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 Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 26  |  Issue : 1  |  Page : 99-102

Primary Sjögren's syndrome without ocular involvement: A rare case report


Department of Oral Medicine and Radiology, Sri Aurobindo College of Dental Science, Indore, Madhya Pradesh, India

Date of Submission12-Aug-2014
Date of Acceptance22-Aug-2014
Date of Web Publication26-Sep-2014

Correspondence Address:
Priya Pande
Flat 305 Vikram Apartment, 91 Chandralok, Saket, Indore, Madhya Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-1363.141872

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   Abstract 

Sjögren's Syndrome (SS) is a chronic systemic autoimmune disorder, characterized by the lymphocytic infiltration of lacrimal and salivary glands, giving rise to dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia). Primary Sjögren's Syndrome commonly presents only with sicca manifestations; whereas, secondary Sjögren's syndrome occurs in connection with other autoimmune rheumatic diseases. Primary Sjögren's syndrome without ocular manifestation is rarely reported in the literature. Here we report a case of a 45-year-old female, who presented to us with complaints of dryness of mouth and dysphagia, without any ocular and systemic manifestations. On further evaluation she was diagnosed as a case of Primary Sjögren's syndrome. With this case report, we intend to emphasize the importance of an early diagnosis of this disorder, along with a brief review of various diagnostic criteria.

Keywords: Autoimmune disorder, sicca symptoms, sjogren′s syndrome


How to cite this article:
Phulambrikar T, Pande P, Singh S, Magar S. Primary Sjögren's syndrome without ocular involvement: A rare case report . J Indian Acad Oral Med Radiol 2014;26:99-102

How to cite this URL:
Phulambrikar T, Pande P, Singh S, Magar S. Primary Sjögren's syndrome without ocular involvement: A rare case report . J Indian Acad Oral Med Radiol [serial online] 2014 [cited 2020 Jan 22];26:99-102. Available from: http://www.jiaomr.in/text.asp?2014/26/1/99/141872


   Introduction Top


Sjögren's Syndrome (SS) is a chronic systemic autoimmune disorder, characterized by the lymphocytic infiltration of lacrimal and salivary glands, giving rise to dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia), respectively. The disease is usually diagnosed at around the fifth decade of life, with a female to male ratio of 9:1. [1] It is classified either as primary (pSS) when occurring alone or secondary (sSS) when associated with other autoimmune diseases. It is cardinal to make an early diagnosis of pSS as there is increased risk of developing life-threatening complications, such as, lymphoma, distal renal tubular acidosis, interstitial nephritis, and so on. [1]

In the last few decades various diagnostic criteria have been given, each having their own merits and demerits, the oldest one being the Copenhagen criteria (1975) and the recent one being the American College of Rheumatology criteria (ACR/SICCA 2012). The Copenhagen (1975) and Japanese-I criteria (1984) focused mainly on the objective ascertainment of the functional impairment of the salivary and lacrimal glands; [2] whereas, the Greek criteria (1986) emphasized on the role of focal sialadenitis. [2] The Californian criteria, for the first time, introduced the significance of auto antibodies like IgM-RF, ANA, anti-SSA or anti-SSB, and histopathology, as distinct items. [2]

To make a diagnosis of pSS, the Japanese-II criteria (1997) required focal sialadenitis only, or two among abnormal salivary gland sialography, eye tests, and positive anti-SSA and/or an anti-SSB antibody. [2] The Japanese-III criteria (2000) required at least two among the four following items shown in [Table 1], to make a diagnosis of pSS. These criteria have a specificity and sensitivity of 90.5 and 96.0%, respectively, which is the highest amongst all the other criteria. [3]
Table 1: The revised Japanese ministry of health criteria for the diagnosis of SS

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The American-European Consensus Group (AECG) criteria laid in 2000, absolutely required a positive lower lip biopsy and/or positive anti-SSA/SSB autoantibodies. It consisted of six different sets of criteria [Table 2] and the diagnosis of pSS was made when-(a) any four of the six criteria (had to include either IV or VI); or (b) any three of the four objective criteria (III, IV, V, VI) were positive. These criteria had a better specificity of 83.1% and sensitivity of 90.9%. [4]
Table 2: The American-European consensus group classification criteria for SS

