An update on recent advances diseases of the salivary glands
in the understanding
of non-neoplastic
C. Scully Centre ,for Study of Orul Diseuse,
Bristol Dentul Hospitul urld School
SUMMARY. The recent advances in the understanding of the non-neoplastic disorders of salivary glands are summarised in this paper. The common infections are discussed and newer aspects of juvenile recurrent parotitis outlined. The benign lympho-epithelial lesion is reviewed in relation to Sjogren’s syndrome and the salivary gland pathology seen in relation to infection with human immunodeficiency virus. A range of unusual conditions that may affect the minor salivary glands in particular, and can cause diagnosis dilemmas, is summarised.
INTKODUCTION The most common non-neoplastic disorders of salivary glands arc inflammatory lesions affecting the major salivary glands. Mostly these are acute viral or. occasionally, bacterial or other infections but obstructive sialadcnitis and chronic disorders such as Sjogren’s syndrome arc also important. Recent advances in these non-ncoplastic disorders are reviewed. restricting references to useful recent reviews and original papers.
INFECTIONS Salivary infections other than with viruses and some bacteria are remarkably rare.
Vim1 siuladenitis Mumps (acute viral sialadcnitis) is nearly always caused by a paramyxovirus and is a self-limiting condition for which no specific treatment is available. Coxsackie, ECHO, influenza or para-influenza viruses, Epstein-Barr virus and human immunodeficiency virus (HIV: see below) arc sometimes implicated in similar syndromes (Scully & Samaranayake, 1992). The association of viruses with salivary gland diseases is reviewed elsewhere (Scully, 1988). Any long-term sequelae of viral sialadcnitides are unclear, though there is concern about possible associations with salivary autoimmune disorders and ncoplasms (Scully, 1988).
Bucteriul siuludenitis Bacterial sialadenitis, formerly often referred to as ‘surgical parotitis’ or ‘surgical mumps‘ is now uncommon, presumably b&use of improved pcrioperative care and antimicrobials. Gram negative
and other bacteria however, readily colonise the ducts of xcrostomic glands or those with structural anomalies (Katz et al., 1990). Most bacterial parotitis is of polymicrobial nature, responsive to flucloxacillin or crythromycin (Lamey et al., 1987; Fox, 1991). Culture and sensitivity are still indicated however, since unusual organisms (for example. Eikenellu corrodens) are occasionally involved (Fox, 1991).
Juvenile recurrent purotitis Juvenile recurrent parotitis, or chronic recurrent parotitisofchildhood, hasbccnofunknownactiology. though infection with Epstein-Barr virus, occasional bacterial infections and allergies have been incriminated and some children were known to have congenital ductal anomalies or hereditary predisposing factors (Myer & Cotton, 1986; Seifert et ul., 1986). One recent extensive study demonstrated congenital ectasia in the pcriphcry of the ductal tree, probably the intralobular ducts in most patients examined, and found infection in 80% of these patients during asymptomatic phases-most infcctions were coccal, half of thcsc being with anaerobic cocci (Ericson et al., 1991). This suggests that congenital ductal ectasia followed by ascending infection may be the pathogcnesis of juvenile recurrent parotitis. Most acute phases respond to penicillin V (Ericson el al., 1991).
BENIGN LYMPHOEPITHELIAL
LESION
Benign lymphocpithelial lesion (BLL) is a characteristic histopathological picture consisting initially of a diffuse lymphocytic infiltrate around intralobular salivary ducts, later with acinar atrophy and proliferation of ductal epithclium which blocks the luminn and produces epithclial (epimyoepithelial)
Non-ncoplastic
islands. BLL can be seen in apparent isolation (Mikulicz disease) or in Sjogren’s syndrome (SS), and a similar picture is seen in HIV salivary gland disease (HIV-SGD: see below). BLL may, in up to 5’%,, be complicated by malignant lymphoma, typically non-Hodgkin’s lymphoma, and in up to 1%I by anaplastic carcinoma. Sjqren’s
syndrome
Many of the recent advances in Sjogren’s syndrome (SS) arc rcviewcd elsewhere (Talal, 1989) and this paper is restricted to the oral features, diagnosis, and management of oral health. It is now quite clear that SS is a hetcrogencous entity, that the autoimmune associationscan be wide and varied (typically with rheumatoid arthritisor primary biliary cirrhosis), and that the immunological changes are very complex. The actiology is unclear but immunogenetic and viral influences arc strongly incriminated (Talal, 1989; Scully, 1988, 1991; Scully & Samaranayake, 1992).
