Indian Journal of Ophthalmology

: 1983  |  Volume : 31  |  Issue : 2  |  Page : 81--83

Sjogren's syndrome in association with retinitis pigmentosa-report of an unusual case

SC Tiwari1, Kapil Dhingra1, KM Khan1, AN Malviya1, Manoj Singh2, DK Gehlot1,  
1 Department of Medicine. Clinical Immunology Division, All India Institute of Medical Sciences, New Delhi-110 029, India
2 Department of Pathology, and Dr. Rajendra Prasad Centre for Ophthalmic Sciences. All India Institute of Medical Sciences, New Delhi-110 029, India

Correspondence Address:
S C Tiwari
Department of Medicine, All India Institute of Medical Sciences. New Delhi-110 029

How to cite this article:
Tiwari S C, Dhingra K, Khan K M, Malviya A N, Singh M, Gehlot D K. Sjogren's syndrome in association with retinitis pigmentosa-report of an unusual case.Indian J Ophthalmol 1983;31:81-83

How to cite this URL:
Tiwari S C, Dhingra K, Khan K M, Malviya A N, Singh M, Gehlot D K. Sjogren's syndrome in association with retinitis pigmentosa-report of an unusual case. Indian J Ophthalmol [serial online] 1983 [cited 2020 Oct 25 ];31:81-83
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Full Text

Sjogren's syndrome (SS) is a chronic infla­mmatory autoimmune disorder characterized by at least two of a triad of manifestations viz. Xerostomia, keratoconjunetivitis sicca and a connective tissue disease. The lymphocyte mediated destruction of exocrine glands leads to diminished or absent glandular secretions and mucosal dryness, which in the eyes leads to destruction of corneal and bulbar conjun­ctival epithelium.[1] We have recently come across a case of SS with retinitis pigmentosa. To our knowledge this is the first reported association of SS and retinitis pigmentosa in world literature.


Mrs. V. K.. a 38-year old lady presented to us with bilateral parotid enlargement with dryness of eyes and mouth of seven years dura­tion, purpuric spots over the lower extremities for five months and intermittent fever for one and a half month prior to admission. She had undergone Right Superficial Parotidec­tomy five years earlier which showed changes of chronic sialadenitis on histopathological examination. A skin biopsy done five months prior to admission had shown evidence of vasculitis. She had been suffering from night blindness since childhood and had progressive diminution of vision for last seven years. On examination, she had enlargement of left parotid gland, angular stornatitis, glossitis and multiple purpuric spots were present over both lower extremities. Examination of the left eye revealed old keratic precipitates, posterior synechiae and posterior polar cata­ract. Fundus examination revealed changes typical of retinitis pigmentosa in both eyes. Schirmer's test was zero min in either eye (against a normal of more than 15 mm in 5 minutes).

Investigations revealed a platelet count 150,000/cmm; positive rheumatoid factor (at a dilution of 1:80) and positive ANF (speckled appearance) at a dilution of 1:160. Serum CRP was 20 µg/ml and C4-6 mg°„ serum electrophoresis revealed a diffuse increase in gamma region. Quantitative estimation of Immunoglobulins revealed a marked increase in 1gM levels (281 mg % at a dilution of 1:4)­X-rays of the hands were normal. Biopsy of labial salivary glands [Figure 1] was consistent with the diagnosis of SS.


Pigmentary retinal degeneration has been seen in a number of diseases considered to have an immunological basis e.g. Behcet's disease, polyarteritis nodosa, rheumatoid arthritis. multiple sclerosis and Harada's disease.[2],[3]

Antigenicity of retinal neuroepithelium has been well documented. It is known that the cornea, uvea, pigment epithelium of retina and the lens share some common antigens. Uveitogenic nature of retina has been demo­nstrated in various animal experiments by different workers[5]. Further work has demon­strated that is is the photoreceptors in the retina which are antigenic[6],[7]. Various immun­oglobulin abnormalities have been described in retinitis pigmentosa. These include rheuma­toid factor positivity in the serum in as many as 38% cases and significantly elevated IgM level in the serum[8],[9]. Some initial success has been reported in treating cases of retinitis pigmentosa with D-penicillamine[10]. The utility of D-Penicillamine in treating diseases like rheumatoid arthritis and PSS has been well established for sometime now.

These observations have raised interesting possibilities:

a) Since it is known that autologous 1gM can lyse neuraminidase treated host cell, it has been suggested that naturally occurring cytotoxic antibodies of this class could be important in the process of ageing[11]. How­ever. selective degeneration of visual cells is not a feature of old age. therefore it is diffi­cult to regard retinitis pigmentosa as a product of premature senility, hence the role of raised IgM levels in retinitis pigmentosa. remains unclear.

(b) Since the production of IgM is geneti­cally controlled and a large production of cases of retinitis pigmentosa are hereditary in origin, it is possible that individuals with raised IgM are more susceptible to retinal degeneration.

(c) Raised IgM in retinitis pigmentosa may be suggestive of an occult infection espe­cially in view of the suggestion that retinitis pigmentosa may be caused by a slow virus infection[12].

Thus, there is great deal of evidence to suggest that retinitis pigmentosa may be a disease of on autoimmune etiology. Whether the coexistence of SS with raised IgM level and retinitis pigmentosa in this patient can be attributed to a common etiology or a cause and effect relationship or is merely an incidental finding is purely conjectural at this stage and needs further elucidation.


A rare case of Sjoeren's Syndrome in association with retinitis pigmentosa is reported.


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