disorder, most commonly in SLE
What is the criteria for diagnosis of Antiphospholipid syndrome? 1. Hx of thrombosis 2. Hx of Spontaneous abortion 3. (+) antibodies: lupus anticoagulant, anticardiolipin, and anti-B2 glycoprotein I antibodies
What is the treatment for antiphospholipid syndrome? Systemic anticoagulation
Anticardiolipin antibodies can cause: False-positive VDRL/RPR
What can produce false-positive VDRL/RPR? Anticardiolipin antibodies
What is a adverse effect of Lupus anticoagulant? Prolonged PTT that cannot be corrected by the addition of normal platelet-free plasma.
What condition is closely associated with Polymyalgia rheumatica? Giant cell (temporal) arteritis
What are the symptoms of Polymyalgia rheumatica? Pain and stiffness in proximal muscles, often with fever, malaise, and weight loss.
What is the population most commonly affected by Polymyalgia rheumatica? Women > 50 years old
A person with Giant cell (temporal) arteritis, is often also found with what muscle condition? Polymyalgia rheumatica
What are the lab findings of Polymyalgia rheumatica? Elevated ESR and CRP, and normal CK
Lab findings: - Increased ESR - Increased CRP - Normal CK Dx? Polymyalgia rheumatica
The treatment of Polymyalgia rheumatica has a -----> Rapid response to low-dose corticosteroids
What is Fibromyalgia? Chronic, widespread musculoskeletal pain associated with "tender points," stiffness, paraesthesias, poor sleep, fatigue, cognitive disturbance.
What is the etiology of Fibromyalgia? Women 20-50 years old
What is the treatment for Fibromyalgia? Regular exercise, antidepressants, and neuropathic pain agents
What neuropathic pain agent is often use in Fibromyalgia treatment? Gabapentin
What type of antidepressants are used in the treatment of Fibromyalgia? TCAs and SNRIs
What are the non-specific lab findings of Polymyositis/Dermatomyositis? (+) ANA and elevated CK
What are the SPECIFIC lab findings of Polymyositis/ Dermatomyositis? 1. (+) anti-Jo-1 (histidyl-tRNA synthetase) 2. (+) anti-SRP (signal recognition particle), 3. (+) anti-Mi-2 (helicase)
Anti-Jo-1 antibody = Histidyl-tRNA synthetase
What is Polymyositis? Progressive symmetric proximal muscle weakness, characterized by endomysial inflammation with CD8+ T cells
Endomysial or Perimysial inflammation is seen with Polymyositis? Endomysial inflammation
Endomysial or Perimysial inflammation is seen with Dermatomyositis? Perimysial inflammation
What are the distinguishing features of dermatomyositis, that separate it from Polymyositis? Gottron papules, photo disturbed facial erythema, "shawl and face" rash, darkening and thickening of fingertips and sides resulting in irregular, "dirty"-appearing marks
Polymyositis + cutaneous involvement. Dx? Dermatomyositis
Perimysial inflammation and atrophy with CD4+ T cells. Dermatomyositis
What CD + T cells are involved in Dermatomyositis? CD4+ T cells
Which CD+ T cells are involved in Polymyositis? CD8+ T cells
What is the facial rash/erythema seen with Dermatomyositis? Heliotrope (violaceus) edema of the eyelids
What part of the body is most often involved in Polymyositis? Shoulders
Helicase antibody = anti-Mi-2 antibody
What are the two most common Neuromuscular junction diseases? Myasthenia gravis and Lambert-Eaton myasthenic syndrome
What is the most common NMJ disorder? Myasthenia gravis
What is the pathophysiology of Myasthenia gravis? Autoantibodies to postsynaptic ACh receptor
What condition is due to autoantibodies to postsynaptic ACh receptor? Myasthenia gravis
What are the clinical fatigable muscle weakness features of Myasthenia gravis? Ptosis; Diplopia; Proximal weakness; Respiratory muscle involvement --> dyspnea Bulbar muscle involvement --> dysphagia, difficulty chewing
What is the result of bulbar muscle involvement in Myasthenia gravis? Dysphagia and difficulty chewing
Myasthenia gravis or Lambert-Eaton myasthenic gravis syndrome, worsens with muscle use? Myasthenia gravis
