2002
2002
Rheumatology
Q1
Which of the following is most specific for SLE?
a) sm
b) anti Jo 1
c) ANA
d) Anti-la
e) Anticentromere
The presence of characteristic antibodies) confirms the diagnosis of SLE. ANAs are the best screening test. If the test substrate contains human nuclei (WIL-2 or HEP-2 cells), more than 95% of lupus patients will be positive. A positive ANA test is not specific for SLE; ANAs occur in some normal individuals (usually in low titer); the frequency increases with aging. Other autoimmune diseases, viral infections, chronic inflammatory processes, and several drugs induce ANAs. Therefore, a positive ANA test supports the diagnosis of SLE but is not specific; a negative ANA test makes the diagnosis unlikely but not impossible. Antibodies to double-stranded DNA (dsDNA) and to Sm are relatively specific for SLE; However, determining the complete autoantibody profile of each patient helps predict clinical subsets. High serum levels of ANAs and anti-dsDNA and low levels of complement usually reflect disease activity, especially in patients with nephritis. Total functional hemolytic complement (CH50) levels are the most sensitive measure of complement activation but are also most subject to laboratory error. Quantitative levels of C3 and C4 are widely available. Very low levels of CH50 with normal levels of C3 suggest inherited deficiency of a complement component, which is highly associated with SLE and with ANA negativity.
Answer is A
Q2
A 25 year old woman in her first pregnancy is concerned about her sister’s history of a child that died in the neonatal period with complete heart block. Best choice of investigations for this woman?
a)ANA
b)anti la (SSB) antibodies
c) anti-phospholipid antibodies
d) anti-cardiolipin antibodies
f) anti DNA
congenital heart block, in which damage to the developing conducting system of the heart as a result of transfer of anti-Ro antibody from the mother results in permanent heart block.
Sera of patients with Sjögren's syndrome often contain a number of autoantibodies directed against non-organ-specific antigens such as immunoglobulins (rheumatoid factors) and extractable nuclear and cytoplasmic antigens (Ro/SS-A, La/SS-B). Ro/SS-A autoantigen consists of three polypeptide chains (52, 54, and 60 kDa) in conjunction with RNAs, whereas the 48-kDa La/SS-B protein is bound to RNA III polymerase transcripts. The presence of autoantibodies to Ro/SS-A and La/SS-B antigens in Sjögren's syndrome is associated with earlier disease onset, longer disease duration, salivary gland enlargement, severity of lymphocytic infiltration of minor salivary glands, and certain extraglandular manifestations such as lymphadenopathy, purpura, and vasculitis. Antibodies to [pic]-fordin (120 kDa), a salivary gland-specific protein, have recently been found in sera of patients with Sjögren's syndrome but not in sera of patients with other connective tissue diseases.
Sensorineural hearing loss was found in one-half of patients with Sjögren's syndrome and correlated with the presence of anticardiolipin antibodies.
As SSA and SSB go together, the answer is B)
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