Orul feutures: Xcrostomia (dry mouth) is frequent though not all patients actually complain of a dry mouth and there arc many patients without SS who do complain of oral dryness. Dysphagia, particularly with dry foods (the ‘cracker sign’). difficulty in talking, a burning sensation in the mouth, and the disease scquelac of xerostomia arc well recognised. Enlargement of the major salivary glands may also affect up to one third of patients with SS. particularly those with primary SS (Scully, 1986; 1988; 1991). Examination may show a lack of salivary pooling. adherence of mucosa to the dental mirror. thickness and frothiness of saliva with obvious spinbarkcit, and absence of or reduced_ frank salivation from major gland duct orifices. There is a predisposition to bacterial sialadenitis. Candidosis may present as oral erythcma, and there may be angular stomatitis. The tongue may be lobulated with atrophy of filiform papillae. Caries affects particularly the smooth surfaces of the teeth but periodontal dtscase is now known not to be increased (Mutlu et ul., 1991). Diugnosis: Most patients with clinically significant SS are revealed by the history and examination, together with ocular, serological and labial salivary gland biopsy findings. Investigation of patients for evidence of systemic disease can be helpful. Raised erythrocytc sedimentation rates (or plasma viscosity), and serum autoantibodies such as Ro (SS-A), La (SS-B), and Hep-2 can be supportive of a diagnosis of an associated connective tissue disease. Investigation of salvary function may include (Scully, 1989): measured as (a) Salivary flow rures (sialometry) resting secretion rate of whole saliva (cl.5 ml/15 min is abnormal) which is probably more closely associated with symptoms than are stimulated flow rates, is easier to assess, is reduced in early SS when stimulated parotid flow rates can be normal. and measures secretions of several glands.
diseases
of salivaryglands
245
(b) Siuiogruphy is of low specificity though findings may be as, or more sensitive than, labial salivary gland (LSG) biopsy in the hands of some workers, and may detect some patients with ncgativc LSG histology. However, sialography can fail to detect some with known SS, is time-consuming, can be painful. and may be complicated by infection or allergic or granulomatous reactions. (c) Ultrusonogruphy usually reveals decreased echogenicity in the parotids in SS, though some have normal cchograms even when LSG biopsy is positive. Ultrasonographic findings in the parotids correlate well with sialographic changes but submandibular ultrasonography can be difficult to assess. (d) Computed tomogruphy (CI’) may be of some value where there arc complications such as lymphoma in the major glands but, in general, CT findings do not correlate with sialographic or ultrasound findings and are not discriminative. (e) Mugnetic resonance imaging has not been thoroughly explored in the diagnosis of SS. (f) Scinbgruphy with technetium pertechnetate shows reduced uptake in, and spontaneous secretion from, salivary glands and diminished secretion following stimulation. It is relatively non-invasive, examines all major glands simultaneously, and may detect early functional deficits (Van den Akker & Buseman-Sokole, 1985). Scintigraphic changes correlate with sialographic, flow rate and LSG histological findings but changes are non-specific and non-discriminatory and can be seen in some patients with apparently healthy glands. Furthermore, scintigraphy is costly, time-consuming, not universally available and not always positive in SS. (g) LSG biopsy shows histopathological changes which correlate in pattern and extent with those in major glands. Focal sialadenitis is the characteristic feature and, if used with a focus score provides a reproducible objective evaluation of severity of inflammation. LSG biopsy is more acceptable to patients, and less hazardous than biopsy of the other glands, but can sometimes fail to dctcct SS. (h) Siulochemisrry shows raised salivary levels of sodium, chloride, albumin, lactofcrrin. IgA and IgG in SS but results arc non-specific and conflicting. A wide range of investigations arc available thcrcfore, but most units now make the diagnosis on the basis of history, examination. ocular findings (positive Schirmcr and Rose Bengal tests), serology and labial salivary gland biopsy. Sialometry may bc helpful for assessing the degree of functional deficit.
