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Multisystem Path > Other Autoimmune Diseases 2 > Flashcards

Other Autoimmune Diseases 2 Flashcards

1
Q

Describe the pathophysiology of Amyloidosis.

A

Body is unhappy due to misfolded proteins that aggregate and form insoluble fibers known as amyloid. Amyloid develops due to:

1) Too much protein production
2) Mutated proteins that misfold/can’t be broken down
3) incomplete protein breakdown

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2
Q

How do you diagnose amyloidosis?

A

Rectal tissue biopsy (easy access)

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3
Q

What do you see on histology for amyloidosis (SUPER HIGH YIELD)

A

B-pleated sheets that show apple green birefringence under polarized light.

Stain: congo red

NEVER FORGET

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4
Q

What is a commonly affected organ in amyloidosis and how does this organ look after it gets all amyloided up

A

Heart

Myocardial hypertrophy leads to ECG changes, arrhythmias, and a waxy looking endocardium.

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5
Q

What is localized amyloidosis and what is an example?

A

Amyloid goes to a single organ to produce a mass (or only be seen on microscopic exam)

Example:

1) Endocrine (medullary carcinoma of the thyroid a/w microscopic amyloid)
2) Alzheimer (brain)

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6
Q

There are 3 types of systemic amyloidosis. Name them

A

1) Primary
2) Secondary
3) Hemodialysis associated

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7
Q

What is primary amyloidosis all about? What is the major fibril found here?

A

Clonal proliferation of plasma cells that make Ig that is prone to misfold and form amyloid

Major fibril: AL (amyloid light chain)

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8
Q

What might you see on labs and histology for primary amyloidosis

A

Serum Electrophoresis: M spike (monoclonal Ig production)

Histo: Bence Jones proteins, Rouleaux formation

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9
Q

What conditions are associated with primary amyloidosis?

A

1) Multiple Myeloma
2) B-cell neoplasm
3) Waldenström macroglobulinemia

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10
Q

Describe the pathophysiology of secondary amyloidosis?

A

1) Chronic inflammation
2) Activated macrophages secrete IL-1/IL-6 which stimulates liver to make SAA protein
3) Lots of SAA because of lots of inflammation
4) Add in a defect in protein break own and you get SAA accumulation and amyloid deposition

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11
Q

What is the major fibril you see in secondary amyloidosis?

A

Major fibril: AA (amyloid-associated)

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12
Q

What disease is commonly associated with secondary amyloidosis?

A

RA

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13
Q

What is Hemodialysis associated amyloidosis and what fibril is it associated with

A

Major fibril: AB2-microglobulin deposition

Path: this protein can’t be filtered through dialysis membranes in renal failure pts on long-term hemodialysis *New dialysis filters make this less common.

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14
Q

What is Familial Mediterranean Fever. What is the major fibril seen?

A

Excessive IL-1 production in response to inflammatory stimulus. Gene implicated in FMF codes for pyrin protein which is a/w proinflammatory response regulation

Major fibril: AA

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15
Q

Who is commonly affected by Familial Mediterranean Fever

A

Armenians, Sephardic Jews and Arabs

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16
Q

Systemic senile amyloidosis (aging). What’s this all about? What’s the major fibril seen?

A

People get old, and get lots of protein shit buildup. Most commonly involves the heart w/ cardiomyopathy and arrhythmias

Major fibril: ATTR (transthyretin amyloidosis)

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17
Q

SARCOIDOSIS. Other than having an AA female, how else will you know your patient has this?

A

Diagnosis of exclusion but also look for:

1) Noncaseating granulomas and enlarged hilar lymph nodes
2) Dyspnea, cough, chest discomfort
3) Lung Crackles
4) Skin lesions
5) Joint pain
6) Anorexia, weight loss
7) Arrhythmias (5%)

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18
Q

What lab findings will you see with sarcoidosis?

A

1) Increased Ca/vit D(active 1-A hydroxylase)
2) Increased ACE
3) Abnormal liver enzymes
4) Hypercalcemia
5) Hypercalciuria

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19
Q

What high yield stuff do you see on histology for sarcoidosis?

A

1) Fibrosis
2) Epithelioid histiocytes (multinucleated giant cell)
3) Star-shaped asteroid bodies
4) Schaumann bodies (laminated calcification)

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20
Q

What triad of sx do you see in Loefgren’s Syndrome (acute sarcoidosis) and what other sx might also come up?

A

Triad:

1) acute polyarthritis
2) erythema nodosum
3) hilar adenopathy

Other sx: malaise, fever, joint disease, uveitis, parotitis

This disease is self limited with rare relapse. Take some NSAIDs for it and you’ll be just fine.

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21
Q

Hypersensitivity Pneumonitis. What’s the idea here?

A

You inhale something, get granuloma formation and have sx of fever, cough, dyspnea. Chronic inhalation can lead to interstitial fibrosis.

You stop inhaling it, and sx go away. MAGIC.

22
Q

What are some fun examples of hypersensitivity pneumonitis?

A

1) Farmer’s lung (dusts from humid hay = rapid proliferation of the spores of thermophilic actinomycetes),
2) Pigeon breeder’s lung (from bird proteins),
3) Humidifier/AC lung (from thermophilic bacteria in heating reservoirs)

23
Q

What the pathology associated with Osteoarthritis?

A

Progressive degeneration of ARTICULAR CARTILAGE (not the synovium like RA!) in joints due to wear and tear

24
Q

What are the many sx associated with osteoarthritis

A

1) Joint pain
2) Limb buckling/instability
3) Limited ROM
4) Crepitus
5) Joint deformity
6) Morning stiffness that WORSENS THROUGH DAY (unlike RA)

25
Q

What lab abnormalities will you see with osteoarthritis?

