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Musculoskeletal - Pathology > SLE > Flashcards

SLE Flashcards

1
Q

Differentiate the following diseases:
• Systemic Lupus Erythematosus (SLE)
• Discoid Lupus Erythematosus (DLE)
• Drug-Induced Lupus Erythematosus (DILE)
• Neonatal Lupus Erythematosus

A

Systemic Lupus Erythematosus (SLE)
• Multisystem

Discoid Lupus Erythematosus (DLE)
• Confined ONLY to skin, no other systems involved

Drug-Induced Lupus Erythematosus (DILE)
• Less Severe than SLE

Neonatal Lupus Erythematosus
• Newborns of mother with SLE

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2
Q

T or F: SLE is a problem of autoreactive B cells

A

False, SLE is a problem of autoreactive T cells

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3
Q

How commonly do people with Discoid Lupus Erthematosus progress to SLE?

A

5 - 15% of people with DLE progress to SLE

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4
Q

What are two defining symptoms of Neonatal Lupus?
• will the resolve with loss of maternal Abs?

A

Skin Rash and Heart Block may be seen.

• Skin Rash will resolve, but Heart Block is permanent

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5
Q

T or F: Drug induced lupus will resolve with removal of the offending drug.

A

True

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6
Q

SLE
• Is there a genetic component to this disease?
• How do we know?
• HLA types associated?

A

Yes there is a genetic component we can see this by the following progression:

  • general population risk of getting SLE is 0.05%
  • Silbings/Dizygotic Twins 5% risk
  • Monozygotic Twins 25-50% risk

HLA DR2 and 3 may be ass’d with disease

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7
Q

What is suggested by the fact that only 25-50% of monozygotic twins with SLE get SLE if the other sibling has it?

A

Since these people share the same genome this suggests and ENVIRONMENTAL component is also responsible

(seems like epigenetics could also be an explanation)

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8
Q

SLE is a disease characterized by symptom flare-ups. What are some predictors of flares in these people? (name 4)

A
  • C3 and C4 complement Deficiency
  • Rising anti-dsDNA titers (may preceed flare by 1 mo.)
  • Increased ESR
  • New Lymphopenia
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9
Q

What is the typical presentation of SLE at the onset of disease?

A

• Abrupt onset of symptoms with increased renal, neurological, hematologic, and serosal involvment.

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10
Q

Jeremy is a 13 year old African American male from one of the poorer communities in Memphis. He presents with a sudden onset of fever, leukopenia, and a Malar rash. Labs reveal the present of Anti-dsDNA and anti-Sm Antibodies.
• what are is his prognosis? explain.

A

Increased Disease severity is characterized by:
• Race (AA’s typically have more severe disease)
• Age of Onset (young = bad)
• Male Gender (these patients tend to do REALLY poorly)
• Low Socioeconomic Status

•••Jeremy is positive for all of these factors and thus has a poor prognosis***

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11
Q

Even in cases where patients have a poor Lupus prognosis, what are 3 things we as physicians could do to improve outcome?

A

• Help Patients understand the importance of Adherance, help them workout a Support System, Educate patients on the disease.

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12
Q

What are the leading causes of death in Lupus?
• 5 year survival rate?

A

5 year survival rate is currently greater than 90% with the leading causes of mortality being Heart Disease, Malignancy, Infection.

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13
Q

What 11 criteria are there for determining if someone has lupus?
• how many of these must your patient have before you diagnose them with this disease?

A

SOAP BRAIN MD

  • *S**erositis
  • *O**ral ulcers
  • *A**rthritis
  • *P**hotosensitivity
  • *B**lood (hemolytic anemia, leukopenia, lymphopenia, etc)
  • *R**enal (Glomerulonephritis)
  • *A**nti-nuclear Antibodies
  • *I**mmunologic Disorder (anti-DNA ab, anti-Sm ab, or anti-PPLipid)
  • *N**eurologic disorder (seizures and/or psychosis)
  • *M**alar Rash
  • *D**iscoid Rash
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14
Q

What is shown here?
• how commonly is this seen in Lupus?
• Are these sensitive or Specific?

