Sjogren's Syndrome by iasiatube


logical base or framework for the social and be-       and well-being, or the lack of it, in any society.
havioral sciences.                                     It is in health care that many of the unsolved
   In any case the realities of the interaction of     problems of such a biological society as has been
the closed biosphere with the human population         discussed come into the sharpest focus. If there
on earth are becoming increasingly evident for all     is to be a better understanding of these problems
to see. For the first time in human history tech-      and if better solutions are to be found, sophisti-
nologic progress has made it possible for the world    cated input will be needed from both medicine
population to increase enough to make the pinch        and society-an input which neither is yet pre-
of the closed biosphere felt in terms such as finite   pared to give.
food and energy resources and an unhealthy                                                      -MSMW
technological pollution of the land, sea and air
environment. In a very real sense the social, eco-
nomic, political and legislative problems engen-
dered by the effects of modem technologic pro-
gress-and inadequately dealt with by sociology,
economics and political science at present-can
be viewed as biological problems and studied           Sjogren's Syndrome
within a biological framework. It seems reasonable
to assume that just as human nature and human          IN 1933, Henrik Sj6gren, a Swedish ophthalmolo-
behavior are fundamentally biological, so are the      gist, published a classic monograph detailing
social, economic and political systems of man-         observations on keratoconjunctivitis sicca, a con-
kind. It is suggested that if this can be recognized   dition characterized by dryness of the eyes. Sjo-
and accepted it begins to be possible to think of      gren's study clearly established that the ocular
the problems of mankind on earth in different          findings of this disorder were but a local manifes-
terms. In such a context as this, health and well-     tation of a more generalized disease process in
being take on new meaning and the principle of         which arthritis, anemia and other systemic fea-
adaptation of man and his environment for health       tures were present. The disease also included de-
and well-being takes on new dimensions. Man            creased secretion of the salivary glands and of
has developed considerable capability to influence     the mucosal glands of the mouth and upper respi-
his environment both favorably and unfavorably,        ratory tract, and parotid enlargement. Subse-
and he has a considerably lesser-but still signifi-    quently termed Sjogren's syndrome, the condition
cant-capability to influence his own nature and        is now recognized as a multisystem disorder inti-
behavior both individually and as a species. Both      mately related to the various connective tissue
should be improved, but it is the latter capability    diseases.
which now sorely needs strengthening. This will           As indicated by Talal in the Medical Staff Con-
 almost certainly require new and more soundly         ference on Sj6gren's syndrome appearing elsewhere
biologically-based approaches within the social        in this issue, the disease is by no means rare; in-
 and behavioral sciences.                              deed, it is the second most frequent collagen dis-
    But still more will be necessary. Human be-        order, surpassed in prevalence only by rheumatoid
havior in adapting to the problems of the closed       arthritis. The diagnosis is, however, frequently
earth biosphere cannot be separated from ethics,       overlooked. Whaley et all recently observed that
from some consideration of what is right and what      Sj6gren's syndrome had been unsuspected before
is wrong. There is no provision in natural law for     referral in all of 94 patients in whom it coexisted
right or wrong. If health and well-being, and im-      with rheumatoid arthritis, who were seen in a
proving something called the quality of life are to    diagnostic center in Glasgow. Most of the referrals
 be accepted as principal goals of mankind on          had been made by consultants, physicians who
 earth, then it may be possible, even necessary, to    should probably have been conversant with the
 construct an ethic of right and wrong around          disorder.
 these goals and do this within the framework of           Although an occasional person may be asympto-
 the natural law within which mankind must func-       matic in spite of significant abnormalities of
 tion within the earth ecosystem.                      lacrimal and salivary glands, most persons with
    There is much to be done. It is important to       Sj6gren's syndrome complain of discomfort and
 note that health care is at the interface of health   dryness of the eyes and mouth, and many note

