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   Table of Contents      
ARTICLE
Year : 1968  |  Volume : 16  |  Issue : 1  |  Page : 19-23

Sjogren's Syndrome - A Clinicopathological Study


Department of Ophthalmology, Jawaharlal Institute of Post-graduate Medical Education and Research, Pondicherry, India

Date of Web Publication22-Dec-2007

Correspondence Address:
N N Sood
Department of Ophthalmology, Jawaharlal Institute of Post-graduate Medical Education and Research, Pondicherry
India
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Source of Support: None, Conflict of Interest: None


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How to cite this article:
Sood N N. Sjogren's Syndrome - A Clinicopathological Study. Indian J Ophthalmol 1968;16:19-23

How to cite this URL:
Sood N N. Sjogren's Syndrome - A Clinicopathological Study. Indian J Ophthalmol [serial online] 1968 [cited 2020 Apr 7];16:19-23. Available from: http://www.ijo.in/text.asp?1968/16/1/19/37488

Table 1

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Table 1

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Sjogren's syndrome is a chronic systemic disorder of idiopathic origin characterized by three major compo­nents keratoconjunctivitis sicca, xe­rostomia and polyarthritis of rheu­matoid origin occurring predomi­nantly in women over forty years. The first person to associate dryness of mouth and conjunctiva due to diminution of salivary and lacrimal gland secretion was FUCHS [4] . The syndrome in its present form was es­tablished by SJOGREN [5] in 1933.

The terms Sjogren's syndrome and keratoconjunctivitis sicca are often used interchangebly, so that diagnos­tic criteria suggested by BUNIM, BUCHNAN, WERTLAKE, SOKO­LOFF, BLOCK, BEEK AND ALEP­PA [2] were employed. If two out of the three major components viz. kera­toconjunctivitis sicca, xerostomia and rheumatoid arthritis are present a diagnosis of Sjogren's syndrome can be made. For each one of these there is an objective and a reliable diagnostic criterion.


  Materials and methods Top


During the years 1964 - 1966 at the General Hospital, Pondicherry and at JIPMER Hospital, fifteen cases in whom a diagnosis of Sjogren's syn­drome and keratoconjunctivitis sicca was made, were selected for study. A detailed ocular and general exami­nation was carried out for each case.

The diagnosis of rheumatoid arth­ritis was made, based on the diag­nostic criteria of the AMERICAN REUMATIC ASSOCIATION. [1]

Schirmer's test was done in each case before examination with light and repeated at a second sitting. A presence of punctate corneal stains on the slit lamp with fluorescein or rose bengal was required for the diagnosis of keratoconjunctivitis Sicca.

Sialogram was attempted in three cases. A present or a past history of swelling of parotids and submaxillary glands was enquired and so was the history of dryness of mouth but sub­jective history of dryness of mouth was not considered to be a reliable major criterion for diagnosis. Ob­jective evidence of swelling or atro­phy of parotid or submaxillary glands was taken as evidence of salivary gland involvement.

Blood was examined for blood counts, E.S.R., serum proteins (Total and A/G Ratio) and sheep cell agglu­tination. In three cases gastric func­tion test for achylia gastrica were done.

Lacrimal gland biopsy was done in seven cases.


  Presentation of data and discussion Top


Bunim [2] et al (1964) suggested that cases of Sjogren's syndrome may fall into 5 groups.

Group A:- includes those cases with classical rheumatoid arth­ritis.

Group B:- Consists of patients with probable rheumatoid arth­ritis.

Group C:- Cases associated with con­nective tissue disorders like scleroderma, dissemi­nated lupus erythematosus and polyarteritis nodosa.

Group D:- Cases associated with po­lymyositis.

Group E:- Cases of keratoconjuncti­vitis sicca and xerostomia unassociated with rheuma­toid arthritis or any con­nective tissue or muscle disorder. The patients in Group E have a higher concentration of serum gamma globulins and a greater frequency of a number of diverse circu­lating antibodies to tissue and subcellular compo­nents.

Out of the fifteen cases, 3 fell in Group A, five in Group B and seven in Group E. It is the latter group which is the centre of controversy. VANSELOW, DODSON, ANGELL AND DUFF [8] reviewing the charts of cases of keratoconjunctivitis sicca at Michigan Medical Centre found the majority of cases to be without any symptoms or signs of salivary glands involvement, rheumatoid arth­ritis or connective tissue disease. Fur­ther the keratoconjunctivitis sicca is the end result of a variety of local ocular and general diseases like tra­choma, radiation to the eyes and sar­coidosis etc. So this sign may not be the hall mark of Sjogren's syn­drome alone. No evidence of serolo­gical studies on these cases with kera­toconjunctivitis sicca is presented by these authors.

BUNIM [2] et al (1964) on the other hand showed that cases with kera­toconjunctivitis sicca and xerostomia show a higher concentration of gamma globulin (usually greater than 2.0 G/100 ml. Analytical ultracentrifuga­tion showed that the major increase in protein concentration of these sera was found in the 7S fraction. Some sera disclosed, in addition, a modest increase in 19S fraction. Further Bentovite flocculation test (which de­tects the rheumatoid factor capable of reacting with human gamma glo­bulins) was positive in 74%. The sheep cell agglutination test which detects the rheumatoid factor capa­ble of reacting with rabbit gamma globulin was positive in 96%. of the 23 cases with sicca complex alone (Group E). These findings compar­ed with the 100% and 72% respect­ively for the Bentonite flocculation test and sheep cell agglutination test for cases with Group A Sjogren's syn­drome. This evidence lends conside­rable support to the fact that in cases of rheumatoid arthritis and in the sicca complex, similar alterations occur in the gamma globulin mole­cule making them antigenic within the host and leading to the produc­tion of rheumatoid or antigamma globulin factors.

