Learning objectives
Clinical scenario
Primary Sjögren's syndrome (pSS) is a chronic systemic autoimmune disease with estimated prevalence between 0.5% and 1% of the general population, predominantly affecting middle-aged women with a female to male ratio of 9:1 [1]. Clinical features range from glandular related symptoms (i.e. mucosal dryness) to systemic multi-organ manifestations and lymphoproliferative disorders. In this regard, it has been reported that B-cell lymphomas occur in 5%-10% of pSS patients, and the risk increases by 2,2% per year of age [2], with a 7-19 fold increased...
Background
Patients
Thirteen consecutive patients with definite (ACR-EULAR classification criteria) or clinically suspected pSS and with clinical and/or laboratoristic and/or US findings suspicious for MALT lymphoma were referred to our institute to undergo a US evaluation of their major SGs followed by US-guided CNB. Biopsy was performed on the parotid gland in nine patients and on the submandibular gland in four cases. The final diagnoses were B cell lymphoma in 5/13, lymphoepithelial sialadenitis in 1/13, other sialadenitis (granulomatous consistent with sarcoidosis, IgG4-related disease, chronic sclerosing, diffuse...
Findings and procedure details
Following the procedure that we designed, only 46,1% of patients reported transient complications while none reported permanent complications, and the pathologic diagnosis was achieved in all cases. For comparison, in the control group that underwent surgical biopsy, 92,3% patients reported transient complications, while 15,4% reported permanent complications – facial nerve damage.
Conclusion
Feasibility of CNB
US-guided CNB is a valuable tool in the assessment of major SGs. With the procedure we developed together with our multidisciplinary team, following the precautions we described step-by-step, it provides enough tissue for pathological diagnosis with an acceptable rate of transient complications and, in our series, without permanent complications.
Personal information and conflict of interest
F. Tulipano Di Franco:
Nothing to disclose
M. Lorenzon:
Nothing to disclose
A. Zabotti:
Nothing to disclose
S. Zandonella Callagher:
Nothing to disclose
E. Pegolo:
Nothing to disclose
M. Robiony:
Nothing to disclose
S. De Vita:
Nothing to disclose
R. Girometti:
Nothing to disclose
C. Zuiani:
Nothing to disclose
References
[1] Mavragani CP, Moutsopoulos HM. Sjögren’s Syndrome.Annual Review of Pathology: Mechanisms of Disease. 2014;9(1):273-285. doi:10.1146/annurev-pathol-012513-104728
[2] Chiu Y-H, Chung C-H, Lin K-T, et al. Predictable biomarkers of developing lymphoma in patients with Sjögren syndrome: a nationwide population-based cohort study.Oncotarget. 2017;8(30):50098-50108. doi:10.18632/oncotarget.15100
[3] Fragkioudaki S, Mavragani CP, Moutsopoulos HM. Predicting the risk for lymphoma development in Sjogren syndrome.Medicine. 2016;95(25):e3766. doi:10.1097/md.0000000000003766
[4] Nocturne G, Pontarini E, Bombardieri M, Mariette X. Lymphomas complicating primary Sjögren’s syndrome: from autoimmunity to lymphoma.Rheumatology. Published online March 5, 2019. doi:10.1093/rheumatology/kez052
[5] Retamozo...