Munqement of orul he&h: Dry and cariogcnic foods. and xerostomia-producing drugs (such as tricyclics). alcohol and smoking should be rcduccd. Salivation can be stimulated with citrus products (their low pH, however, may damage the teeth); masticatory stimuli (for example, sucrose-free or parasympathomimetics diabetic chewing gum), (pilocarpinc or bcthanecol) and anctholctrithionc. or rarely electrical stimulation. Artificial salivas can bc helpful. They contain carboxymcthyl cellulose or mucin as lubricants; sugars such as xylitol, mannitol
246
British Journal of Oral and Maxillofacial
Surgery
or sorbitol--all of low cariogenicity; salts and flavourings; some have calcium. phosphate and/or fluoride ions to assist tooth rcmincralization; and most contain preservatives such as mcthyl-phydroxybcnzoatc. Those liquids currently available in the UK include Saliva Orthana (Nycomed), Glandosane (Fraenius) and Luborant (Antigen). Saliva Orthana also make a pastille. Oral health care also includes the prevention and treatment of infections, including caries. Fluorides and control of sucrose intake are essential to control caries. Daily use of fluoride gels or mouth rinses is useful. Oral candidosis is almost invariable and topical antifungals such as nystatin or miconazole are often indicated. Dentures should be left out of the mouth at night in antifungal solutions such as sodium hypochlorite or chlorhexidine. Patients, especially those with primary SS, should be reviewed regularly to exclude the development of lymphoma.
Salivury gland involvement in HIV infection HIV-associated salivary gland disease (1 IIV-SGD) is a sicca syndrome characterised by xerostomia, parotid enlargement, lymphadenopathy and pulassociated with a diffuse monary insufficiency, infiltrative lymphocytosis but no Ko or La autoantibodies. Cystic benign lymphoepithelial lesions may develop in the parotids (Itescu, et al., 1989; Schiodt. et al., 1989). Computed tomography, sialocitrate scanning, and magnetic graphy, “‘Gallium resonance imaging can help diagnosis but technetium pertechnetate scanning is of little value (Scully et al., 1992). Lymphomas may occasionally be associated with HIV-SGD and cannot be reliably excluded radio-graphically or by ultrasound. Fine needle aspiration may help but superficial parotidectomy or parotid biopsy may well be indicated where this possibility is strongly entertained. MISCELLANEOUS DISORDERS AFFECTING MAINLY THE MAJOR SALVARY GLANDS
Siufolithiusis (salivary calculi) More than 90% of salivary calculi are found in the submandibular duct though other major, and occasionally minor glands, may be affected. Lithotripsy has now been used to remove calculi (Cook et al., 1988) but further studies are required.
Siufoudenosis (sialosis) Sialosis is an insidious asymptomatic and usually persistent bilateral enlargement of the parotids. Most cases are in the 6th decade and associated with alcoholism or malnutrition or diabetes but a diverse range of aetiologies including bulimia is recognised, with a common feature of autonomic ncuropathy (Batsakis, 1988). Acinar cell hypertrophy with later fatty infltration is the major histopathological feature. Salivary amylase levels arc increased but there is no xerostomia. The condition is self-limiting but the underlying predisposing factors should be elicited and treated where possible.
Kuttner tumour Chronic submandibular sialadcnitis can produce a firm tumour-like swelling, known as the Kuttncr tumour. Focal sialadenitis is followed by diffuse lymphocytic sialadcnitis with fibrosis, then chronic sclerosing sialadenitis and finally chronic progressive sialadenitis (Yoshihara et al., 1983).
MISCELLANEOUS DISORDERS AFFECTING MAINLY THE MINOR SAIdWARY GLANDS
Siq~erficiul mucoceles Subepithclial mucoceles that may cause confusion with vesiculoerosive disorders have recently been described (Eveson, 1988). These are typically seen in the palate or buccal mucosa of middle aged or older females and present as vesicles or bulla which are sub- or intra-epithelial. They may thus simulate pemphigoid or pemphigus, but in superficial mucocclcs immunostaining of biopsies fails to demonstrate the immune deposits characteristic of the autoimmune disorders.
Adenomutoid
hyperplusiu
Minor salivary glands may rarely undergo hypertrophy sometimes with focal mucus spillage, and produce a tumor-like mass-typically in the palate. The actiology is usually idiopathic but reactive hypcrplastic may also be seen. Excision is not followed by recurrence (Buchner et al., 1991).
Necrotising siulometuplasiu Nectrotising sialometaplasia has achieved some repute because this benign self-limiting idiopathic condition associated with ischacmic infarction, can present as aggressive ulceration with pseudoepitheliomatous hyperplasia thus leading to the misdiagnosis of malignancy (Van der Wal & Van der Waal, 1990). Typically seen in the palate in young adult males. this condition may be seen in any site where there is salivary tissue.