A

IF showing ANAs (against many different components in the nucleus), these are present in 95-98% of pts.
ANAs are super sensitive, but not very specific for SLE

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15
Q

A patient has a negative ANA. What are the chances that they have lupus?

A

ANA is very sensitive so if ANA is negative there is a very small chance of them having Lupus

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16
Q

What are some reasons for having a positive ANA other than Lupus?
• How common is it to see ANA in someone without any disease?

A

**ANAs are present in about 3-4% of the general population**

  1. Other Autoimmune Diseases:
    • Scleroderma (95%)
    • Hashimoto’s thyroiditis (50%)
    • Idiopathic pulmonary fibrosis (50%)
  2. Old Age
  3. Chronic Infections/Conditions
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17
Q

What are 7 autoantibodies commonly seen in Lupus Patients?

A
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18
Q

Which of the 7 antibodies commonly seen in lupus are most specific to this disease?

A
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19
Q

Which antibodies in Lupus have low specificity for the disease?

A
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20
Q

You notice that your lupus patient has severe nephritis, what autoantibody is likely present in this patient?
• why does this one cause this?

A

Anti-dsDNA antibodies form immune complexes that deposit in the glomerulus

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21
Q

Your lupus patient presents with Arthritis, Myositis, and Lung disease. What antibody would you expect to see in this patient’s panel?

A

Anti-RNP (Histones etc.)

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22
Q

What antibody might you look for if you suspect that a patient has Subacute Cutaneous Lupus Erthematosus or Neonatal Lupus?
• What symptoms might go along with the presence of this Ab?

A

Neonatal Lupus and Subacute Cutaneous Lupus Erythematosus (SCLE) are associated with Anti-SSA and SSB antibodies.

Symptoms:
• Dry Eyes/Mouth
• Photosensitivity

***Note the discoid lessions on this kid’s head***

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23
Q

Complement activation has a role in causing nearly adverse effect seen in lupus patients. What should you look for to determine if complement is flaring up in these patients?

A

Drop in C4 will tell you the classical pathway is getting kicked off

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24
Q

What is the KEY difference between arthritis induced by Lupus and those of RA?
• is the arthritis symmetrical or asymmetrical?
• Can it cause deformity?

A

These look very similar but RA deformities show destruction of cartilage and bone in the joint space. This is not a feature of lupus.

• lupus arthritis can be symmetrical or asymmetrical and may have deformity

25
Q

How do you treatment options differ if this defect was caused by Lupus or by RA?
• Which of the two twas this caused by and how do you know?

A

This has been caused by lupus. You can tell because there is no damage to the bones or cartilage. Treatment differs in that these joints can be reduced. This is not an option in RA.

26
Q

What are some of the places that serositis manifests in Lupus?
• what are some of the symptoms of serositis?

A

Serositis:
Pleura:
• Pleuritic chest pain/Pleural Effusion

Pericardium
• Chest Pain/Pericardial Effusion

Peritoneal Cavity:
• Abdominal pain, Subclinical fluid accumulations

27
Q

What is seen on this CXR?
• what is the difference in the L and R?

A

Serositis of the Pleura has resulted in a pleural effusion in this patient. The Right image is the same patient lying on their right side.

28
Q

What percentage of SLE patients experience renal problems?
• In what part of the kidney is this pathology found?

A

Renal Pathology is found in 50% of patients with SLE
Any part of the kidney can be involved

29
Q

Explain the pathogenesis of kidney damage in SLE patients.

A

Immune complexes circulate and are pushed through the glomerulus and produces subendothelial dense deposits.

Immune complexes may may also form IN SITU from local B-cell that produce Ig against components of kidney cells

***Damage occurs in most cases from complement activation and MAC formation***

30
Q

What hemotologic disturbances are characteristic of SLE?
• affected cell line and pathogenesis?
• what is indicated by a low platelet count?