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Although there have been various sets of diagnostic criteria, none has been endorsed by the American College of Rheumatology (ACR) or the European League Against Rheumatism (EULAR). Thus, in the year 2012, a collaboration of the ACR and Sjögren's International Collaborative Clinical Alliance (SICCA) had come up with the newer set of criteria called the ACR/SICCA-criteria. [5] It applies to individuals with signs/symptoms that may be suggestive of SS, and will be met in patients who have at least two of the three objective features [Table 3].
Table 3: The American college of rheumatology classification criteria for SS

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   Case Report Top


A 45-year-old female came to us with complaint of dryness of mouth along with dysphagia since one year; without any history of dry eyes, swelling of the salivary glands or any musculoskeletal complaints. She had a history of Barrett's esophagitis for the past two years and was on Pantoprazole 40 mg OD for the same, without any significant improvement. Her past medical, family, and personal histories were insignificant.

Her general and extraoral examinations were normal [Figure 1]. The intraoral examination showed dry lips with angular chelitis, an erythematous tongue [Figure 2], and dry buccal mucosa, with depapillated tongue [Figure 3]. The hard tissue examination showed multiple carious teeth. Salivary flow was scanty from the Stenson's ducts and Wharton's ducts on both the sides. Her ophthalmological examination, which included clinical consultation along with Schirmer-I Test and Rose Bengal Test, were normal. Her hematological, biochemical, and immunological parameters showed results as mentioned in [Table 4]. The radiological investigations included x-ray chest, which was normal. Ultrasonography of the parotid gland showed hyperechoic nodules in both parotids [Figure 4]. Minor salivary gland biopsy was taken from the lower lip, which showed lymphocytic infiltration [Figure 5].
Table 4: Hematological and serological reports of patient

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Figure 1: Extraoral view

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Figure 2: Dry lips, angular chelitis

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Figure 3: Depapillated tongue

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Figure 4: Parotid gland showing hyperechoic nodules

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Figure 5: Histopathology showing lymphocytic infiltration

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On the basis of the history, clinical examination, and laboratory evaluation, diagnosis of primary Sjögren's syndrome was made, which also satisfied the ACR 2012 criteria. The differential diagnosis included were diabetes mellitus, hypothyroidism, chronic hepatits C infection, sarcoidosis, HIV infection, anxiety neurosis, antipsychotic medications, anticholinergic drugs, and head and neck radiation therapy, which were very much ruled out.

She was treated with Pilocarpine 5 mg QID for three months, chewable Vitamin C lozenges, and artificial saliva 'Bioxtra' for oral dryness. She is under continuous follow up with 50 to 60% relief, without any systemic complications.


   Discussion Top


Sjögren's syndrome is a common systemic autoimmune disease, predominantly affecting females, as in our case. The prevalence of pSS in the general population has been estimated to be around 1 to 3%; [6] whereas, it has been infrequently described from India. Porkodi et al. diagnosed 36 patients out of 8000 patients with musculoskeletal complaints, over four-and-a-half years; this figure comes to about 0.0045%. [7] Misra et al. diagnosed 26 cases over a 10-year period. [8] This is far less compared to the prevalence reported in various Western literature. Lack of awareness of attending physicians, ophthalmologists, and dental surgeons may be the most important factors for less 'prevalence' of pSS.

Although sicca symptoms are the hallmarks of pSS, as a result of multiple organ involvement, variable clinical manifestations are present. The incidence of ocular involvement among the reported cases is 86.1%; [9] whereas, our patient did not have any ocular involvement. To the best of our knowledge there has been no case report published from India on pSS without ocular involvement, and very scarce data is present in the literature. It is reported that the enlargement of the major salivary glands occurs in 25-66% of pSS, [9] which was not manifested in our patient. Various immunological markers found in different studies from India and abroad have shown different results with RF (60%), ANA (65%), and anti-SSA, SSB antibodies. The sensitivity for Rheumatoid Factor and ANA for pSS is 72.3 and 72.8, respectively. [7] All these parameters were positive in our patient.

To assess disease activity and treatment efficacy, two indices have been developed: EULAR Sjögren's Syndrome Patients Reported Index (ESSPRI) and EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI). The ESSDAI and ESSPRI seem to be promising tools for the outcome assessment of patients with pSS.

