The importance of minor salivary gland biopsy in sjögren syndrome diagnosis and the clinicopathological correlation (original) (raw)
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Clinical Reviews in Allergy & Immunology, 2009
The current 2002 classification criteria do not cover the broad clinical and immunological heterogeneity of primary Sjögren syndrome (SS), since five of the six criteria focus exclusively on glandular involvement and the remaining criterion is the mandatory presence of anti-Ro/La antibodies. The aim of this study was to analyze the clinical features of patients with a well-established diagnosis of primary SS who do not fulfill the 2002 classification criteria. Five hundred seven patients diagnosed with primary SS (1993 criteria) were consecutively included and followed up. Two hundred twenty-one (44%) patients did not fulfill the 2002 criteria. These patients were older at diagnosis (p<0.001) and had a lower frequency of parotid enlargement (p=0.002), fever (p=0.041), arthritis (p=0.041), vasculitis (p=0.050), peripheral neuropathy (p=0.002), cranial nerve involvement (p=0.015), raised erythrocyte sedimentation rate (ESR) levels (p<0.001), anemia (p<0.001), leukopenia (p=0.037), hypergammaglobulinemia (p<0.001), positive rheumatoid factor (RF; p=0.002), and cryoglobulinemia (p=0.049) in comparison with those fulfilling 2002 criteria. However, there were no significant differences in the prevalence of sicca features, diagnostic tests, overall systemic involvement, antinuclear antibodies, complement levels, development of B-cell lymphoma, or survival. Patients with anti-Ro antibodies had the highest frequencies of systemic features, hematological abnormalities, and altered immunological markers. In conclusion, patients fulfilling the 2002 criteria, who have either a specific histological diagnosis (lymphocytic infiltration) or highly specific autoantibodies (Ro/La), might well be considered to have Sjögren "disease." In contrast, etiopathogenic mechanisms other than lymphocytic-mediated epithelial damage could be involved in patients with negative Ro and negative biopsy, in whom the term Sjögren "syndrome" seems more adequate.
Current concepts on Sjögren's syndrome - classification criteria and biomarkers
European journal of oral sciences, 2018
Sjögren's syndrome is a lymphoproliferative disease with autoimmune features characterized by mononuclear cell infiltration of exocrine glands, notably the lacrimal and salivary glands. These lymphoid infiltrations lead to dryness of the eyes (keratoconjunctivitis sicca), dryness of the mouth (xerostomia), and, frequently, dryness of other surfaces connected to exocrine glands. Sjögren's syndrome is associated with the production of autoantibodies because B-cell activation is a consistent immunoregulatory abnormality. The spectrum of the disease extends from an organ-specific autoimmune disorder to a systemic process and is also associated with an increased risk of B-cell lymphoma. Current treatments are mainly symptomatic. As a result of the diverse presentation of the syndrome, a major challenge remains to improve diagnosis and therapy. For this purpose an international set of classification criteria for primary Sjögren's syndrome has recently been developed and valida...
Histopathological and immunohistochemical profile in primary Sjögren's syndrome
Romanian journal of morphology and embryology = Revue roumaine de morphologie et embryologie, 2017
Sjögren's syndrome (SS) is an autoimmune disease characterized by hypofunction of the salivary and lachrymal glands. Main clinical features of SS are sicca symptoms, due to the altered glandular function. Also, in advanced stages, bilateral swelling of the parotid glands can be noted, indicative of severe glandular involvement. Phenotypic expression of various mononuclear cells present in the affected tissue offers additional insight into cellular proliferation, survival, migration, antibody secretion and also the potential of forming tertiary lymphoid tissue, i.e., germinal centers. The main objective of the present study was to evaluate various autoimmune activity patterns present in salivary glands by means of immunohistochemistry (IHC) analysis. The study group comprised of 10 primary SS patients, with various degrees of lymphocytic infiltrates confirmed on minor salivary gland (MSG) biopsy. We could identify both morphological changes, i.e., ductal system abnormalities or i...
Novel autoantibodies in Sjogren's syndrome
Clinical Immunology, 2012
Sjogren's syndrome (SS) is defined by autoantibodies to Ro and La. The current studies identified additional autoantibodies in SS to salivary gland protein 1 (SP-1), carbonic anhydrase 6 (CA6) and parotid secretory protein (PSP). These autoantibodies were present in two animal models for SS and occurred earlier in the course of the disease than antibodies to Ro or La. Patients with SS also produced antibodies to SP-1, CA6 and PSP. These antibodies were found in 45% of patients meeting the criteria for SS who lacked antibodies to Ro or La. Furthermore, in patients with idiopathic xerostomia and xerophthalmia for less than 2 years, 76% had antibodies to SP-1 and/or CA6 while only 31% had antibodies to Ro or La. Antibodies to SP-1, CA6 and PSP may be useful markers for identifying patients with SS at early stages of the disease or those that lack antibodies to either Ro or La.
