Dr. Samyak Gupta, Dr. Prabhat Nangia, Dr. Vinay Nangia
Dr. Samyak Gupta, Dr. Prabhat Nangia, Dr. Vinay Nangia
Suraj Eye Institute, Nagpur
Case Description
A female, 50 years of age, came to our institute for burning sensation associated with pricking sensation, photophobia and severe dryness in both eyes (OU). Her visual acuity was 6/24, N18 in the right eye (OD) and 6/12, N10 in the left eye (OS). Anterior segment examination showed conjunctival congestion in OU with meibomian gland dysfunction. There was nebular grade corneal epithelial scarring. Tear film appeared to be slightly low. She had severe punctate epithelial erosions in both eyes. Intra-ocular pressure was 14 mmHg in right eye and 18 mmHg in left eye. Fundus evaluation of both eyes was within normal limits. She was a known case of rheumatoid arthritis on treatment since 25 years.
Her schirmer values at 5 min were 3 mm in the right eye and 4 mm in the left eye. Her fluorescein clearance test (FCT) values in OD were 0 mm in 1 minute after 10 minutes of fluorescein staining, 2 mm in 1 minute after 20 minutes of staining and 1 mm in 1 minute after nasal stimulation, 30 minutes after staining. FCT values in OS were 1 mm in 1 minute after 10 minutes of fluorescein staining, 1 mm in 1 minute after 20 minutes of staining and 1 mm in 1 minute after nasal stimulation, 30 minutes after staining. FCT values of both eyes suggested aqueous tear deficiency with no reflex tearing. Meibography showed 20 percent meibomian gland loss in both eyes.
Figure 1(b) – Anterior segment photograph of left eye under diffuse illumination showing an irregular light reflex (red arrows) with conjunctival congestion (yellow arrow)
Figure 2(b) – Left eye anterior segment photograph under cobalt blue illumination with Wratten yellow filter showing extensive punctate epithelial erosions (red arrows)
Figure 6(b) – Left eye upper lid meibography image showing areas of meibomian gland dropouts (demarcated with dashed red line) and dilated meibomian glands (yellow arrow)
Discussion
| Sjogren syndrome is a chronic inflammatory disorder of autoimmune origin. It primarily causes lymphocytic infiltration of the lacrimal and salivary glands resulting in the classic sicca complex characterized by dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia). Environmental triggers (eg. Exposure to Epstein Barr virus) trigger inflammation in genetically predisposed individuals. This inflammation causes cellular necrosis or apoptosis with expression of Sjogren SS-A protein on the glandular cell surface. This causes an upregulation of major histocompatibility antigens and adhesive molecules resulting in production of autoantibodies and activation of lymphocytes within the glandular micro-environment. These lymphocytes act on glandular epithelial cells and cause decreased secretion by residual glandular acini due to decreased neural innervation. Lacrimal gland dysfunction in Sjogren syndrome results in aqueous tear deficiency. Dry eye disease in Sjogren is also associated with decreased meibomian gland expressibility with a deficient lipid layer. Treatment options include short course of topical corticosteroids to control inflammation, long term preservative free lubricating eye drops with lubricating ointment/gel and steroid sparing agents such as cyclosporine 0.05%. Rheumatologist opinion is required for systemic control of disease and to prevent exacerbation of symptoms. Sjogren syndrome is more common in middle aged women and can occur in isolation (Primary Sjogren) or in association with other rheumatic diseases(Secondary Sjogren). Rheumatoid arthritis is the most common connective tissue disease occurring in association with Sjogren syndrome. Our patient was a known case of rheumatoid arthritis under treatment. Her Rheumatoid factor (RA factor) levels at presentation were 256 IU/ml (normal: <15 IU/ml) with raised ESR and CRP suggesting active inflammation. Her anterior segment examination showed several punctate erosions in both eyes (red arrows in Fig.1 and Fig. 2) suggestive of severe dry eye disease. Her Schirmer and FCT values also showed aqueous tear deficiency with absence of reflex tearing (Fig.3-5). Meibography showed loss of meibomian glands(Fig.6 and Fig.7). Given her presentation and absence of reflex tearing, she was initially advised a short course of steroids with lubricants in both eyes for immediate symptomatic relief followed by thermal punctal cautery in both eyes to facilitate tear retention and partial to complete resolution of symptoms in the long term. She was also advised to be under the care of a rheumatologist. |
ReadWise
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- Shih KC, Lun CN, Jhanji V, Thong BY, Tong L. Systematic review of randomized controlled trials in the treatment of dry eye disease in Sjogren syndrome. Journal of inflammation. 2017 Dec;14:1-https://link.springer.com/article/10.1186/s12950-017-0174-3
- Kuo MT, Fang PC, Chao TL, Chen A, Lai YH, Huang YT, Tseng CY. Tear proteomics approach to monitoring Sjögren syndrome or dry eye disease. International journal of molecular sciences. 2019 Apr 19;20(8):1932. https://doi.org/10.3390/ijms20081932
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Correspondence
Dr Prabhat Nangia
DNB, FICO, FMRF, FAICO
Consultant
Department of Cornea and Ocular surface
Suraj Eye Institute
Email – education@surajeye.org
QuizWise
Q1: Sjogren syndrome primarily affects which tissue?
A) Cornea
B) Retina
C) Exocrine glands
D) Uvea
Q2: Most specific test for diagnosing Sjogren syndrome:
A) Salivary gland biopsy
B) Schirmer
C) PCR
D) All of the above
Q3: Not included in the ACR-EULAR classification criteria for primary Sjogren syndrome:
A) Ocular surface staining > 5 in atleast one eye
B) Anti-SSA/Anti-Ro positive
C) Raised RA factor
D) Schirmer’s test < 5 mm/5 min in at least one eye
Q4: Secondary Sjogren syndrome is associated with which disease:
A) Rheumatoid arthritis
B) Systemic sclerosis
C) Systemic lupus erythematosus
D) All of the above