Lupus Flashcards

1
Q

ACR Lupus Criteria 1997

A
Malar Rash
Discoid rash 
Photosensitivity 
Serositis
Hematological d/o
Neurological d/o
Arthritis 
Renal disease 
Autoantibodies
ANA 
Oral Ulcers 

Need four of 11

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2
Q

Pathways involved in clearing apoptotic materials

A
Complement and receptors 
PD1
BCL1
Fas/Fas ligand 
Immunoglobulin receptors
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3
Q

What are NETs rich in?

A
  • DNA
  • LL37
  • High mobility group protein B1 (HMGB1)
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4
Q

Adaptive Immunity pathology of lupus

A

IC are formed and activate pDC
pDC produce IFNa
Fc gammaR IIa on the pDC membrane delivers IC to TLR7 (if RNA) and TLR9 ( if DNA)
TLR induced production of IFNa leads to differentiation of monocyte to DCs and activation of immature myeloid DCs
myeloid DCs activate auto reactive T and B cells, leading to increased autoantibody production
mDCs also cause CD8T cells to differientate into cytotoxic T cells

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5
Q

Risk of a sibling of a patient with lupus having lupus

A

20 fold increased compared to general population

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6
Q

Which single gene mutations can cause lupus?

A
homozygous c1q deficiency (90%)
C2 deficiency (1/3), higher association with Ro antibodies 
C4 (increased risk)
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7
Q

Name a disease in which hormones play a role in the development of lupus

A

Klinefelter syndrome has an increased risk of lupus but they generally have milder disease as compared to XY adult men with SLE (higher risk of nephritis)

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8
Q

Viruses implicated in lupus

A

EBV

CMV

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9
Q

Drugs implicated in DIL

A
Hydralazine (HIGH) 
Procainamide (HIGH) 
Isoniazid
Methyldopa 
Quinidine (Moderate) 
Minocycline 
Chlorpromazine 

In children-anti epileptics meds are commonly implicated

TNFi can cause signs suggestive of SLE (anti ds DNA, low complement etc)

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10
Q

Anti histone ab

A

Suggestive of DIL but not diagnostic

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11
Q

Pathogenesis of LN

A

Formation of ICs lead to activation of complement and FC gamma receptors

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12
Q

DNASE I

A

Major renal nuclease
Found to be down regulated in LN leading to impaired clearance of apoptotic cells leading to further exposure of chromatin fragments

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13
Q

What is a full house?

A

Deposition of IgA, IgM, IgG, C3, C1q, and kappa and lambda light chains

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14
Q

Acute nephritic syndrome

A

Microscopic hematuria (occ macro)
HTN
Proteinuria (mild to nephrotic)

*Commonly a manifestation of Class III or IV

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15
Q

What to consider if you notice gross hematuria in a lupus patient

A

Renal vein thrombosis
Thrombotic microangiopathy
Clotting factor deficiency (PT deficiency)

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16
Q

Nephrotic Syndrome

A

Nephrotic range proteinuria
Hyperlipidemia
hypoalbuminemia
Edema

*Commonly seen in class V

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17
Q

Complications associated with Class V

A

Thrombosis

CV events

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18
Q

Predictors of poor renal outcomes

A
African American race 
low GFR (<60 mL/min/1.73m2 
Nephrotic range proteinuria at presentation
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19
Q

Recurrence of SLE after transplantation

A

20-30% though recurrence is generally mild and not associated with decreased patient survival

20
Q

Leading cause of death in pediatric LN patients before and after ESRD

A

Infection

Cardiovascular complications

21
Q

Which antibody triggers neutrophils to become apoptotic?

22
Q

Percentage of first degree relatives of patients with SLE who also have the disease

23
Q

Percentage of children with a mother with lupus who have a positive ANA

24
Q

Pathology of acute cutaneous lupus

A

Disruption of the dermal-epidermal junction, infiltration of T cells and fibrinoid degeneration

25
Pathology of chronic cutaneous lesions
Thickening of the keratotic layer, epidermal atrophy, follicular plugging, and fibrosis of the elastic tissues
26
Most common sign of neuropsychiatric disease in cSLE
Headache --> described as a migraine or unrelenting, severe headache requiring narcotic analgesia
27
Most common cardiac manifestation of lupus
Pericarditis - Chest x ray may show an enlarged cardiac silhouette - EKG shows elevated ST segments and peaked t waves - Treatment if mild is usually steroids and NSAIDs - May require IV steroids pulse
28
Signs of myocarditis
- Elevation in CK mB and troponin - Enlarged silhouette - EKG shows nonspecific ST and T wave changes and arrythymias - Echo shows global hyperkinesis, reduced LV EF, regional wall motion abnormalities
29
Most common valvular disease in lupus
Libman Sacks endocarditis - Sterile, small, verrucous value lesions typically on the mitral valve but also found on other valves, chordae tendineae and endocardium - most common in longer disease activity, with APLs, high disease activity - Usually asymptomatic
30
Most common PFT finding in lupus
restrictive disease and or impaired diffusion.
31
Bronchoscopy findings supportive of PAL
Frank blood in airways | serosangionous BAL fluid, hemosiderin laden macrophages, absence of purulent sputum, lack of infectious organisms
32
Cause of Autoimmune hemolytic anemia
Warm agglutinins
33
Signs of anemia of chronic disease
Low Hg, normocytic, normochromic, low iron, low relic count, high ferritin
34
Signs of HUS
MAHA, thrombocytopenia, acute kidney injury --look for schistocytes
35
Signs of TTP
MAHA, thrombocytopenia, fever, AKI, neurological deficits -look for schistocytes
36
Leukocytoclastic vasculitis
Erythematous or violaceous punctate, non blanching less of the fingertips or palms
37
Cause of thrombocytopenia associated with ITP
Antibodies against GP11B/IIa and thrombopoietin receptor TPOR
38
Most common areas to clot with positive LAC
Legs Cerebral veins Pulmonary vasculature
39
Anti Ro/SS-A
Strongly associated with neonatal lupus and subacute cutaneous lupus
40
Anti- C1q
Good marker of LN activity and correlates with severity of nephritis and proteinuria - also associated with angioedema symptoms
41
What can be tested to find those at risk of AZA toxicity ?
Thiopurine methyltransferase (TPMT)
42
Most often causes of death
Infection, renal failure, cardiopulmonary
43
Findings suggestive of lupus in lymphoid tissue
Hematoxylin bodies
44
What can be found in patients with transverse myelitis?
- APls
45
When do clinical manifestations of drug induced lupus resolve?
- 6 months after the drug is removed
46
When do ds DNA antibodies go away?
- 12 to 24 months