Treatment of SS depends on the extent and severity of the clinical manifestations and is better instituted through a multidisciplinary approach. From a dentist's perspective, xerostomia is mainly due to decreased salivation and altered salivary composition, which results in dental caries, periodontal diseases of the teeth, and intraoral candidiasis. Fastidious dental care is required, including frequent dental examinations, along with fluoride application and anti-fungal drugs such as nystatin. In addition, clotrimazole lozenges, taken five times daily for 14 days, may also be used. Salivary substitutes, saliva replacement products, and sugar-free chewing gums may be effective for stimulating saliva secretion. Salivary secretion can be improved with drugs such as pilocarpine hydrochloride (5-10 mg TID) and civemeline hydrochloride (30 mg TID), which stimulate the muscarinic receptors. Dentures may not be suitable for patients with SS, as the tongue adheres to and dislodges the denture; an implant-supported denture may be considered. For musculoskeletal manifestations, hydroxychloroquine, methotrexate, cyclophosphamide, and corticosteroids may be used. Among the biologics, anti-TNF drugs (Infliximab and Etanercept) did not show any benefit. On the other hand Rituximab (anti CD-20) showed benefit in lymphoma management and was effective in overcoming the sicca manifestations. The role of the B-cell activating factor (BAFF) antagonist is under research. [10]


   Conclusion Top


This is a case report of pSS with xerostomia, without keratoconjunctivitis, which is a rarity, and hardly reported in literature. Therefore, we should keep a high index of suspicion in patients presenting with sicca symptoms as a delay in diagnosis can be detrimental and may lead to serious complications like non-Hodgkin's lymphoma. In spite of the limited clinical presentation of our patient the early diagnosis of this rare disorder was possible by using the latest ACR diagnostic criteria.



 
   References Top

1.Margaix-Muñoz M, Bagán JV, Poveda R, Jiménez Y, Sarrión G. Sjögren's syndrome of the oral cavity. Review and update. Med Oral Patol Oral Cir Bucal 2009;14:E325-30.  Back to cited text no. 1
    
2.Manthorpe R. New criteria for diagnosing Sjögren's syndrome: A step forward? - or... Scand J Rheumatol Suppl2001;115:14-22.  Back to cited text no. 2
    
3.Tsuboi H, Hagiwara S, Asashima H, Umehara H, Kawakami A, Nakamura H, et al. Validation of different sets of criteria for the diagnosis of Sjögren's syndrome in Japanese patients. Mod Rheumatol 2013;23:219-25.  Back to cited text no. 3
    
4.Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, et al.; European Study Group on Classification Criteria for Sjögren's Syndrome. Classification criteria for Sjögren's syndrome: A revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 2002;61:554-8.  Back to cited text no. 4
    
5.Shiboski SC, Shiboski CH, Criswell L, Baer A, Challacombe S, Lanfranchi H, et al.; Sjögren's International Collaborative Clinical Alliance (SICCA) Research Groups.American College of Rheumatology classification criteria for Sjögren'ssyndrome: A data-driven, expert consensus approach in the Sjögren's International Collaborative Clinical Alliance cohort.Arthritis Care Res (Hoboken) 2012;64:475-87.  Back to cited text no. 5
    
6.Willeke P, Gaubitz M, Schotte H, Becker H, Mickholz E, Domschke W, et al. Clinical and immunological characteristics of patients with Sjögren's syndrome in relation to alpha-fodrin antibodies. Rheumatology (Oxford) 2007;46:479-83.  Back to cited text no. 6
    
7.Porkodi R, Rukmangatharajan S, Kanakarani P, Parthiban M, Vasanthy N, Madavan R, et al. Primary Sjögren's syndrome- Clinical and immunological features. J Indian Rheumatol Assoc 2003;11:63-5.  Back to cited text no. 7
    
8.Misra R, Hissaria P, Tandon V, Aggarwal A, Krishnani N, Dabadghao S. Primary Sjögren's syndrome: Rarity in India. J Assoc Physicians India 2003;51:859-62.  Back to cited text no. 8
    
9.Tzioufas AG, Youinou P, Moutsopoulos HM. Sjögren's syndrome. In: Maddison PJ, Isenberg DA, Woo P, Glass DN, editors. Oxford Text Book of Rheumatology. 2 nd ed. Oxford: Oxford University Press; 1998. p. 1301-17.  Back to cited text no. 9
    
10.Gottenberg JE, Guillevin L, Lambotte O, Combe B, Allanore Y, Cantagrel A, et al.; Club Rheumatismes et Inflammation (CRI). Tolerance and short term efficacy of rituximab in 43 patients with systemic autoimmune diseases. Ann Rheum Dis 2005;64:913-20.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
 
 
    Tables

  [Table 1], [Table 2], [Table 3], [Table 4]



 

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