Clinical Features And Treatment Outcomes Of IgG4-related Sclerosing Sialadenitis: A Preliminary Study

Objective: To discuss the clinical characristics of IgG4-related sclerosing sialadenitis.A comparative analysis of IgG4-related sclerosing sialadenitis and Sj gren’ssyndrome was made to find out the differences and similarities in the clinical features.To observe and evaluate the efficacy of glucocorticoid therapy to IgG4-relatedsclerosing sialadenitis patients.Methods: All patients with salivary glands swelling were referred to the Departmentof Oral and Maxillofacial Surgery, Shanghai Ninth People’s Hospital AffiliatedShanghai Jiao Tong University School of Medicine, between December2012andFebrary2014. The patients underwent a series exams to identify the diagnosis.Clinical information of patients who were finally diagnosed as IgG4-relatedsclerosing sialadenitis was collected comprehensively. We retrospectively studied theclinical examination, serology, pathology and imaging features of these patients andcompared them with those of Sj gren’s syndrome patients’. The patients were treatedby corticosteroids and followed-up for more than3months. We measured the clinicalsymptoms, degree of dry mouth and some key serologic items to evaluate the efficacyof glucocorticoid therapy.Results: We finally diagnosed25patients as IgG4-related sclerosing sialadenitis with10female and15male. The mean age was (59±14.1) years old. The results of clinicalfeatures showed that all of the patients presented persistent, symmetrical, painlessbilateral salivary glands swelling with mild to modest dry mouth, elevated levels ofserum IgG4and/or IgG4-positive plasmacytes infiltration with tissue fibrosis. Theautoantibodies were all negative. The sonographic appearance was classified into3types: localized tumor-forming type, diffuse focal type and a combination type. MRIindicated diffuse lesion in salivary glands.Compared with IgG4-related sclerosing sialadenitis,the patients with Sj gren’ssyndrome are mostly mid-aged women. Only10%patients presented gland swelling.Most of them suffered from modest to severe dry mouth. The mean of Saxon test result was （1.49±0.80）g/2min. About94%and58%patients showed SSA andSSB positive. RF was found elevated in74%patients while IgG4was found normalin all patients. Focal lymphocytic sialoadenitis with at least one focus score was foundin about90%SS patients and16%IgG4-related sclerosing sialadenitis patients.We obtained17patients follow-up data after3months’ steroids treatment. All ofthe patients had an obvious reduction in glandular swelling. Two patients had acomplete recovery from the glands swelling. However two patients had a replaseduring the treatment. The replase was under control by prescribing30mg/d prednisone.The treatment led to a relief in dry mouth symptom in12patients. The mean result ofSaxon test was improved from2.17g/2min to2.57g/2min. Both serum IgG4and IgGlevels decreased significantly. The median of serum IgG4concentration decreasedfrom11.8g/L to8.33g/L and the serum IgG concentration decreased from19.1g/Lto15.1g/L.Conclusion: IgG4-related sclerosing sialadenitis often occurs in mid-agedpeople. The patients present persistent, symmetrical, painless bilateral salivary glandsswelling with dry mouth and dysfunction of gland secretion. Elevated levels of serumIgG4and IgG can be observed. The autoantibodies were all negative. Most patientsalso present elevated levels of serum IgE, β2-MG and ESR. IgG4-positiveplasmacytes infiltration with tissue fibrosis. The autoantibodies were all negative. Thepathology manifestation show preservation of lobular structure, loss of acinar tissuewith lymphoplasmacytic composed of lymphocytes and plasma cells (IgG4+)infiltrated and presence of striking fibrosis.The sonographic appearance was classifiedinto3types: localized tumor-forming type, diffuse focal type and a combination type.MRI indicated diffuse lesion in salivary glands. A comprehensive understanding ofthe clinical, serological, pathological and imaging presentation is the key to diagnose.There are many differences between IgG4-related sclerosing sialadenitis and SSin all aspects. An accurate diagnosis and a reasonable treatment plan should be madeaccording to medical condition.Small-dose glucocorticoid treatment is effective to IgG4-related sclerosingsialadenitis. It helps reduce the swelling glands as well as serum IgG4and IgG leveland improve the gland secretion leading to a relief of dry mouth.