     68     JANUARY 1975 * 122 * 1

abnormalities of smell and taste. That such symp-        gation for an association with other collagen dis-
toms may be overlooked or considered trivial in          eases is that recently embarked upon by Alarcon-
comparison with the serious joint disease or vis-        Segovia and coworkers.6-8 They looked first for an
ceral involvement of their associated connective         association with primary biliary cirrhosis because
tissue disorder is understandable and may in part        it, like Sj6gren's syndrome, has elements suggest-
explain the frequent nonrecognition of the ocular        ing an immunologic imbalance, and because its
manifestations of this disease. Anderson et al2          coexistence with Sjogren's syndrome had been ob-
have demonstrated that these manifestations are          served. In all of 14 consecutive patients with
sufficiently distinctive that the diagnosis can be       biopsy-proved primary biliary cirrhosis, the cri-
made with a high degree of accuracy by physicians        teria for the diagnosis of Sj6gren's syndrome were
who have no ophthalmologic training but are              satisfied.6 Similar investigations have now been
aware of the symptoms and physical findings.             reported in unselected series of patients with
    Once the diagnosis is suspected, it may be cor-      scleroderma7 and systemic lupus erythematosus.5
roborated by such procedures as the Schirmer test,       Surprisingly, in these series also, both clinical and
ocular staining and measurement of tear lysozyme,        pathologic evidence of Sj6gren's syndrome was
as well as by the techniques that assess salivary        found in almost every patient examined. This fre-
gland function which are described by Daniels in         quency far exceeds that observed in rheumatoid
the Medical Staff Conference. Diagnostic confir-         arthritis, albeit the disease state in patients with
mation is best accomplished by microscopic ex-           systemic lupus erythematosus or scleroderma
amination of salivary gland tissue. The mucous           tends to be milder and is often subclinical. There
glands of the lip are almost always involved in the      is no clear explanation for the remarkable fre-
polyglandular inflammation that characterizes Sj6-       quency with which these disorders and Sj6gren's
gren's syndrome; the labial mucosa is a preferred        syndrome coexist.
site for biopsy: here, removal of tissue causes             Most probably the clarification of the reasons
minimal discomfort and entails minimal risk. The         for this striking association, and of other puzzling
degree of lymphocytic infiltration is quantifiable,      aspects of these disorders such as the heightened
and has been shown to correlate well with clinical       incidence of neoplasms and the immunologic aber-
manifestations and objective measures of disease         rations must await better understanding of the
severity.3'4 Since this technique has come into use,     causes and pathogenesis of the various connective
biopsy of the parotid and submandibular glands           tissue diseases. Interdisciplinary investigative ac-
is seldom required.                                      tivities of the type described by Talal and other
   In addition to broadening our understanding of        members of the study group in this Medical Stafl
the nature and spectrum of Sjoigren's syndrome,          Conference can be expected to yield information
early diagnosis has practical value. The institution     of the kind that is required before such questions
of simple measures such as artificial tears and          can be answered.
careful dental prophylaxis often averts serious
ocular complications and preserves dental integ-                           MARTIN A. SHEARN, MD
                                                                               Associate Clinical Professor of Medicine
rity. Establishing the diagnosis alerts the physician                          University of California, San Francisco
                                                                               Director of Medical Education
to anticipate some of the potential complications                              Kaiser-Permanente Medical Center, Oakland
and may enable him to deal with them earlier and
hence more effectively. Among these are renal
                                                           1. Whaley K, Williamson J, Chisholm DM, et al: Siogren's syn-
tubular acidosis, chronic interstitial disease of kid-   drome-I. Sicca components. Q J Med, NS 42:279-304, 1973
ney and lung, pancreatitis, vasculitis, recurrent          2. Anderson JA, Whaley K, Williamson J, et al: A statistical
                                                         aid to the diagnosis of keratoconjunctivitis sicca. Q J Med, NS
respiratory infections, hyperviscosity syndrome          41:175-189, 1972
                                                           3. Greenspan JS, Daniels TE, Talal N, et al: The histopathology
and the development of lymphoma and macro-               of Sjogren's syndrome in labial salivary gland biopsies. Oral Surg
                                                         37:217-229, 1974
globulinemia.5                                             4. Daniels TE, Silverman S Jr, Michalski JP, et al: The oral
   The association of Sj6gren's syndrome with            component of Sjogren's syndrome. (Submitted for publication)
                                                           5. Shearn MA: Sjogren's Syndrome. Philadelphia, W. B. Saun-
rheumatoid arthritis is well known. When large           ders Co., 1971
                                                           6. Alarc6n-Segovia D, Diaz-Jouanen E, Fishbein E: Features
series of patients with rheumatoid arthritis have        of Sjogren's syndrome in primary biliary cirrhosis. Ann Intern
been screened for Sj6gren's syndrome, the co-            Med 79:31-36, 1973
                                                           7. Alarc6n-Segovia D, lbaiiez G, Hernandez-Ortiz J, et al:
existence was noted in a significant minority, and       Sjogren's syndrome in progressive systemic sclerosis (scleroderma).
                                                         Am J Med 57:78-85, 1974
varied directly with the severity and duration of the      8. Alarc6n-Segovia D, lbaiiez G, Velizquez-Forero F, et al: Sjd-
                                                         gren's syndrome in systemic lupus erythematosus; clinical and
rheumatoid condition. The first systematic investi-      subclinical manifestations. Ann Intern Med 81:577-583, 1974

                                                         THE WESTERN JOURNAL OF MEDICINE                         69

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