Another evidence for this is from the passive cutaneous anaphylaxis test. If the diluted serum is injected intradermally into the skin of guinea pigs and after a latent period of seve­ral hours, the animal is challenged intravenously with Evans blue follow­ed by a suspension of tissue extract (calf thymus extract) at sites of anti­gen antibody interaction, there is in­creased capillary permeability leading to extravasation of dye. Such a test was positive in 14% of Group A pati­ents and 67% of Group E patients in Bunim's series.

In our series there were seven cases with keratoconjunctivitis sicca. The details of the physical and laboratory findings are shown in [Table - 1].

The cases of sicca complex showed the presence of other symptoms like dryness of mouth, swelling of salivary glands, together with an increase of serum globulin (over 2.OG/1000 ml.) and a positive sheep cell agglutina­tion test (Rose Waaler Test).

Taking all the evidence together, these seven cases of sicca complex would fit in with the broader concept of Sjogren's syndrome as suggested by Bunim.

It would be of interest to note as a parallel that eye signs (Band Kera­topathy, Iridocyclitis, complicated cataracts) may occur in Still's dis­ease without any rheumatoid arth­ritis. Such cases show again the changes in the blood, in the form of raised serum proteins, changed albu­min globulin ratio and a positive sheep cell agglutination (SOOD AND AGARWAL. [5] )

How can the occurrence of rheu­matoid factor in Group E patients be explained? Presence of rheumatoid factor may herald the future deve­lopment of rheumatoid arthritis or possibly the sicca complex may be an alternate expression of rheuma­toid disease without joint involve­ment, or in the rheumatoid arthritis and in the sicca complex, similar al­terations occur in gamma globulin molecules, making them antigenic within the host and leading to the production of rheumatoid or anti­gamma globulin factors.

The typical ocular changes in Sjo­gren's syndrome consist of filamen­tary keratitis. In fact many of these patients may be treated for months for chronic conjunctivitis without re­alizing the true significance of the ocular changes.

In one of the cases belonging to Group A - the ocular changes per­sisted for nearly two years before the polyarthritis of classical rheuma­toid type set in (Sood) [7] The signifi­cance of ocular changes was not re­cognized until a few months after the onset of rheumatoid arthritis.

Filaments are visible in the lower fornix and punctate corneal stains are visible on the cornea. Recurrent ulceration of the cornea may occur in some cases (l0% of cases VANSE­-LOW [8] et al,). Two of the cases in the present series showed corneal ulceration. Serious corneal compli­cations are rare. Serious complica­tions like pannus (DUKE ELDER AND GOLDSMITH, [3] ), and pannus and leukomatous corneal opacities (SOOD [7] ) have, however, been des­cribed.

Histopathological examination of the lacrimal gland shows infiltration by lymphocytes, plasma cells and later characterized by acinar atrophy and dilatation of ducts.

Lacrimal gland biopsy was done in seven cases, one in Group A Sjo­gren's syndrome and six in Group E. In one of the latter cases the lacri­mal gland was essentially normal [Figure - 1] but all the other cases show­ed dense infiltration by lymphocytes and plasma cells [Figure - 2]. Acinar atrophy was noticeable in three cases.


  Summary Top


1) Fifteen cases of Sjogren's syn­drome are described.

2) Seven of these cases presenting only with keratoconjunctivitis sicca showed an increase of serum globulin. Rose Waaler test was positive in a titre of 1 : 32 or more in six cases.

3) It is suggested that serum studies should be carried out on patients presenting with keratoconjuneti­vitis sicca where no local ocular cause is discernible.


  Acknowledgment Top


I am grateful to Dr. A. Lesigneur, M. D., Medical Superintendent, General Hospital, Pondicherry and Dr. D. J. Reddy, M. D., Principal, Jawaharlal Institute of Post­graduate Medical Education and Research. Pondicherry for facilities provided to use the Hospital Records.

 
  References Top

1.
AMERICAN RHFUMATIC ASSOCI­ATION) (1958) Revision of Diagnostic Criteria for Rheumatoid Arthritis. Arthritis Rheum. 2: 16, 1959.  Back to cited text no. 1
    
2.
BUNIM, J. J., BUCHMAN, W. W., WERTLAKE, P. T., SOKOLOFF, L., BLOCK, K. J., BECK, J. S., ALEPPA, F. B.: Ann. Int. Med. 61: 509. 1964.  Back to cited text no. 2
    
3.
DUKE ELDER, W. S., GOLDSMITH, A. J. B.: (1951) Recent Advances in Ophthalmology, J & A Churchill, Lon­don.  Back to cited text no. 3
    
4.
FUCHS: Cited by Duke Elder, S: System of Ophthalmology. Disease of the Outer Eye, Vol. VIII, Part I, p. 131. Henry Kimpton, London, 1965.  Back to cited text no. 4
    
5.
SJOGREN, II.; (1933) Acta Ophthal. Suppl. 2, cited by Duke Elder.  Back to cited text no. 5
    
6.
SOOD, N. N., AGARWAL, T. P.: (1966) Orient. Arch. Ophthal. 4, 300.  Back to cited text no. 6
    
7.
SOOD, N. N.: - Personal observa­tions - Under publication.  Back to cited text no. 7
    
8.
VANSELOW, N. A., DODSON, V. N., ANGELL, D. C., DUFF, I. F.: (1963) Ann. Int. Med. 58: 124.  Back to cited text no. 8
    


    Figures

  [Figure - 1], [Figure - 2]
 
 
    Tables

  [Table - 1]



 

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