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The prototype of a salivary lymphoepithelial lesion is the autoimmune disease Sjögren's syndrome with the characteristic lymphoepithelial duct lesions (LEL). The distinction of Sjögren's syndrome from cases with initial transformation into associated marginal zone B-cell lymphoma (MALT type) can be very challenging, whereby the presence of small "halos" can lead to over-diagnosis. The HIV-associated cystic lymphoepithelial lesion can be histologically almost identical to Sjögren's syndrome and often needs clinical correlation. The sporadic lymphoepithelial cyst of the parotid gland is a frequent finding and has no clinical consequence; however, this entity needs to be identified and distinguished from the above-mentioned entities. The most frequent diagnosis in resected submandibular glands is chronic-fibrosing sialadenitis, so-called Küttner's tumour. Altogether, there is a wide spectrum of lymphoepithelial interaction in the area of salivary glands, including biphasic lymphoepithelial tumours with an obligate lymphoid component, epithelial tumours with facultative tumour-associated lymphoid proliferation, and different processes of intraparotid lymph nodes. The immunohistological reaction for pan-keratin can be very helpful for a thorough pattern analysis of the different lymphoepithelial lesions. The relative frequency of the lesions in different salivary glands can also be diagnostically helpful.

A classification of nonmalignant lymphoproliferation in Sjögren's syndrome is presented, based on the results of international meetings regarding Sjögren's syndrome-associated lymphomagenesis and on our results of a clinico-pathologic and molecular study and long-term follow-up in well-characterized patients. Sjögren's syndrome pathobiology has similarities to hepatitis C virus-related B-cell lymphoproliferation. Antigen stimulation with the preferential expansion of rheumatoid factor-positive clones and specific immunoglobulin gene expression and recombination represent key biologic events in lymphoproliferation. This classification is based on the coupling of molecular and histological studies and may result in more selective treatment approaches.

Background: Chronic sialectatic parotitis (CSP) is classified as a characteristic form of chronic recurrent parotitis the etiology and pathogenesis of which still remains unclear. The multiplicity of different therapeutic advices, especially the permanent failure of antibiotic treatment, underlines the lack of an appropriate causal therapy., Case Report: Detailed histopathological investigations of an 41-year old woman were possible over a seven-year period. These follow-up observations enabled clarification of the histopathogenesis of CSP by means of immunohistochemistry., Results: During the course and development of CSP different stages can be observed: The initial phase is characterised by mild infiltration of B-lymphocytes (CD20, CD45 R) and plasma cells in the environment of ectatic ducts. Progredient stages show neogenetic lymph follicles periductular as well as metaplasia of the ductal epithelium. Terminal phases of CSP are characterised by near-total lymphatic transformation of parenchyma, follicular lymphatic hyperplasia (KiM4) and myoepithelial proliferation. In this phase myoepithelial sialadenitis (MESA, i.e. benign lymphoepithelial lesion, possibly part of Sjögren's syndrome) develops. Beyond it low grade non-Hodgkin's lymphoma of the MALT-Type of the submandibular gland occurred finally., Conclusions: CSP presents as a precursor of MESA. Immunohistological detection of follicular dentritic network (KiM4) within extensive lymphatic hyperplasia periductular demonstrates overshooting humoral immune reaction of B-lymphocytes. Hence, CSP should be classified with regard to pathogenesis as an immunopathological disorder of the MALT system.

Sjören syndrome favors the development of lymphoma, particularly in the salivary glands with MALT lymphomas. The differential diagnosis with benign lymphoepithelial sialadenitis can be difficult. A 78-year-old woman had an oculo-buccal sicca syndrome for 10 years and developed parotid hypertrophy. The first biopsy, performed 7 years before the present investigation had showed chronic lymphoepithelial sialadenitis. A second biopsy showed MALT lymphoma. Search for extension revealed a second gastric localization of the lymphoma. This patient had a particular immunophenotype, showing a CD5+ tumoral population frequently observed in mantel lymphomas and usually lacking in MALT lymphomas. Recently, however, another case similar to our own, has been reported in the literature. The observations raise the problem of distinguishing between mantel lymphoma and MALT lymphoma.

Common and differential clinical and morphological signs of chronic parotitis, parotid gland sarcoidosis and Sjögren's disease are outlined. 5 cases of sarcoidosis and 28 cases of Sjögren's disease served as illustration.

In order to characterize typical MRI-findings in patients with Sjögren's syndrome who present recurrent parotid swelling 15 patients with clinical diagnosis of this disease were examined in a prospectives study. Most of the patients showed bilateral enlarged glands with an inhomogeneous, nodular pattern. The signal intensity was very high, especially in the T2 weighted sequences.

Histologic methods were used to examine biopsy specimens of minor (labial) salivary glands obtained to verify Sjogren's disease in 14 patients with this condition, 12 ones with chronic parenchymatous parotitis, and in 4 subjects with chronic interstitial parotitis. Sjogren's disease, chronic parenchymatous parotitis, and, to a lesser degree, chronic interstitial parotitis were found to be associated with development in the minor salivary glands of an immune inflammation with the predominance of features characterizing delayed-type hypersensitivity reaction presenting as mainly lymphoid macrophagal infiltration of tissue first near the ducts and then near the end portions. This or that degree of glandular lymphoid macrophagal infiltration is present during all periods of disease and it therefore cannot be considered as a process secondary in relation to extension of the secretory portion of the gland. These data may be useful in interpretation of morphologic changes in the minor salivary glands in various forms of chronic sialadenitis.

Sequential quantitative scintigraphy of parotid glands was performed in 16 cases with recurrent parotitis in childhood, 33 with chronic obstructive parotitis, 37 with Sjögren's syndrome, 4 with sialadenosis and 30 normal controls. Uptake function was normal, but excretion was retarded in recurrent parotitis in childhood. Chronic obstructive parotitis exhibited that excretion was obstructed and the uptake and excretion function between bilateral parotids was marked different. In Sjögren's syndrome, uptake was very low and excretion was heavily delayed. Sialadenosis showed that excretion was retarded and uptake function was not definitely involved. The possible mechanism of the scintigraphical features were analyzed combined with the histopathological findings.

Clinical stomatologic and cytologic studies were carried out in 31 patients with Sjogren's disease, 12 ones with chronic parotitis, and 5 normal subjects. Patients with Sjogren's disease, in contrast to those with chronic parotitis, develop clinically manifest signs of dry exfoliative cheilitis, xerostomia, atrophic changes in the buccal mucosa; this condition is associated with a very high caries intensity and no clinical manifestations of periodontitis or tartar. Elevated leukocyte levels in oral washings and oral fluid of patients with Sjogren's disease and chronic parotitis point to inflammations in the salivary glands, buccal mucosa, and periodontal tissues. Assessment of the leukocyte levels may be used for monitoring the disease course and treatment efficacy. A 3-5-fold enlargement of the deep-layer epitheliocytes, seen on the cytogram of oral washings, is characteristic of patients with Sjogren's disease, in contrast to those with chronic parotitis or normal subjects. A differentiated estimation of the epitheliocyte types in the cytogram of oral washings may be used in the diagnosis of Sjogren's disease along with the criteria suggested before.

This short report describes the development of life-threatening upper airways obstruction in a patient with primary Sjögren's syndrome (PSS), and reviews the published literature on this unusual development.