Treatment of serologically negative Sjögren’s syndrome with tacrolimus: A case report

Abstract

We herein report an unusual case of primary Sjögren’s syndrome in a 38-year-old woman with typical clinical symptoms (joint pain, dry mouth, and positive Schirmer test) and immunoglobulin G positivity but negativity for antinuclear antibody and all antinuclear antibody spectrum antibodies. Emission computed tomography demonstrated normal ingestion but impaired secretion by the submandibular and bilateral parotid glands. Labial gland biopsy revealed chronic tissue inflammatory changes and Chisholm grade 4 lymphocyte infiltration, confirming primary Sjögren’s syndrome. The patient’s condition was successfully controlled by nonsteroidal treatment with tacrolimus. Patients presenting with chronic dry mouth should be examined by a Schirmer test, lip gland biopsy, and salivary gland emission computed tomography for possible Sjögren’s syndrome, even if serological autoantibodies are negative, to facilitate early intervention. Tacrolimus is a potential treatment option in patients intolerant of steroidal drugs.

Tacrolimus is effective in relapsed/refractory autoimmune cytopenias: results of a single-center retrospective study

“…Tacrolimus, an immunosuppressive agent, is usually used to prevent or treat graft-versus-host disease (GVHD) after organ transplantation. Recent studies showed that tacrolimus can also be used for refractory autoimmune-related systemic diseases, such as systematic lupus erythematosus and Sjogren's syndrome [5][6][7]. Recently, there have been a few case reports of successful treatment with tacrolimus for autoimmune cytopenias, such as refractory severe ITP after allogeneic bone marrow transplantation or refractory Evans syndrome [8,9].…”

Current Salivary Glands Biopsy Techniques: A Comprehensive Review

“…The studies were from four categories: before-after (pre-post) studies with no control group [ 15 , 16 , 17 , 18 , 19 ], observational cohort and cross-sectional studies [ 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 ], systematic reviews and meta-analyses [ 42 , 43 , 44 , 45 , 46 , 47 ], and non-randomized studies [ 48 , 49 , 50 , 51 , 52 ].…”

“…In addition, MSGB allows for histopathological diagnosis of other inflammatory or immune-reactive diseases of salivary glandular parenchyma, thanks to value scales and histo-morphologic criteria proposed by Chisholm and Mason, Greenspan and Daniels, and Tarpley. The diagnostic criteria of Chisholm and Mason, established in 1968, include five grades of glandular tissue impairment (0 to 4), based on the presence of peri-glandular lymphocytic infiltration [ 19 ]. Greenspan and Daniels in 1974 proposed a second classification, adding the concept of focus score (FS), defined as the number of lymphocyte foci in a 4 mm 2 area of glandular tissue, and focal lymphocytic sialadenitis (FLS) when FS > 1 is detected [ 52 ].…”