Cheilitis glandularis Cheilitis glandularis is a rare idiopathic disorder associated with hypertrophicd minor salivary glands, typically in the lower lip but occasionally in the upper lip or palate (Winchester et uf., 1986; Williams & Williams, 1989). It may resolve with intralesional corticosteroids but it is said to predispose to carcinoma in up to 30%.
References
13uchncr. A.. Mcrrell. P. W.. Carpcntcr. W. M. B Lcider. A. S. (I99l). Adenomatoid hypcrplasia of minor salivary glands. OIUI .Sur,qery. Orul Medicine und Orcd Purlwlo~~~v. 7 I, 583.
Non-neoplastic Cook. H. P.. Borrows. D. J. & Milroy. E. J. G. (IYXX). I.ithotripsy of inaccessible salivary duct stone. I.ur~ccr. 2,213. Ericson. S.. Zetterlund, B. & Ohman, Z. (IYYI). Recurrent parotitis and sialcctasis in childhood: clinical. radiologic. immunologic. bacteriologic and histologic study. Annuls oj
Orology und RhinolurynKoloSy 100,527. Evcson. J. W. (IYXX). Superficial mucocclcs: pitfall in clinical and microscopic diagnosis. Orul Surgery. Orul iMedicine und Orul PorholoCqy,66, 31X. Fox. P. C. (lY91). Bacterial infections of salivaryglands. C‘urrmr Itescu. S.. Brancato. L. J. & Winchester. K. (IYXY). A sicca syndrome in HIV infection: association with I ILA-IIRS and cDX lymphocytosis. /.cntcer. 2,466. Katz.J.. Fisher. D.. Lcviner. E.. Bcnoliol. R. &&la. M. N. (IYOO). Bacterial colonizationofthcparotidduct in xerostomia. InternarionulJournalc~~‘Orulund Muxillofaciul
Surgery, 19,7. I.amcy. P. J.. Boyle. M. A.. MacFarlanc, ‘I‘. W. & Samaranayake. L. I’. (19X7). Acute suppurative parotitis in out-patients: microbiologic and post-trcatmcnt %ialographic findings. Orul Surgery. Owl Medicine,, Orul Putholqy, 63,
s7. Mutlu. S.. Porter. S. R., Richards. A., Porter, K. M.. Maddison. P. & Scully. C. (199 I). Periodontal disease in Sjogrcn‘s syndrome. Clinicnl and Experimen/ul Rheumufology, 9, 3 17. Mycr. C. & Cotton, R. T. (IYX6) Salivary gland discas in children: a rcvicw. C‘linicul t’ediarric.s. 25, 314. Schiodt. M.. Greenspan. I>.. Dan&, ‘I‘. E., Nelson. J.. Leggott. P. J., Wara, D. W. & Grccnspan, J. S. (IYXY). Parotid gland enlargement and xcrostomia associated with labial sialadcnitis in HIV-infected patients. Jmtrna/o/
2,415.
Scully, C. (lYX6). Sjogren’s syndrome: a review of immunopnthogcnesis. clinical and laboratory fcaturcs. and manaPcmcnt in relation to dentistry. Oral Surpry. Orul
Medicine, Orul Puihology. 62,‘s 10. Scully. C. (IYXX). Viruses and salivary gland &case: arc there associations? Orul Surgery. Orul Medicine, Orul I’utholog,v.
247
Sauvezic(ecJs),pp.41-58. Paris: McrckSharpDohme Chibret. Scully. C., Davies. R., Porter.S., Eveson,J. bi Luker. J. (lYY2). Salivaryscintiscanningwith tcchnctiumpcrtechnetatcin HIV-salivaryglanddiscase. OrulSurgcry, OralMedicine, Oral Puthology (in press). Scully. C. &Samaranayakc, L. P. (1992). ClinicalVirologyin Dentislryund OralMedicine, pp. 370-378. Cambridge: Cambridge University Press. Seifert.
G., Miehlkc.
A., ilaubrich.
J. & Chilla.
iXseuses of rhe Sulivary Glands. Stuttgart:
Opinion in Dentistry. I, 41 I.
Auloimmuniiy.
diseases of salivary glands
Verlag. ‘I‘alal. N. (IYXY). Sjqqren’s Syndrome:
R. (19X6). Georg Thicmc
A Model Fo:o,
d’ndersrunding Au/oimmuniry.