Sjögren’s syndrome: a systemic autoimmune disease
Clinical and Experimental Medicine, 2021
Sjögren's syndrome is a chronic autoimmune disease characterized by ocular and oral dryness resulting from lacrimal and salivary gland dysfunction. Besides, a variety of systemic manifestations may occur, involving virtually any organ system. As a result, the disease is characterized by pleomorphic clinical manifestations whose characteristics and severity may vary greatly from one patient to another. Sjögren's syndrome can be defined as primary or secondary, depending on whether it occurs alone or in association with other systemic autoimmune diseases, respectively. The pathogenesis of Sjögren's syndrome is still elusive, nevertheless, different, not mutually exclusive, models involving genetic and environmental factors have been proposed to explain its development. Anyhow, the emergence of aberrant autoreactive B-lymphocytes, conducting to autoantibody production and immune complex formation, seems to be crucial in the development of the disease. The diagnosis of Sjögren's syndrome is based on characteristic clinical signs and symptoms, as well as on specific tests including salivary gland histopathology and autoantibodies. Recently, new classification criteria and disease activity scores have been developed primarily for research purposes and they can also be useful tools in everyday clinical practice. Treatment of Sjögren's syndrome ranges from local and symptomatic therapies aimed to control dryness to systemic medications, including disease-modifying agents and biological drugs. The objective of this review paper is to summarize the recent literature on Sjögren's syndrome, starting from its pathogenesis to current therapeutic options.
Primary Sjögren’s Syndrome and Autoantibodies
Autoantibodies and Cytokines [Working Title], 2018
The presence of certain autoantibodies in the serum of patients facilitates the diagnosis of particular autoimmune diseases. Some antibodies may also be significant for the prognosis of the disease development and internal organs involvement. In the case of Sjögren's syndrome, it is known that overactivity of B-lymphocytes leads to the production of a number of autoantibodies-both markers for pSS (such as antibodies to ribonucleoproteins) and nonspecific antibodies (such as rheumatoid factor). The range of autoantibodies found in pSS is constantly expanding, but their significance is not fully established. At present, only anti-SS-A antibodies are introduced to the criteria for the pSS diagnosis. However, this does not stop an interest in other autoantibodies and the significance of their presence for the course of this disease. This chapter outlines the autoantibodies found in pSS and discusses their importance in clinical practice.
Sjögren’s syndrome: still not fully understood disease
Rheumatology International, 2014
hyper-reactivity and polyclonal production of immunoglobulins, and as a consequence, autoantibodies against pSS affected exocrine glands, especially lacrimal glands and salivary glands, but also other external glands such as the pancreas, mucous glands of the gastrointestinal and respiratory tract or bile secretion. In some patients, abnormal H+ secretion in the distal tubules has also been observed and caused distal renal tubular acidosis (type 1 RTA). In connection with the clinical symptoms which will result from the degree of attachment of these glands and epithelial injury, the pSS often uses the term "autoimmune epithelitis" or "autoimmune exocrinopathy" for better imaging of primary initiation sites of inflammation and autoimmunization. A brief history First lacrimal and salivary glands enlargement was described in a lecture of a Polish surgeon Jan Mikulicz-Radecki in 1888. In 1925, French dermatologists Henri Gougerot described a few cases of atrophy of the salivary glands with dryness of eyes, mouth and vagina. Later in 1933, Swedish ophthalmologists Henrik Sjögren in his doctoral thesis described keratoconjunctivitis sicca, and his description became a basis for pSS picture. Epidemiology It is estimated that primary Sjögren's syndrome occurs from 0.1 to 3.0 % in general population. The disease is more common for women (female/male ratio 9:1), mainly between the ages of 40-60, with the disease most frequently occurring around 50 years of age. Pathogenesis of pSS is not clear, nowadays several factors responsible for the development of the disease, such as Abstract Primary Sjögren's syndrome is an autoimmune disorder with external exocrine glands dysfunction and multiorgan involvement. The pathogenesis of primary Sjogren's syndrome is still unclear; however, our knowledge of the involvement of different cells (e.g., B and T cells, macrophages and dendritic cells) and pathways (BAFF/APRIL and interferons) leading to the development of autoimmunity is continually expanding. For clinicians, the most frequent symptoms are dryness of eyes and mouth, but often the patients have musculoskeletal symptoms and systemic manifestations. However, the increased risk of lymphoproliferative disorders in this group of patients, most commonly B-cell marginal zone lymphoma, is particularly important. Recent separation of IgG4-related diseases and attempts to create further diagnostic criteria for pSS testify to the difficulties, and at the same time a large interest, in understanding the disease so as to allow the effective treatment. This article draws attention to the problems faced by the clinician wishing to securely identify pSS by using accurate laboratory biomarkers and useful imaging tools and predict the development of complications associated with this, still not fully understood, autoimmune disease.