A

NONE

ha tricked ya didn’t I. Nah, probs not cuz you so smaht

26
Q

Describe the joints typically involved in oastoarthrits?

A

1) Hips
2) Knees
3) MTP
4) Cervical/lumbar spine
5) CMC squaring
6) DIP (Heberden nodes) and PIP (Bouchard nodes)

27
Q

Joint mice are ________.

A

Bone fragments floating in joint space. Seen in osteoarthritis

28
Q

RA is a type of inflammatory arthritis. Another kind of inflammatory arthritis that is associated with a skin rash is _____________

A

Psoriatic Arthritis.

29
Q

What other sx are associated with Psoriatic Arthritis.

A
  1. SAUSAGE fingers and toes (dactylitis)
  2. Axial joint involvement
  3. Nail pits
  4. Arthritis mutilans (shortening of fingers by arthritis, sometimes so severe that hands look like paws)
30
Q

What X-ray findings are associated with psoriatic arthritis?

A
  1. *Pencil-in-cup deformities
  2. Soft tissue swelling
  3. Lack of osteopenia
  4. Decreased bone mineralization
31
Q

What 3 things classify the seronegative spondylarthropathies?

A
  1. Negative rheumatoid factor
  2. Axial skeleton involvement
  3. HLA-B27 association
32
Q

The 2 seronegative spondylarthropathies we will talk about are __________ and ____________.

A
  1. Ankylosing Spondylitis

2. Reactive Arthritis

33
Q

Ankylosing Spondylitis first. 1) What are the sx, 2) who is typically affected, and 3) what key finding will you see on X-ray.

A
  1. low back pain, uveitis
  2. Affects males, typically young adults
  3. X-ray finding: bamboo spine: fusion of vertebrae
34
Q

Now for reactive arthritis. What kinds of infections lead to this disorder and what symptoms will you see?

A
  1. Infections include GU (Chlamydia) or GI (shigella, samonella, yersinia, campy)
  2. Sx are constitutional, asymmetric polyarthritis in knee/large joints
35
Q

In infectious arthritis, what kind of organism is typically causing the infection? How does it get to the joint??

A

Bacteria usually spread hematogenously from nearby infected tissue.

*Gotta be super careful of this kind of infection in ARTIFICIAL JOINTS.

36
Q

Acute infectious arthritis is typically bacterial or viral. In bacterial, what joints are involved and what symptoms will you see?

A

Usually SINGLE joint, (usually knee, rarely axial)

Symptoms will include severe, acute pain, warm joint, limited ROM and fever

37
Q

What are the common criminals of infectious bacterial arthritis? How do you get rid of em?

A

N. gonorrhea, S. aureus (spreads from infected mucosa).

Tx includes IV antibiotics

38
Q

Who are the common culprits of viral infectious arthritis?

A
  1. Rubella
  2. Parvo B19
  3. HepA/B/C
  4. HIV
  5. HTLV1
  6. Chikungunya
39
Q

When you treat Lyme disease, does it typically recur?

A

Yes.

40
Q

Tuberculosis can give you some arthritis too. This develops over weeks. What is the name of TB in the joints

A

Pott disease. Affects the spine predominantly.

41
Q

What fungus gives you arthritis?

A

CANDIDADADADADADA

42
Q

Describe the pathophysiology involved in gout.

A
  1. Purine metabolism produces uric acid. Excess production or decreased excretion of uric acid –> hyperuricemia.
  2. Monosodium urate crystals (MUC) deposit in tissues/joints
  3. Crystals trigger acute inflammatory response. Mostly neutrophils.
43
Q

What symptoms will you see in gout?

A
  1. Sudden onset painful arthritis
  2. Red, warm joint
  3. First attack to great toe (Podagra)
  4. Mild fever
44
Q

What lab findings and synovial fluid findings will you see in gout?

A
  1. Labs: hyperuricemia, plasma urate >6.8

2. Synovial fluid: yellow needle-shaped crystals with negative birefringence under polarized light

45
Q

What are the risk factors for gout? So mannyyyyyy

A
  1. Old age
  2. Male
  3. Family history of the condition
  4. Obesity
  5. drugs (diuretics, low-dose aspirin, cyclosporine, or levodopa)
  6. Binge drinking 7. Lead toxicity
  7. Organ transplants
  8. Thyroid problems
46
Q

What might trigger a gout attack?

A
  1. Dehydration
  2. Joint injury
  3. Overindulging in alcohol or purine-rich foods
  4. Severe illness or infection
  5. Sudden weight loss/ “crash diets”
  6. Surgery
47
Q

What’s the difference between primary and secondary gout?

A

Primary: unknown cause of hyperuricemia
Secondary: caused by underlying condition like LEUKEMIA, LESCH-NYHAN syndrome or RENAL INFUFFICIENCY

48
Q

What is tophaceous gout?

A

repeated attacks of acute gout leads to chronic inflammation and cartilage damage. You will see tophi formation, which is white, chalky aggregates of uric acid crystals. If the crystal deposit in the kidney tubules this can lead to kidney failure

49
Q

What do you see on X-ray for tophaceous gout?

A

juxta-articular bone erosion by osteoclasts

50
Q

What’s the difference between gout and pseudo gout?

A

Same clinical presentation but:

  1. Deposition of calcium pyrophosphate dehydrate (CPPD) crystals (not MSU)
  2. Blue rhomboid shaped crystals that have positive birefringence under polarized light
51
Q

Juvenile Idiopathic Arthritis shows was joint distribution and affects what demographic?

A
  1. Asymmetric, LE spondyloarthropathy

2. boys 7-16 years old

Multisystem Path (11 decks)

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