SYSTEMIC LUPUS ERYTHEMATOSUS

Question 1

Autoimmune disorder characterized by the production of auto Abs and inflammation in multiple organs

Answer 1

SLE (systemic lupus erythematosus)

Question 2

What is an LE cell phenomenon?

Answer 2

Neutrophil that has engulfed an antibody-coated nucleus of another neutrophil

Question 3

Most striking feature of the disease, however it is not diagnostic:

Answer 3

Antinuclear antibodies

Question 4

What is the source of antigens for ANA testing of LE?

Answer 4

Mouse kidney or human epithelial HEp-2 cells are fixed to a slide

Question 5

Most pathognomonic and most specific for SLE, homogenous or nuclear (peripheral) pattern in indirect IF

Answer 5

Anti-dsDNA Abs

Question 6

Also highly specific for SLE, speckled (mottled) pattern in indirect IF

Answer 6

Anti-Sm Abs

Question 7

Anti-histone Immunofluorescent pattern?

Answer 7

Homogenous

Question 8

Anti-histone disease association:

Answer 8

Drug-induced SLE

Question 9

Characteristic of antigen of Anti-Scl-70:

Answer 9

DNA topoisomerase I

Question 10

Anti-Scl-70 immunofluorescent pattern:

Answer 10

Atypical speckled

Question 11

Anti-Scl-70 disease association:

Answer 11

Systemic sclerosis
Scleroderma

Question 12

What autoantibody is not detected on the routine screen? (Indirect immunofluorescence)

Answer 12

Anti-ss DNA

Question 13

Characteristics of antigen of Anti-Sm

Answer 13

Extractable nuclear antigen (RNA component)

Question 14

Staining pattern of ANA

• Results from antibodies to DNA (nDNA, dsDNA, DNP)
• Active stage of SLE
• Sjoren’s syndrome, lupus nephritis

Answer 14

Nuclear rim (peripheral)

Question 15

Staining pattern of ANA

• Results form anti-DNA-nucleoprotein abs (nDNA, dsDNA, ssDNA, histones, DNP)
• Seen in rheumatoid disorders and other connective tissue defect
• SLE

Answer 15

Homogenous (solid/diffuse)

Question 16

Staining pattern of ANA

• Involves extractable nuclear antigens (ENA) such as Smith (Sm) antigen and ribonucleoprotein (RNP)
• high RNP titer suggests MCTD (mixed connective tissue disease)
• Anti-Sm abs have been seen to he highly specific for patients with SLE and appear to be a marker Ab
• Also present in scleroderma

Answer 16

Speckled (mottled)

Question 17

Staining patterns of ANA

• Reflects antibodies to nuclear RNA
• Rare in SLE or RA
• Present in progressive systemic sclerosis, Sjogren’s syndrome
• Does not stain the matrix

Answer 17

Nucleolar

Question 18

Staining pattern of ANA
- may be seen in SLE but not in RA

Answer 18

Thready

Question 19

Staining pattern of ANA

• Abs to CENTROMERE, DNA, RNA, ENA
• Crest, variant of PSS (progressive systemic sclerosis)

Answer 19

Discrete, speckled