|Year : 2016 | Volume
| Issue : 4 | Page : 184-185
Sjögren's syndrome: After all not so dry!
Sapan C Pandya
Department of Rheumatology, Vedanta Institute of Medical Sciences and VS Municipal Hospital, Ahmedabad, Gujarat, India
|Date of Web Publication||8-Nov-2016|
Dr. Sapan C Pandya
Vedanta Institute of Medical Sciences and VS Municipal Hospital, Ahmedabad, Gujarat
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Pandya SC. Sjögren's syndrome: After all not so dry!. Indian J Rheumatol 2016;11:184-5
Primary Sjögren's syndrome (pSS) is among the most common autoimmune diseases affecting predominantly women of middle age. The estimated worldwide prevalence stands at 0.1%–0.4% today. For a long time, this disease was believed to be uncommon in India and the subcontinent. While epidemiologic studies are still lacking in India, it is being increasingly diagnosed early and more often, due to availability of more rheumatology centers, better infrastructure, and awareness about the disease among the primary health caregivers.
Roughly 70%–80% of pSS patients present with sicca and have dryness as the dominant symptom throughout their lives. The rest (about 20%–30% from different series) have the “extraglandular complications” of the disease. The latter gains significance being the subset more likely to evolve into a lymphoma. While sicca would go to the dentist/ophthalmologist more often than internists, patients with extraglandular manifestations do not infrequently land up with a rheumatologist. Not uncommonly again, sicca is picked up when they have come with these complications, sometimes in the Intensive Care Unit (e.g., with hypokalemic periodic paralysis resulting from renal tubular acidosis following interstitial nephritis).
In the study titled, “Study on demography and outcome of extraglandular manifestations of Primary Sjögren's syndrome” appearing in this issue of the journal, the investigators, through their prospectively collected data, have validated this issue. While the demographic profile of their patients matched that with an earlier published series from India, there are some notable differences. Both the average age and total duration of illness of patients with extraglandular manifestations are lesser in this series. They have used ESSDAI, a validated outcome measure in pSS, which has been seldom used in the Indian population. Three points that come out strikingly through the study are: (1) To a rheumatologist, extraglandular manifestations are more often a presenting feature, (2) extraglandular manifestations appear early at times even before dryness has set in, and (3) dental caries is a substitute for dryness, and the latter should be looked for and asked for, especially if more florid. While the strength of the study lies in its prospective nature, the authors also go on to discuss the limitations of the study – mainly not using the 2012 American College of Rheumatology classification criteria for Sjögren's syndrome which is more objective based  (due to lack of ocular staining opportunities in our country and lack of salivary flow assessment or scintigraphy, again, procedures not easily done here).
While the questionnaire for pSS in many clinics does include sicca symptoms and dental caries/visits to dentists, we give less importance to the latter. Dental visits/procedures, even implants/early dentures, could be a surrogate for sicca. Dryness is such a common symptom – also seen in diabetes, hypothyroidism, and other common ailments that it is not given the due importance either by the sufferer or the health caregiver. Furthermore, patients get used to their dryness, and there are instances where patients complain of dysphagia rather than dryness and only after endoscopy is a diagnosis reached. In this study, 62/82 patients had extraordinary general meeting and 50/82 had predominant sicca. Referral bias has been cited as the reason which is very likely the case. Renal tubular acidosis presenting as hypokalemic palsy has been seen as a presenting manifestation of pSS. The other feature has been gravitational purpura – the so-called Waldenstrom's hypergammaglobulinemic purpura though not the second most common in this study. Nonhealing ulcers in lower limbs, interstitial lung diseases (Non Specific Interstitial Pneumonitis> Lymphocytic Interstitial Pneumonitis> Usual Interstitial Pneumonitis), and neurologic syndromes (especially demyelinating lesions in the magnetic resonance imaging akin to multiple sclerosis) are among the other presentations encountered. Due to increasing awareness in the medical fraternity now about such cases, many a times antinuclear antibodies are ordered, especially if the patient is a young- or middle-aged woman which turn out to be positive and that's how a rheumatologist comes into the picture. The catch here, as has been pointed out in this study, is that quite a few, if not many of them, do not have sicca. The blots show anti-Ro and anti-La positivity, most have raised globulins, high erythrocyte sedimentation rates, high titer circulating rheumatoid factors, and low complements. The question that then comes to the mind is, are these form fruste Sjögren's cases? The cytokine profiles would probably show an interferon signature. What has not been done before and for the same we must credit the investigators of this study is to biopsy the salivary glands of these patients and as the results show, most of them confirmed the diagnosis of Sjögren's syndrome histologically. It appears thus that in biposy the immune-inflammatory stage was picked up. Following on, it is logical to think that by the time patients develop sicca, patients have the consequence, or, in technical terms, damage, at least in this “extraglandular dominant” subset of pSS. Sicca here is damage and not activity! Should we be picking up these cases at this early stage and induce them with treatment, be it corticosteroids or immunosuppressives or even B-cell depletion and preventing sicca?
A question only time and further studies will answer.
| References|| |
Reksten TR, Jonsson MV. Sjögren's syndrome: An update on epidemiology and current insights on pathophysiology. Oral Maxillofac Surg Clin North Am 2014;26:1-12.
Qin B, Wang J, Yang Z, Yang M, Ma N, Huang F, et al.
Epidemiology of primary Sjögren's syndrome: A systematic review and meta-analysis. Ann Rheum Dis 2015;74:1983-9.
Baldini C, Pepe P, Quartuccio L, Priori R, Bartoloni E, Alunno A, et al.
Primary Sjögren's syndrome as a multi-organ disease: Impact of the serological profile on the clinical presentation of the disease in a large cohort of Italian patients. Rheumatology (Oxford) 2014;53:839-44.
Mohanasundaram K, Mani M, Chinnadurai S, Mahendran B, Balaji C, Bhoorasamy A, et al
. Study on demography and outcome of extraglandular manifestations of primary Sjögren's syndrome. Ind J Rheumatol 2016;11:202-6.
Seror R, Theander E, Bootsma H, Bowman SJ, Tzioufas A, Gottenberg JE, et al.
Outcome measures for primary Sjögren's syndrome: A comprehensive review. J Autoimmun 2014;51:51-6.
Shiboski SC, Shiboski CH, Criswell L, Baer A, Challacombe S, Lanfranchi H, et al.
American College of Rheumatology classification criteria for Sjögren's syndrome: A data-driven, expert consensus approach in the Sjögren's international collaborative clinical alliance cohort. Arthritis Care Res (Hoboken) 2012;64:475-87.
Goules AV, Tatouli IP, Tzioufas AG. Clinically significant renal involvement in primary Sjögren's syndrome: Clinical presentation and outcome. Arthritis Rheum 2013;65:2945-53.
Finder KA, McCollough ML, Dixon SL, Majka AJ, Jaremko W. Hypergammaglobulinemic purpura of Waldenström. J Am Acad Dermatol 1990;23:669-76.
Sada PR, Isenberg D, Ciurtin C. Biologic treatment in Sjögren's syndrome. Rheumatology (Oxford) 2015;54:219-30.