London: Academic Press. Winchester. L.. Scully. C.. Prime, S. S. & Evcson. J. W. (IYXO). Chcilitis elandularis: a case affecting the upper lip. Orrr/
Swgrry. &I
!Medicine. Orul Pcrrho&y .6i, 654:
Williams. II. K. & Williams. D. M. (IYXO). Persistent sialadenitis of the minor plands~tomatitis‘~lan~ularis. Bri/ish Journul of Orul und ~axillofuciul Swgcr)?, 27, 2 12. Van den Akkcr. H. P. & Buscman-Sokolc. E. (IYXS). Ahsolutc ‘i’M.*.C_ indications for salivary gland scintigraphv with pertcchnctate. Orul Surgery, Orul Medic~ine, Orul I’u~hology.
60, 440. Van der Wal. J. E. & Van dcr Waal, I. (IYYO). Nccroti7ing sialometaplasia: report of 12 new cases. Rriibh Journulo]’
Orul und Muxillojkiul Surgery. 28,326. Yoshihara, T.. Kanda. T.. Yaku. Y. & Kaneko. T. (1YX.I). Chronic sialadenitis of the submandibular gland (so-called Kuttncr tumor). Auris-Nu.su.s Larynx, IO, 117.
The Author Professor C. Scully, PhD. MD, MDS, FDS, WI), MRCPalh Centrc for the Study of Oral Disease University Department of Oral Medicine. Surgery and Pathology Bristol Dental Hospital and School I_owcr Maudlin Street Bristol BSI 21-Y
66,179. Scully. C. (19X9). Oral parameters in the diagnosis of Sjogrcn‘s syndrome. Cliniculund Experimcntul Rheurnutology~ 7, I 13.
Correspondence
Scully.C.(
Paper received 13 December IYY I Accepted 24 February 1902
1991).0ralcomponentofSjogrcn’ssyndrome. G. Bctail and B.
t,eSyndrr,medeG‘ou~crot-Sjogr~n.
In:
and requests for offprints
to Professor
C. Scully
in the understanding
of non-neoplastic
C. Scully Centre ,for Study of Orul Diseuse,
Bristol Dentul Hospitul urld School
SUMMARY. The recent advances in the understanding of the non-neoplastic disorders of salivary glands are summarised in this paper. The common infections are discussed and newer aspects of juvenile recurrent parotitis outlined. The benign lympho-epithelial lesion is reviewed in relation to Sjogren’s syndrome and the salivary gland pathology seen in relation to infection with human immunodeficiency virus. A range of unusual conditions that may affect the minor salivary glands in particular, and can cause diagnosis dilemmas, is summarised.
INTKODUCTION The most common non-neoplastic disorders of salivary glands arc inflammatory lesions affecting the major salivary glands. Mostly these are acute viral or. occasionally, bacterial or other infections but obstructive sialadcnitis and chronic disorders such as Sjogren’s syndrome arc also important. Recent advances in these non-ncoplastic disorders are reviewed. restricting references to useful recent reviews and original papers.
INFECTIONS Salivary infections other than with viruses and some bacteria are remarkably rare.
Vim1 siuladenitis Mumps (acute viral sialadcnitis) is nearly always caused by a paramyxovirus and is a self-limiting condition for which no specific treatment is available. Coxsackie, ECHO, influenza or para-influenza viruses, Epstein-Barr virus and human immunodeficiency virus (HIV: see below) arc sometimes implicated in similar syndromes (Scully & Samaranayake, 1992). The association of viruses with salivary gland diseases is reviewed elsewhere (Scully, 1988). Any long-term sequelae of viral sialadcnitides are unclear, though there is concern about possible associations with salivary autoimmune disorders and ncoplasms (Scully, 1988).
Bucteriul siuludenitis Bacterial sialadenitis, formerly often referred to as ‘surgical parotitis’ or ‘surgical mumps‘ is now uncommon, presumably b&use of improved pcrioperative care and antimicrobials. Gram negative
and other bacteria however, readily colonise the ducts of xcrostomic glands or those with structural anomalies (Katz et al., 1990). Most bacterial parotitis is of polymicrobial nature, responsive to flucloxacillin or crythromycin (Lamey et al., 1987; Fox, 1991). Culture and sensitivity are still indicated however, since unusual organisms (for example. Eikenellu corrodens) are occasionally involved (Fox, 1991).
