Diagnosis of Systemic Diseases in Patients Presenting with Sicca Syndrome Using a Minimally-Invasive Minor Salivary Gland Biopsy: Analysis of 901 Patients

Soledad Retamozo^1,2,3, Pilar Brito-Zerón^1,4,5, Alejandro Alvarellos³, Veronica Saurit³, Ana C. Alvarez³, Maria Soledad Fiorentino³, Nadia Benzaquén³, Juan Pablo Pirola³, Diego Baenas³, Maria Jezabel Haye Salinas³, Albert Bove⁶, Isabel Sánchez-Berná⁷, César Morcillo⁸, Francisco Caeiro³ and Manuel Ramos-Casals^2,5,9, ¹Laboratory of Systemic Autoimmune Diseases “Josep Font”, CELLEX, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Department of Systemic Autoimmune Diseases, ICMID, Hospital Clinic, Barcelona, Barcelona, Spain, ²Sjögren Syndrome Research Group (AGAUR), Barcelona, Spain, ³Rheumatology Unit, Hospital Privado Centro Médico de Córdoba, Postgraduate Career of Rheumatology Catholic University of Córdoba, Fundación para las Ciencias Biomédicas de Córdoba (FUCIBICO), Cordoba, Argentina, ⁴Autoimmune Diseases Unit, Department of Medicine, Hospital CIMA- Sanitas, Barcelona., Bacelona, Spain, ⁵Laboratory of Systemic Autoimmune Diseases “Josep Font”, CELLEX, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Department of Systemic Autoimmune Diseases, ICMID, Hospital Clinic, Barcelona, Spain, Barcelona, Spain, ⁶Department of Autoimmune Diseases, ICMiD, Hospital Clínic, Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain, ⁷Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, Institutd’Investigacions Biomèdiques August Pi iSunyer (IDIBAPS), Department of Autoimmune Diseases, ICMiD, Barcelona, Spain, ⁸Department of Internal Medicine, Hospital CIMA-Sanitas, Barcelona, Spain, Barcelona, Spain, ⁹Department of Medicine, University of Barcelona, Barcelona, Spain., Barcelona, Spain

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords: SICCA, Sjogren's syndrome and biopsies

Session Information

Date: Tuesday, November 15, 2016

Title: Sjögren's Syndrome - Poster II: Clinical Science

Session Type: ACR Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose: Sicca syndrome is a clinical presentation that is common for several systemic diseases that may infiltrate the exocrine glands. The most frequent disease is Sjögren’s syndrome (SS), but other systemic diseases such as sarcoidosis, amyloidosis of IgG4-related disease may also infiltrate salivary glands. The objective was to analyze the safety and usefulness of the minimally-invasive biopsy of minor salivary glands in patients presenting with sicca syndrome suspecting a systemic disease.

Methods: We present a retrospective analysis of 901 patients presenting with sicca syndrome (xerostomia, xerophthalmia, positive ocular tests and/or parotid scintigraphy, overwhelmingly negative for anti-Ro/La antibodies) in whom a minimally-invasive biopsy of minor salivary glands was carried out between January 2005 and June 2016. All biopsy samples were obtained with the same technique and following the same study protocol, which included the evaluation of cumulative focus score of lymphoplasmacytic infiltration (Chisholm Mason score, CMs), together with investigation of granuloma, amyloid, IgG4-related disease or lipids.

Results: All biopsies but six disclosed salivary gland tissue. There are 793 (88%) women and 108 (12%) men, with a mean age of 54,46 years (range 14-86). The main histopathological diagnosis included non-specific chronic sialoadenitis (NSCS, CMs=1-2) in 429 (48%) patients, focal lymphocytic sialoadenitis diagnostic of Sjögren syndrome (FLS, CMs=3-4) in 255 (28%), normal glandular tissue (CMs=0) in 148 (16%) and chronic atrophic sialadenitis (CAS, CMs unclassifiable) in 34 (4%) patients. Other infiltrative diseases included lipoid infiltration (n=10), amyloidosis (n=4), IgG4-related disease (n=3) and sarcoidosis (n=1). The highest mean age was found in patients with CAS (66,35 years), followed by those with NSCS (54,66 years), FLS (54,04 years) and normal result (50,82 years) (p<0.001). Patients diagnosed with FLS were more frequently women (91% vs 87%, p=0.05), had a higher frequency of abnormal ocular tests (91% vs 66%, p<0.001), a higher frequency of ANA (59% vs 36%, p<0.001), RF (41% vs 18%, p<0.001), anti-Ro (28% vs 7%, p<0.001) and anti-La (11% vs 2%, p<0.001), and a higher frequency of associated autoimmune diseases (20% vs 10%, p<0.001). The percentage of patients diagnosed with SS varied according to the association or not with other autoimmune/viral diseases: 27% of patients with no associated diseases vs 34% of those with organ-specific autoimmune diseases, 42% of those with chronic viral diseases and 50% of those with other systemic autoimmune diseases (p<0.05). Abnormal ocular tests (p<0.001), ANA (p<0.001), RF (p<0.001) and anti-Ro (p<0.001) remained independent variables significantly associated with SS after adjustment by age and gender.

Conclusion: Minimally-invasive biopsy of minor salivary glands is a simple, safe, and reliable tool for the diagnosis of infiltrative systemic diseases of exocrine glands, overwhelmingly Sjögren syndrome but also amyloidosis IgG4-related disease and sarcoidosis. Lack of severe lymphocytic infiltration and atrophic histopathological data closely correlated with an older age.

Disclosure: S. Retamozo, None; P. Brito-Zerón, None; A. Alvarellos, None; V. Saurit, None; A. C. Alvarez, None; M. S. Fiorentino, None; N. Benzaquén, None; J. P. Pirola, None; D. Baenas, None; M. J. Haye Salinas, None; A. Bove, None; I. Sánchez-Berná, None; C. Morcillo, None; F. Caeiro, None; M. Ramos-Casals, None.

To cite this abstract in AMA style:

Retamozo S, Brito-Zerón P, Alvarellos A, Saurit V, Alvarez AC, Fiorentino MS, Benzaquén N, Pirola JP, Baenas D, Haye Salinas MJ, Bove A, Sánchez-Berná I, Morcillo C, Caeiro F, Ramos-Casals M. Diagnosis of Systemic Diseases in Patients Presenting with Sicca Syndrome Using a Minimally-Invasive Minor Salivary Gland Biopsy: Analysis of 901 Patients [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/diagnosis-of-systemic-diseases-in-patients-presenting-with-sicca-syndrome-using-a-minimally-invasive-minor-salivary-gland-biopsy-analysis-of-901-patients/. Accessed .

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