вход по аккаунту


Sj syndromecomments on the proposed crieteria for classificaton.

код для вставкиСкачать
Sjogren’s syndrome: comments on the proposed
criteria for classification
To the Editor:
We read with great interest the paper by Fox and
colleagues (I), who critically reviewed some of the current
criteria for the diagnosis of Sjogren’s syndrome (SS). Because much confusion exists concerning the diagnosis of SS,
we agree with those investigators that it is important to draw
rheurnatologists’ attention to the various criteria used. During the last decade, we have been actively involved in
defining criteria for this disease ( 2 ) , and, as organizers of
the First International Seminar on Sjogren’s Syndrome,
held in Copenhagen in May 1986, where these matters
were discussed, we would like to comment on the article by
Fox r:t al.
The Copenhagen criteria were formulated in 19761977, and are based upon the use of objective tests only-not
symptoms-in the formulation of diagnosis. The relationships between keratoconjunctivitis sicca (KCS), xerostomia,
and primary and secondary SS are illustrated in Figure 1.
Primary SS is defined in the Copenhagen criteria by
the simultaneous presence of KCS and xerostomia in patients who do not fulfill internationally accepted criteria for
other chronic inflammatory connective tissue diseases (e.g.,
rheumatoid arthritis, systemic lupus erythematosus, or progressive systemic sclerosis). If the patient has another
chronic inflammatory tissue disease and, at the same time,
KCS andlor xerostomia, secondary SS is diagnosed.
In the Copenhagen criteria, the diagnoses of KCS
and xerostomia are both said to be present if results of at
least :1 tests for each organ are abnormal. We routinely use 3
tests (Figure 2). The local SS center determines which
objective tests should be used, but any test may be replaced
by another test for which results are reproducible and have
a high degree of sensitivity and specificity.
For the diagnosis of KCS, we use the following
ocular tests (Figure 2 ) : Schirmer-I test (abnormal findings
510 r n d 5 minutes), break-up time (abnormal findings 5 1 0
seconds), and van Bijsterveld score (abnormal findings 2 4
points on a scale of 0-9 points). The van Bijsterveld score is
a semiquantitation of the 1% rose bengal vital staining.
For the diagnosis of xerostomia, we use the following
oral tests (Figure 2): unstimulated whole sialometry (abnormal findings 51.5 mV15 minutes), salivary gland scintigraphy
(abnormal if uptake or spontaneous secretion or secretion
following citrus stimulation is diminished), and lower lip
biopsy (abnormal if focal sialoadenitis with a focus score > 1
is present).
If the patient fulfills only the criteria for KCS and not
xerostomia (or vice versa), we prefer to use the diagnosis
KCS (or xerostomia), which may correspond to the diagnosis “probable SS” or “incipient SS.”
Fox et al (1) state that their criteria for KCS require
abnormal results on at least 2 of 3 objective tests. This is
consistent with the Copenhagen criteria. While the normal
and abnormal values for the Schirmer-I test, break-up time,
and van Bijsterveld score are stated in the Copenhagen
Arthritis and Rheumatism Vol. 30, No. 8 (August 1987)
Figure 2. Objective tests used for the diagnosis of keratoconjunctivitis sicca and xerostomia, according to the Copenhagen criteria.
criteria, abnormal values for only the Schirmer-I test are
given by Fox et al. Although the rose bengal and fluorescein
staining measure different corneal abnormalities, these 2
tests are given equal value, in contrast to the Copenhagen
It is surprising that, in their evaluation for xerostomia, Fox et a1 include “symptomatic xerostomia,” since
they state that the diagnoses of KCS and xerostomia should
be based on objective findings only. Their objective test for
xerostomia is measurement of the spontaneous and stimulated saliva flow rates by use of the Lashley cup, and they
thus examine the functional capacity of I parotid gland only.
Reduced salivation from 1 or 2 of the large salivary glands
does not necessarily reflect similar malfunction of other
salivary glands; consequently, most SS centers prefer measurement of the whole saliva secretion rate.
Fox et a1 include the histopathologic findings in labial
minor salivary glands as a separate criterion for SS. The
xerostomia component of SS thus makes up half of the
proposed inclusion criteria, i.e., 2 of 4, whereas the KCS
component counts only one-fourth. To us, there is nothing to
suggest that the objective oral abnormalities should carry
more weight as inclusion criteria than the objective ophthalmologic abnormalities. Furthermore, recent studies (3,4)
have indicated that the focal sialoadenitis that is characteristic of SS is probably not so specific for the diagnosis of SS.
Also, according to Fox et al, patients who refuse a lower lip
biopsy cannot fulfill the proposed criteria for definite SS.