Juvenile recurrent purotitis Juvenile recurrent parotitis, or chronic recurrent parotitisofchildhood, hasbccnofunknownactiology. though infection with Epstein-Barr virus, occasional bacterial infections and allergies have been incriminated and some children were known to have congenital ductal anomalies or hereditary predisposing factors (Myer & Cotton, 1986; Seifert et ul., 1986). One recent extensive study demonstrated congenital ectasia in the pcriphcry of the ductal tree, probably the intralobular ducts in most patients examined, and found infection in 80% of these patients during asymptomatic phases-most infcctions were coccal, half of thcsc being with anaerobic cocci (Ericson et al., 1991). This suggests that congenital ductal ectasia followed by ascending infection may be the pathogcnesis of juvenile recurrent parotitis. Most acute phases respond to penicillin V (Ericson el al., 1991).
BENIGN LYMPHOEPITHELIAL
LESION
Benign lymphocpithelial lesion (BLL) is a characteristic histopathological picture consisting initially of a diffuse lymphocytic infiltrate around intralobular salivary ducts, later with acinar atrophy and proliferation of ductal epithclium which blocks the luminn and produces epithclial (epimyoepithelial)
Non-ncoplastic
islands. BLL can be seen in apparent isolation (Mikulicz disease) or in Sjogren’s syndrome (SS), and a similar picture is seen in HIV salivary gland disease (HIV-SGD: see below). BLL may, in up to 5’%,, be complicated by malignant lymphoma, typically non-Hodgkin’s lymphoma, and in up to 1%I by anaplastic carcinoma. Sjqren’s
syndrome
Many of the recent advances in Sjogren’s syndrome (SS) arc rcviewcd elsewhere (Talal, 1989) and this paper is restricted to the oral features, diagnosis, and management of oral health. It is now quite clear that SS is a hetcrogencous entity, that the autoimmune associationscan be wide and varied (typically with rheumatoid arthritisor primary biliary cirrhosis), and that the immunological changes are very complex. The actiology is unclear but immunogenetic and viral influences arc strongly incriminated (Talal, 1989; Scully, 1988, 1991; Scully & Samaranayake, 1992).
Orul feutures: Xcrostomia (dry mouth) is frequent though not all patients actually complain of a dry mouth and there arc many patients without SS who do complain of oral dryness. Dysphagia, particularly with dry foods (the ‘cracker sign’). difficulty in talking, a burning sensation in the mouth, and the disease scquelac of xerostomia arc well recognised. Enlargement of the major salivary glands may also affect up to one third of patients with SS. particularly those with primary SS (Scully, 1986; 1988; 1991). Examination may show a lack of salivary pooling. adherence of mucosa to the dental mirror. thickness and frothiness of saliva with obvious spinbarkcit, and absence of or reduced_ frank salivation from major gland duct orifices. There is a predisposition to bacterial sialadenitis. Candidosis may present as oral erythcma, and there may be angular stomatitis. The tongue may be lobulated with atrophy of filiform papillae. Caries affects particularly the smooth surfaces of the teeth but periodontal dtscase is now known not to be increased (Mutlu et ul., 1991). Diugnosis: Most patients with clinically significant SS are revealed by the history and examination, together with ocular, serological and labial salivary gland biopsy findings. Investigation of patients for evidence of systemic disease can be helpful. Raised erythrocytc sedimentation rates (or plasma viscosity), and serum autoantibodies such as Ro (SS-A), La (SS-B), and Hep-2 can be supportive of a diagnosis of an associated connective tissue disease. Investigation of salvary function may include (Scully, 1989): measured as (a) Salivary flow rures (sialometry) resting secretion rate of whole saliva (cl.5 ml/15 min is abnormal) which is probably more closely associated with symptoms than are stimulated flow rates, is easier to assess, is reduced in early SS when stimulated parotid flow rates can be normal. and measures secretions of several glands.
