SM_235a: Crystalline Diseases Flashcards
All crystalline arthropathies are characterized by _____ and _____
All crystalline arthropathies are characterized by inflammation in the joint and crystals in synovial fluid

Describe inflammatory synovial fluid analysis
Inflammatory synovial fluid analysis
- Slightly turbid appearance
- 2,000-50,000 WBCs/mm^3
- 20-70% PMNs

____, ____, and ____ are the main crystal types
Basic calcium phosphate (BCP), calcium pryophosphate dihydrate (CPPD), and monosodium urate (gout) are the main crystal types
Basic calcium phosphate crystal are also known as _____
Basic calcium phosphate crystal are also known as calcium hydroxyapatite
Basic calcium phosphate (BCP) crystals deposit in _____, _____, and _____
Basic calcium phosphate crystals (BCP) deposit in soft tissue (acute calcific periarthritis), joints (BCP arthropathy), and tendons (calcific tendinitis)
- Soft tissue (acute calcific periarthritis): 1st MTP in young women (pseudo-podagra)
- Joints (BCP arthropathy): Milwaukee shoulder syndrome
- Tendons (calcific tendinitis): shoulder, most commonly in supraspinatus tendon
Acute calcific periarthritis occurs when BCP crystals are shed from ____ and deposit in ____
Acute calcific periarthritis occurs when BCP crystals are shed from calcific deposit and deposit in soft tissues
- BCP crystals shed from calcific deposit of BCP arthropathy or calcific tendinitis
- Intense local inflammation

BCP causes acute calcific periarthritis of ____ in young women (pseudo-podagra)
BCP causes acute calcific periarthritis of 1st MTP in young women (pseudo-podagra)

BCP deposit in ____ causes ____, which is severe degenerative arthritis of the shoulder joint
BCP deposit in joints causes Milwaukee Shoulder Syndrome, which is severe degenerative arthritis of the shoulder joint
- Loss of rotator cuff, cannot abduct arm, huge swelling
- BCP crystals identified
- Woman > 70s
- Bilateral involvement common
- Can affect other joints such as finger (Philadelphia finger)

BCP deposit in joints of the finger causes _____
BCP deposit in joints of the finger causes Philadelphia finger

BCP deposits in tendons causes _____, most commonly in tendons of the _____
BCP deposits in tendons causes calcific tendinitis, most commonly in tendons of the shoulder
- Usually in muscles that abduct and stabilize the shoulder
- Bursitis, impingement syndrome
- Can also involve hand, wrist, hip, knee, foot, neck

BCP appearance on microscopy includes _____, _____, and _____
BCP appearance on microscopy includes shiny coins on ordinary light microscopy, NOT birefringent on polarized light microscopy, and joint aspirate stained for alizarin red

BCP is _____ on polarized light microscopy
BCP is NOT birefringent on polarized light microscopy

EM of BCP crystals shows _____ in synovial fluid demonstrating _____
EM of BCP crystals shows rice bodies in synovial fluid demonstrating apatite crystals

Synovial fluid in BCP crystals is _____
Synovial fluid in BCP crystals is non-inflammatory
(like OA)

BCP arthropathy is treated if the patient is _____ and includes _____, _____, _____, and _____
BCP arthropathy is treated if the patient is asymptomatic and includes NSAIDs, PT, intra-articular steroids, and surgery if indicated
Describe the ABCs of BCP arthropathy
ABCs of BCP arthropathy
- A: acute calcific periarthritis, alizarin red stain, atypical gout (young woman)
- B: BCP arthropathy, NOT birefringent
- C: calcific tendinitis, cuff (supraspinatus), coins shiny on light microscopy

Describe calcium pyrophosphate dihydrate (CPPD) arthropathy
CPPD arthropathy (formerly known as pseudogout)
- Calcium pyrophosphate deposition in joints
- Major risk factor is aging: peaks 65-75 years
- Female to male is 2-7:1
- Predilecton for joints affected by osteoarthritis
- Associated with numerous metabolic disturbances: screen if onset of disease < 55 years

Check ____, ____, and ____ levels is suspecting CPPD
Check Mg, iron/ferritin, and TSH levels is suspecting CPPD

Describe the clinical presentations of CPPD
CPPD clinical presentations
- Pseudogout (gout mimic): acute monoarthritis (knees > wrist > shoulder > ankle and elbow)
- Chronic polyarthritis (RA mimic): symmetric, small joints
- Progressive osteoarthritis: involves large and small joints, crowned dens syndrome (spinal involvement)
- Asymptomatic chondrocalcinosis

CPPD mimics ____ by causing ____
CPPD mimics gout by causing acute monoarthritis
(knees > wrist > shoulder > ankle and elbow)

CPPD mimics ____ by causing ____
CPPD mimics RA by causing chronic polyarthritis
(symmetric, small joints)

CPPD with spinal involvement causes _____
CPPD with spinal involvement causes crowned dens syndrome