Taking all these arguments into consideration, we think that
the histologic labial salivary gland examination should be
included as 1 of other objective tests for xerostomia, as in
the Copenhagen criteria.
For the diagnosis of definite SS, Fox et a1 propose
that only patients with serologic abnormalities (positive
rheumatoid factor at titers 2 1: 160, positive antinuclear
antibodies at titers ?1:?160, or positive anti-SS-A or
anti-SS-B antibodies) be included. To our knowledge, no
data have shown that these autoantibodies correlate to
prognosis of the disease or to outcome of treatment. The
autoantibodies are not specific for SS; they are found in
patients with other rheumatic disorders and in normal persons as well. Furthermore, not all the autoantibodies mentioned can be examined reliably throughout the world. At the
present time, therefore, we find that examination for
autoantibodies should not be included as a criterion for SS.
Although the distinction between primary SS and
secondary SS is discussed by Fox et al, the proposed criteria
do not separate the 2. Such a distinction was agreed upon by
90% of the participants at the First International Seminar on
Sjogren’s Syndrome (5). Because primary and secondary SS
are separate entities, it is important that the diagnostic
criteria for SS clearly distinguish between the 2 disorders.
When new diagnostic criteria for a disease are proposed, they should be compared with already existing criteria. The proposed criteria for SS have not been compared
with the Copenhagen criteria, the Japanese criteria (6), or
the Greek criteria (7), which all are in use. We propose,
therefore, that the established sets of criteria for SS be
compared in multicenter studies.
Rolf Manthorpe, MD
University of Lund
Malmo, Sweden
Peter Oxholm, MD
Jan Ulrik Prause, MD
Morten Schiadt, DDS
University of Copenhagen
Copenhagen, Denmark
Fox RI, Robinson CA, Curd JG, Kozin F, Howell FV: Sjogren’s
syndrome: proposed criteria for classification. Arthritis Rheum
29~577-585, 1986
Manthorpe R, Frost-Larsen K, Isager H, Prause J U : Sjogren’s
syndrome: a review with emphasis on immunological features.
Allergy 36:139-153. 1981
3. SyjGen S: Salivary glands in rheumatoid arthritis (thesis).
Kuopio University, Finland, 1982
4. Lindahl G, Hedfors E: Focal lymphocytic infiltrates of salivary
glands are not confined to Sjogren’s syndrome. Scand J
Rheumatol (suppl) 6152-55, 1986
5. Prause JU, Manthorpe R, Oxholm P, Schifidt M: Definition and
criteria for Sjogren’s syndrome used by the contributors to the
First International Seminar on Sjogren’s syndrome: 1986. Scand
J Rheumatol (suppl) 61:17-18, 1986
6. Homma M, Tojo T, Akizuki M, Yamagata H: Criteria for Sjogren’s
syndrome in Japan. Scand J Rheumatol (suppl) 61:26-27, 1986
7. Skopouli FN, Drosos AA, Papaioannou T, Moutsopoulos HM:
Preliminary diagnostic criteria for Sjogren’s syndrome. Scand J
Rheumatol (suppl) 61:22-25, 1986
To the Editor:
As stated in their letter, Manthorpe and coworkers
agree that there is a need to carefully define the criteria for
Sjogren’s syndrome (SS). Important differences between our
proposed criteria (1) and their criteria (2) for definite SS
include: (a) our requirement of objective signs of keratoconjunctivitis sicca and xerostomia, (b) biopsy-proven salivary
gland infiltrates, (c) presence of autoantibodies, and (d) our
failure to initially separate primary SS (SS-1)from secondary
ss (SS-2).
Our aim was to identify a subset of patients who had
sicca symptoms with objective evidence of salivary gland
destruction due to a systemic autoimmune disease process.
Rose bengal conjunctival staining and parotid flow rate
measurements were used. Abnormal findings on these rapid,
inexpensive tests provided objective evidence of the sicca
state. The detection of these physical signs helps the physician to evaluate the significance of the subjective complaints
of eye or mouth dryness, particularly in depressed patients.
We agree with Manthorpe and coworkers that other methods
of quantitating flow rates may yield important information to
satisfy this goal.
We included autoantibodies as a criterion because
their presence emphasizes that SS is a systemic autoimmune
disorder. Also, the presence of autoantibodies helps distinguish these patients from those in whom sicca symptoms
may be a consequence of aging (3-5). Furthermore, the
absence of particular autoantibodies suggests other diagnoses as a cause for salivary gland dysfunction, including
sarcoidosis or lymphoma (6,7). We recognize that autoantibody assays are not currently standardized, but we
Без категории
Размер файла
229 Кб
classificaton, proposed, syndromecomments, crieteria
Пожаловаться на содержимое документа