diseases
of salivaryglands
245
(b) Siuiogruphy is of low specificity though findings may be as, or more sensitive than, labial salivary gland (LSG) biopsy in the hands of some workers, and may detect some patients with ncgativc LSG histology. However, sialography can fail to detect some with known SS, is time-consuming, can be painful. and may be complicated by infection or allergic or granulomatous reactions. (c) Ultrusonogruphy usually reveals decreased echogenicity in the parotids in SS, though some have normal cchograms even when LSG biopsy is positive. Ultrasonographic findings in the parotids correlate well with sialographic changes but submandibular ultrasonography can be difficult to assess. (d) Computed tomogruphy (CI’) may be of some value where there arc complications such as lymphoma in the major glands but, in general, CT findings do not correlate with sialographic or ultrasound findings and are not discriminative. (e) Mugnetic resonance imaging has not been thoroughly explored in the diagnosis of SS. (f) Scinbgruphy with technetium pertechnetate shows reduced uptake in, and spontaneous secretion from, salivary glands and diminished secretion following stimulation. It is relatively non-invasive, examines all major glands simultaneously, and may detect early functional deficits (Van den Akker & Buseman-Sokole, 1985). Scintigraphic changes correlate with sialographic, flow rate and LSG histological findings but changes are non-specific and non-discriminatory and can be seen in some patients with apparently healthy glands. Furthermore, scintigraphy is costly, time-consuming, not universally available and not always positive in SS. (g) LSG biopsy shows histopathological changes which correlate in pattern and extent with those in major glands. Focal sialadenitis is the characteristic feature and, if used with a focus score provides a reproducible objective evaluation of severity of inflammation. LSG biopsy is more acceptable to patients, and less hazardous than biopsy of the other glands, but can sometimes fail to dctcct SS. (h) Siulochemisrry shows raised salivary levels of sodium, chloride, albumin, lactofcrrin. IgA and IgG in SS but results arc non-specific and conflicting. A wide range of investigations arc available thcrcfore, but most units now make the diagnosis on the basis of history, examination. ocular findings (positive Schirmcr and Rose Bengal tests), serology and labial salivary gland biopsy. Sialometry may bc helpful for assessing the degree of functional deficit.
Munqement of orul he&h: Dry and cariogcnic foods. and xerostomia-producing drugs (such as tricyclics). alcohol and smoking should be rcduccd. Salivation can be stimulated with citrus products (their low pH, however, may damage the teeth); masticatory stimuli (for example, sucrose-free or parasympathomimetics diabetic chewing gum), (pilocarpinc or bcthanecol) and anctholctrithionc. or rarely electrical stimulation. Artificial salivas can bc helpful. They contain carboxymcthyl cellulose or mucin as lubricants; sugars such as xylitol, mannitol
246
British Journal of Oral and Maxillofacial
Surgery
or sorbitol--all of low cariogenicity; salts and flavourings; some have calcium. phosphate and/or fluoride ions to assist tooth rcmincralization; and most contain preservatives such as mcthyl-phydroxybcnzoatc. Those liquids currently available in the UK include Saliva Orthana (Nycomed), Glandosane (Fraenius) and Luborant (Antigen). Saliva Orthana also make a pastille. Oral health care also includes the prevention and treatment of infections, including caries. Fluorides and control of sucrose intake are essential to control caries. Daily use of fluoride gels or mouth rinses is useful. Oral candidosis is almost invariable and topical antifungals such as nystatin or miconazole are often indicated. Dentures should be left out of the mouth at night in antifungal solutions such as sodium hypochlorite or chlorhexidine. Patients, especially those with primary SS, should be reviewed regularly to exclude the development of lymphoma.
Salivury gland involvement in HIV infection HIV-associated salivary gland disease (1 IIV-SGD) is a sicca syndrome characterised by xerostomia, parotid enlargement, lymphadenopathy and pulassociated with a diffuse monary insufficiency, infiltrative lymphocytosis but no Ko or La autoantibodies. Cystic benign lymphoepithelial lesions may develop in the parotids (Itescu, et al., 1989; Schiodt. et al., 1989). Computed tomography, sialocitrate scanning, and magnetic graphy, “‘Gallium resonance imaging can help diagnosis but technetium pertechnetate scanning is of little value (Scully et al., 1992). Lymphomas may occasionally be associated with HIV-SGD and cannot be reliably excluded radio-graphically or by ultrasound. Fine needle aspiration may help but superficial parotidectomy or parotid biopsy may well be indicated where this possibility is strongly entertained. MISCELLANEOUS DISORDERS AFFECTING MAINLY THE MAJOR SALVARY GLANDS
Siufolithiusis (salivary calculi) More than 90% of salivary calculi are found in the submandibular duct though other major, and occasionally minor glands, may be affected. Lithotripsy has now been used to remove calculi (Cook et al., 1988) but further studies are required.
