Lupus

Question 1

What is lupus?

Answer 1

Chronic inflammatory autoimmune disease

Question 2

What are the courses of lupus?

Answer 2

Benign with occasional flares
Frequent relapses
Remission
Permanent organ damage
Life-threatening complications

Question 3

Patho of lupus?

Answer 3

1. Abnormal immune response
2. Pathogenic autoantibodies
3. Deposit in tissue

Question 4

What are the autoantibodies in lupus?

Answer 4

Antinuclear
Antiphospholipid
Anti-double-stranded DNA

Question 5

What are part of the pathogenesis in lupus?

Answer 5

Genetics
Environmental triggers
Immunologic triggers
Hormonal

Question 6

What is involved with gentics in lupus?

Answer 6

IRAK1 on X-chromosome

10% of SLE pts have family history

Question 7

What is involved with enviromental factors in lupus?

Answer 7

Ultraviolet light

Drugs

• Procainamide
• Hydralazine
• Quinidine
• Isoniazid

Question 8

What is involved with immunologic in lupus?

Answer 8

Viruses- Epstein Barr

Dietary factors
-amino acid: L-canavanine

Question 9

What should you be concerned with in history within lupus?

Answer 9

Medications: procainamide, hydralazine
Sunlight exposure
Hair loss
Raynaud’s phenomenon
Antiphospholipid syndrome
FH

Question 10

What is involved with MSK in lupus?

Answer 10

Nonerosive in 2 or more peripheral joints
Wrist 
MCP/PIP
Pain/stiffness out of proportion to PE
Some pt have Fibromyalgia

Question 11

What is involved with kidneys in lupus?

Answer 11

Major cause of morbidity and mortality
Within 1st 2 yrs of onset
Leads to renal insufficiency and failure
Need renal biopsy for definitive diagnosis

Question 12

What is involved with cardiopulmonary in lupus?

Answer 12

Pleuritis
Parenchymal lung disease
Pulmonary thrombosis
Pericarditis
Myocarditis 
Not part of diagnostic criteria

Question 13

What is the major cause of death in SLE?

Answer 13

Accelerated artherosclerosis with vascular endothelial dysfunction

Question 14

What are antiphospholipid antibodies involved with?

Answer 14

Pulmonary thrombosis

Question 15

What are the constitutional signs in lupus?

Answer 15

fatigue
malaise
wt loss
fever
lymphadenopathy

Question 16

What are the skin/hair signs in lupus?

Answer 16

malar rash
discoid rash
photosensitive rash 
Raynaud’s phenomenon 
alopecia

Question 17

What are the msk signs in lupus?

Answer 17

polyarthritis
polyarthralgias
myositis

Question 18

What are the kidney signs in lupus?

Answer 18

HTN
proteinuria
hematuria
renal failure

Question 19

What are the CNS signs in lupus?

Answer 19

seizures
psychosis
stroke
depression
H/A

Question 20

What are the CV signs in lupus?

Answer 20

pericarditis
myocarditis
conduction abnormalities

Question 21

What are the GI signs in lupus?

Answer 21

hepatosplenomegaly pancreatitis

bowel vasculitis

Question 22

What are the ocular signs in lupus?

Answer 22

retinal vasculitis

scleritis

Question 23

What are the hematologic signs in lupus?

Answer 23

hemolytic anemia

thrombocytopenia

Question 24

What is involved with neuropsychiatric in lupus?

Answer 24

Affects CNS, PNS and ANS
Seizures and psychosis are only sysptoms listed in ACR
Cognitive impairment = MOST COMMON manifestation

Question 25

What is included in American College of Rheumatology: Classification Criteria?

Answer 25

1. Malar rash
2. Discoid rash
3. Photosensitivity (60-100%)
4. Oral ulcers
5. Arthritis
6. Serositis (pleuritic or pericarditis)
7. Renal disorder
   - Proteinuria (> 3+)
   - Cellular casts (red cell; hemoglobin; granular; tubular or mixed)
8. Neurologic disorder
   - Seizures or psychosis
9. Hematologic disorder
   - Hemolytic anemia w/ reticulocytosis; leukopenia; lymphopenia; thrombocytopenia
10. Immunologic disorder
    - ANA to double-stranded DNA; + antibody to Smith nuclear antigen; + Antiphospholipid antibody
11. Antinuclear antibody (ANA)
    *****Need 4 or more of the 11 features
    95% specific; 85% sensitive

Question 26

What is your DDX with suspected lupus?

Answer 26

RA
Mixed connective tissue disease
Systemic vasculitis
Neoplastic disorder
Systemic infection

Question 27

What are the labs used in lupus?

Answer 27

CBC with diff
CMP
ESR
CRP
PT
PTT
ANA
UA

Question 28

What imaging is used in diagnosis of lupus?

Answer 28

CXR
- Pleural effusion
- Pulmonary infiltrates
ECG
Echocardiogram

Question 29

What is the initial approach to the treatment of lupus?

Answer 29

Screening
- Artherosclerotic risk factors- leading cause of m/m in SLE

Counseling
- Smoking cessation

Conservative vs medicinal treatment
- Will depend on the severity of disease

Question 30

What is the conservative treatment in lupus?

Answer 30

Lifestyle modifications

• Avoid sunlight/ high SPF sunscreen
• Avoid tobacco
• Avoid fatigue
• Adequate sleep

Question 31

What medications are used in treatment of lupus?

Answer 31

Ibuprofen
- Not in acute nephritis

Hydroxychloroquine

• Skin lesions
• Arthralgia
• Arthritis
• Alopecia
• Malaise
• ACR guidelines for pts with lupus nephritis
• Improved lipid profiles, decreases thrombosis and prevents flares

Topical glucocorticoids
- Isolated skin lesions

Systemic glucocorticoids
- Vary based on disease process

Immunosuppressive therapy (usually prescribed by Rheumatology)

• Cyclophosphamide
• Life or organ threatening manifestations

Methotrexate

Question 32

What is the prognosis of lupus?

Answer 32

Newly dx

• 90% 5 yr survival rate
• Immunosuppressive therapy

85%
- 15 yr survival rate

Worse prognosis
- AA, Asian, and Hispanics

Leading cause of death in SLE patients
- Atherosclerosis

Question 33

Table comparing antibodies.

Answer 33

Anti-dsDNA 60% 95%
specificity for SLE; fluctuates with dz; assc with glomerulonephritis

Anti-Smith 20-30%
99% specific for SLE

Anti-U1RNP 30%
mixed connective tissue dz

Anti-Ro/SSA 30%
Sjӧgren’s syndrome, photosensitivity

Anti-La/SSB 20%
Sjӧgren’s syndrome

Antihistone 70%
Drug induced lupus

Antiphospholipid 30%
Arterial and venous thrombosis; pregnancy morbidity