A

***ALL cell lines may be affected***

RBCs
• may be reduced as a result of CHRONIC INFLAMMATION (hepcidin, ferroportin)
• Autoantibodies may be generated

WBCs
• Autoantibodies
• Medications (azothioprine, etc)

Platelets
• Autoantibodies - LOW PLATELET COUNT INDICATES ACTIVE dz.

31
Q

What are some common psychiatric abnormalities of lupus?

A

Depression is most common, but just about any psychiatric pathology may manifest. YOU MUST EXCLUDE OTHER CAUSES.

32
Q

How often will you see GI involvment in Lupus?
• what are the symptoms when you do see it?

A

GI involvment is LESS common

Pts. with GI involvment experience:
• Abdomonial pain - MOST COMMON SYMPTOM
• Serositis, vasculitis => bowel perforation, and peritonitis may also occur
• Liver may also be involved causing autoimmune hepititis

33
Q

What is seen here?
• what problems might the mother have had before delivering this child?
• what may wrong with this child?

A

Neonatal Lupus => Transplacental transfer of Abs.

Mother may have had problems with recurrent fetal loss due to antiphospholipid antibody causing thrombosis in the placenta. The child may be small for his gestational age. Problems seen in kids with neonatal lupus include Rash, Leukopenia, Heart Block

34
Q

What CV involvment do we see in SLE?

A

ALL parts of the heart are affected:

  • *• Pericardium** - Shaggy looking pericarditis
  • *• Myocardium** - myocarditis
  • *• Valves** - Endocarditis - LIBMAN SACKS
  • *• CAD (**coronary artery disease) - Due to meds and Lupus
  • *• Peripheral Vasculature** - Vasculitis and Raynauds
35
Q

At what age to people with lupus often start developing coronary artery disease?

A

20 and 30’s

36
Q

What is shown here?

A

Libman Sacks Endocarditis

37
Q

What complication of Lupus has lead to the pathology seen here?

A

SEVERE Raynaud’s as a result of vasculitis

38
Q

Drug induced lupus is not typically associated with _______________ unless ______________ were administered to patients.

A

Drug induced lupus is not typically associated with Nephritis unless TNF-alpha antagonists were administered to patients.

39
Q

What are some common drugs that have been established as Lupus Inducing Drugs?
• should you give these drugs to a lupus patient?
• What natural factors can cause a similar effect?

A

Common Drugs:
• Hydralazine - (vasodilating antihypertensive)
• Procainamide - (class IA antiarhythmic agent - blocks fast Na+ channels to slow conduction velocity)
• Isoniazide - (TB drug activated by KatG in MTB and forms adducts with B6)
• Hydantoins
• Chlorpromazine
• Methyldopa (CNS alpha-2 blocker)
• Pinicillamine
• Minocycline (Tetracycline abx)
• TNF-alpha inhbitors
• Interferon-alpha

40
Q

What is believed to be one of the reasons females are more susceptible to lupus than males?

A

Estrogen and Prolactin

41
Q

What are some dietary foods/Habits that are thought to increase your chances of inducing lupus?

A

• VITAMIN D DEFICIENCY - seen in almost ALL lupus patients on first visit
• Alfalpha sprouts and other related sprouts
• Canavanine
• Pristane and other hydrocarbons
• Infectious agents other than EBV
• Bacterial DNA
• Smoking

42
Q

What therapy is currently in use for SLE?
• which of these are the go-to’s?

A
  • *• Corticosteroids** (NFkB inhibition via IkB, HDAC action, PLP2)
  • *• Cyclophosphamide** (alkylating agent, hemorrhagic cystitis)
  • *• #3 Methotrexate** (DHFR, FAICAR inhibit => Adenosine, Met block)
  • *• Mycophenolate mofetil** (IMP dehydro-ase in de novo Purine syn)
  • *• #1 Azathioprine** (de novo purine block => see image)
  • *• #2 Hydroxychloroquine** (increase in lysosomal pH, EYE and CNS)
  • *• Belimumab** (anti BLYS ab.)
43
Q

Your lupus patient presents with a high titer of anti-RNP antibodies.
• what drug that is typically not your go-to for lupus might you use?
• Why?