Myasthenia gravis or Lambert-Eaton myasthenic gravis syndrome, improves with muscle use? Lambert-Eaton myasthenic syndrome
What associated conditions with Myasthenia gravis? Thymoma and Thymic hyperplasia
AChE inhibitor administration in Myasthenia gravis? Reverses symptoms
What is a common medication used in Myasthenia gravis to reverse symptoms? Pyridostigmine
Pyridostigmine is used to reverse ________________________ symptoms. Myasthenia gravis
What is the pathophysiology of Lambert-Eaton myasthenic syndrome? Antibodies to presynaptic Ca2+ channel leading to a decrease in ACh release
Myasthenia gravis involves pre- or postsynaptic receptors in NMJ? Postsynaptic ACh receptors
Lambert-Eaton myasthenic syndrome, involves pre- or postsynaptic receptors? Presynaptic Ca2+ channels
The antibodies in LEMS that attach the presynaptic Ca2+ channels cause ---> Decrease in ACh release
What are clinical characteristics of LEMS? Proximal muscle weakness, autonomic symptoms (dry mouth, constipation, and impotence) Hyporeflexia Improves with muscle use
What condition is associated with Lambert-Eaton myasthenic syndrome (LEMS)? Small cell lung cancer
Which NMJ condition is often associated with Small cell lung cancer? Lambert-Eaton myasthenic syndrome
Which NMJ condition is associated with minimal effect when treated with AChE inhibitors? Lambert-Eaton myasthenic syndrome
What is the color change seen in Raynaud phenomenon? From white to blue to red
What does the color white in Raynaud phenomenon represent? Ischemia
What causes the change in color into blue form white in Raynaud phenomenon? Hypoxia
What does the change from blue to red in Raynaud phenomenon? Reperfusion
What is the cause of Raynaud phenomenon? Decrease blood flow to skin due to arteriolar (small vessel) vasospasm in response to cold or stress
What areas of the body are most often seen with Raynaud phenomenon? Fingers and toes
When is Raynaud phenomenon called Raynaud disease? Only when it is idiopathic
When does Raynaud syndrome occur? Secondary to a disease process such as mixed connective tissue disease, SLE, or CREST syndrome.
Digital ulceration is seen in Raynaud phenomenon, disease, or syndrome? In secondary Raynaud's syndrome
What is a key feature of Raynaud syndrome? Digital ulceration
What is the common treatment of Raynaud phenomenon? Ca2+ channel blockers
What is another form to refer to Scleroderma? Systemic sclerosis
What is the triad that defines Scleroderma? 1. Autoimmunity 2. Non-inflammatory vasculopathy 3. Collagen deposition with fibrosis
What common features of Systemic sclerosis? Sclerosis of skin manifested as puffy, taut skin without wrinkles, fingertip pitting.
Which other organ systems, besides skin, are often associated or involved in Scleroderma? Renal, pulmonary, gastrointestinal, and cardiovascular systems.
What is the feature of renal involvement in Scleroderma? Renal crisis treated with ACE inhibitors
What are pulmonary conditions seen with Scleroderma? Interstitial fibrosis and Pulmonary HTN
What GI conditions are associated with Scleroderma? Esophageal dysmotility and reflux
What are the 2 major types of Scleroderma? 1. Diffuse scleroderma 2. Limited scleroderma
What is Diffuse scleroderma? Widespread skin involvement, rapid progression, ealy visceral involvement.
What is the antibody associated with Diffuse Scleroderma? anti-Scl-70 antibody
What is another name for anti-Scl-70 antibody? anti-DNA topoisomerase I antibody
(+) anti-DNA topoisomerase I antibody. Dx? Diffuse scleroderma
The extend of Limited Scleroderma involves which areas? LImited skin involvement confined to fingers and face
What syndrome is associated with Limited Scleroderma? CREST syndrome
What are the characteristics of CREST syndrome? Calcinosis cutis anti-Centromere antibody Raynaud phenomenon Esophageal dysmotility Sclerodactyly Telangiectasia
What is the antibody seen with CREST syndrome? anti-Centromere antibody
Created by: rakomi
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