Siufoudenosis (sialosis) Sialosis is an insidious asymptomatic and usually persistent bilateral enlargement of the parotids. Most cases are in the 6th decade and associated with alcoholism or malnutrition or diabetes but a diverse range of aetiologies including bulimia is recognised, with a common feature of autonomic ncuropathy (Batsakis, 1988). Acinar cell hypertrophy with later fatty infltration is the major histopathological feature. Salivary amylase levels arc increased but there is no xerostomia. The condition is self-limiting but the underlying predisposing factors should be elicited and treated where possible.
Kuttner tumour Chronic submandibular sialadcnitis can produce a firm tumour-like swelling, known as the Kuttncr tumour. Focal sialadenitis is followed by diffuse lymphocytic sialadcnitis with fibrosis, then chronic sclerosing sialadenitis and finally chronic progressive sialadenitis (Yoshihara et al., 1983).
MISCELLANEOUS DISORDERS AFFECTING MAINLY THE MINOR SAIdWARY GLANDS
Siq~erficiul mucoceles Subepithclial mucoceles that may cause confusion with vesiculoerosive disorders have recently been described (Eveson, 1988). These are typically seen in the palate or buccal mucosa of middle aged or older females and present as vesicles or bulla which are sub- or intra-epithelial. They may thus simulate pemphigoid or pemphigus, but in superficial mucocclcs immunostaining of biopsies fails to demonstrate the immune deposits characteristic of the autoimmune disorders.
Adenomutoid
hyperplusiu
Minor salivary glands may rarely undergo hypertrophy sometimes with focal mucus spillage, and produce a tumor-like mass-typically in the palate. The actiology is usually idiopathic but reactive hypcrplastic may also be seen. Excision is not followed by recurrence (Buchner et al., 1991).
Necrotising siulometuplasiu Nectrotising sialometaplasia has achieved some repute because this benign self-limiting idiopathic condition associated with ischacmic infarction, can present as aggressive ulceration with pseudoepitheliomatous hyperplasia thus leading to the misdiagnosis of malignancy (Van der Wal & Van der Waal, 1990). Typically seen in the palate in young adult males. this condition may be seen in any site where there is salivary tissue.
Cheilitis glandularis Cheilitis glandularis is a rare idiopathic disorder associated with hypertrophicd minor salivary glands, typically in the lower lip but occasionally in the upper lip or palate (Winchester et uf., 1986; Williams & Williams, 1989). It may resolve with intralesional corticosteroids but it is said to predispose to carcinoma in up to 30%.
References
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s7. Mutlu. S.. Porter. S. R., Richards. A., Porter, K. M.. Maddison. P. & Scully. C. (199 I). Periodontal disease in Sjogrcn‘s syndrome. Clinicnl and Experimen/ul Rheumufology, 9, 3 17. Mycr. C. & Cotton, R. T. (IYX6) Salivary gland discas in children: a rcvicw. C‘linicul t’ediarric.s. 25, 314. Schiodt. M.. Greenspan. I>.. Dan&, ‘I‘. E., Nelson. J.. Leggott. P. J., Wara, D. W. & Grccnspan, J. S. (IYXY). Parotid gland enlargement and xcrostomia associated with labial sialadcnitis in HIV-infected patients. Jmtrna/o/
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Auloimmuniiy.
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Verlag. ‘I‘alal. N. (IYXY). Sjqqren’s Syndrome:
R. (19X6). Georg Thicmc
A Model Fo:o,
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London: Academic Press. Winchester. L.. Scully. C.. Prime, S. S. & Evcson. J. W. (IYXO). Chcilitis elandularis: a case affecting the upper lip. Orrr/
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The Author Professor C. Scully, PhD. MD, MDS, FDS, WI), MRCPalh Centrc for the Study of Oral Disease University Department of Oral Medicine. Surgery and Pathology Bristol Dental Hospital and School I_owcr Maudlin Street Bristol BSI 21-Y
66,179. Scully. C. (19X9). Oral parameters in the diagnosis of Sjogrcn‘s syndrome. Cliniculund Experimcntul Rheurnutology~ 7, I 13.
Correspondence
Scully.C.(
Paper received 13 December IYY I Accepted 24 February 1902
1991).0ralcomponentofSjogrcn’ssyndrome. G. Bctail and B.
t,eSyndrr,medeG‘ou~crot-Sjogr~n.
In:
and requests for offprints
to Professor
C. Scully