A

Anti-RNP is often associated with a lot of lupus associated arthritis. If this person had a lot of Lupus arthritis then you might want to consider using MTX because it works great for these purposes.

44
Q

What are some common side effects of using corticosteroids to treat lupus?

A
45
Q

Anti-phospholipid Sydrome:
• What phospholipid is typically targeted?
• What complications are most commonly seen? (arterial, venous?)
• What complication are most feared?

A

APLS:
• Typically results from abs. directed against th ß2-glycoprotein I. Most common complication on the arterial side is stroke, while DVTs are the most common venous complications. Often patients are asymptomatic with the most feared complication being Catasphrophic APS that results in multiple thromboses of medium and small arteries over a period days.

46
Q

What is required to make the diagnosis of APS in someone ?

A
  1. Persistant Presence of anti-phospholipid antibodies (2 positive tests measured 12 weeks apart)
  2. A Clinical Event must also occur (thombosis or pregnancy morbidity)
47
Q

How common is it for an otherwise normal person to have a positive aPL test?
• SLE pts?
• RA pts?

A
  • 10% of normal blood donors are positive
  • 10-40% of SLE patients are positive
  • 20% of RA patients
48
Q

What are some clinical signs that a patient may anti-phospholipid syndrome?

A
  • Livedo Reticularis (remember Amantidine also causes this)
  • Thrombocytopenia
  • Autoimmune hemolytic anemia (Schistocytes)
  • Cardiac valve disease (thickening or vegetations)
  • Mulitple scleroisis-like syndrome, chorea, or other myelopathy
49
Q

What Labortatory Findings allow for the diagnosis of APLS?

A

IgA anticardiolipin ab.
IgA anti-ß2-glycoprotein I

50
Q

What are the key characteristics of this biopsy taken from a lupus patient?

A

Superficial and Deep perivascular inflammation (see blue densities of inflammatory cells in both epidermis and dermis). Mucin gives the reticular dermis a whispy blue appearance. (often times a mucin stain is also done)

51
Q

what is shown here?

A

• Inflammation at the Dermal-Epidermal junction in an SLE patient

52
Q

What do you look for on immunofluorescence on a skin biopsy of patients with SLE?
• Where should you take this biopsy from?
• is this diagnositic of SLE?

A
  • Dpeosition of Ig AND complement along the dermoepidermal junction. (take the biopsy from a non-sunexposed area because sun-exposed skin may also show this pattern)
  • The pattern seen is NOT specific to SLE and may be seen in other diseases like scleroderma
53
Q

What are you most likely to see in the kidney biopsy of someone with SLE?
• what is the prognosis?

A

You’re likely to see Class IV lupus nephritis => Diffuse Proliferative Glomerulonephritis. Wire Loops will be present and more than 50% of the glomeruli will be involved.

54
Q

What is shown here?

A

Focal proliferative GN in a patient with lupus (class III)

55
Q

What differentiates this tissue from normal tissue?

A

• Huge Increase in cellularity

56
Q

Note You need a PAS stain to see the WIRE LOOPs, that’s why they weren’t seen in the previous card with H and E staining

A
57
Q

What do you expect to see on immunofluorscence of a Kidney with Lupus?
• antibody type?

A

• IgG antibody is dispersed in a GRANULAR PATTERN

58
Q

What complication of Lupus is this patient likely experiencing?
• what kidney related symptoms was this lady likely experiencing?

A

Thrombosis as a result of APS => note all of the thrombi in this glomerulus

• Microhematuria and non-nephrotic proteinuria

59
Q

What is seen here?

A
  • Renal Artery thickening (left)
  • Recanalization and Arteriosclerosis (right)

Musculoskeletal - Pathology (17 decks)

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