Describe radiographic manifestation of CPPD
CPPD radiographic manifestations
- Can have chondrocalcinosis without pseudogout and vice versa
- Meniscus and triangular fibrocartilage of wrist often show classic findings

CPPD crystals are ____ and ____ on polarized light microscopy
CPPD crystals are pleomorphic and weakly positive birefringent on polarized light microscopy
(look rhomboidal, weak tint)

Describe CPPD treatment
CPPD treatment
- NSAIDs
- Intra-articular or oral steroids
- Colchicine: use caution given elderly population
- Anakinra: not FDA approved
- Weak data on hydroxychloroquine and methotrexate
- No true prophylaxis and treat acute gout attach

____ and ____ cause non-gout arthropathies
BCP and CPPD crystals cause non-gout arthropathies
- BCP: soft tissue (acute calcific periarthritis) - 1st MTP in young women (pseudo-podagra), joints (BCP arthropathy) - Milwaukee Shoulder Syndrome, tendons (calcific tendinits) - shoulder most commonly supraspinatus tendon
- CPPD: spectrum of presentations, Crowned Dens syndrome, primary (pseudogout, RA, OA, Charcot, asymptomatic, etc), secondary (check PTH, Ca, P, Mg, TSH levels)

Describe gout
Gout
- Increased prevalence with increased age and increased uric acid
- [uric acid] rises decades before development of goat
- Males: onset of puberty -> disease onset > 30 yo
- Females: perimenopausal phase -> disease onset 60 yo (estrogen is uricosuric)
- Male predominant disease until menopause after which rates equalize
- 50% have gout if UA > 10 mg/dl
- Positive FMHx in 50%

Describe risk factors for gout
Gout risk factors
- Alcohol consumption especially beer
- Obesity, metabolic syndrome, insulin resistance
- Diet rich in purines: meat, shellfish, ethanol, soft drinks, high fructose syrup
- Drugs: thiazide, cyclosporine, low dose ASA (<1 g / day)
- Renal insufficiency
- Organ transplantation
- Inborn errors of metabolism: Lesch-Nyhan, PRPP synthetase
Amount of alcohol consumed directly correlates with goat risk because _____ while _____
Amount of alcohol consumed directly correlates with goat risk because increased ATP degradation leads to increase urate synthesis while increased lactic acid leads to decreased urate excretion
(beer has highest purine content)
Hyperuricemia is primarily caused by _____
Hyperuricemia is primarily caused by underexcretion
(decreased renal function, drugs, alcohol use)

Describe the stages of gout
Gout
- Asymptomatic hyperuricemia: 15% will develop gout after 20 years of hyperuricemia
- Acute gouty arthritis: 85-90% in a single joint
- Intercritical gout (quiescent intervals between gout attachs): 60% have another attack within 24 months, 5-10% never have another attack
- Chronic tophaceous gout: after 10 years of uncontrolled disease

Describe the acute clinical presentation of gout
Gout acute clinical presentation
- Acute monoarthritis: usually involving 1st MTP (podagra)
- Pain often begins very early in morning: very acute
- First few attacks are often self-limited
- Subsequent attacks may be more frequent, severe, and resistant to therapy
- If hyperuricemia persists, the disease can become chronic and polyarticular

Chronic tophaceous gout involves deposits in the ____ and _____ in addition to the toe
Chronic tophaceous gout involves deposits in the pinna and elbow in addition to the toe

Gout is _____ shaped and _____ when parallel to a polarizer
Gout is needle shaped and yellow when parallel to a polarizer
(blue when perpendicular to polarizer, treat with allopurinol)

Describe radiographic manifestations of gout
Gout radiographic manifestations
- Large erosions
- Punched-out lesions
- Soft tissue tophi
- Sclerotic overhanging edges
- Preserved joint spaces

Therapeutic goals for gout are ____, ____, and ____
Therapeutic goals for gout are providing rapid and safe pain relief, preventing further attacks, and preventing formation of tophic and destructive arthritis
First line therapy for gout includes ____, ____, and ____
First line therapy for gout includes NSAIDs, colchicine, and corticosteroids

Indications for chronic therapy for gout are ____, ____, ____, and ____
Indications for chronic therapy for gout are > 2-3 gout attacks for 1-2 years, renal stones, tophaceous gout, and erosions on X-ray
Describe chronic management of gout
Gout chronic management
- Xanthine oxidase inhibitors (decrease production of urate): allopurinol (risk for hypersensitivity reactions), febuxostat
- Uricosuric agents (increase excretion of urate): probenecid (risk for kidney stones), lesinurad
- Pegloticase: uricase IV, very potent urate lowering agent, immediate hypersensitivity reactions common
- Anakinra (anti-IL1 biologic therapy)

Primary chronic management of gout is _____
Primary chronic management of gout is xanthine oxidase inhibitors (allopurinol, febuxostat)
(allopurinol has risk of hypersensitivity reactions)

Summarize BCP, CCPD, and gout crystals
BCP, CCPD, and gout crystals
