Cutaneous mucosa-associated lymphoid tissue lymphoma complicating Sjögren's syndrome:A case report and review of literature

BACKGROUND The association of Sjögren's syndrome(SS)and lymphoma is similar.Mucosaassociated lymphoid tissue(MALT)or extranodal marginal zone B-cell lymphoma was the most common lymphomatous histology in SS patients.MALT in SS patients is frequently located in the parotid gland,while MALT lymphoma of the skin with SS is an exceedingly rare entity that needs to be recognized.CASE SUMMARY A 60-year-old woman presented with a 3-year history of progressive dry mouth associated with a 1-year history of enlarging cutaneous nodules.Physical examination revealed two hard subcutaneous nodules on her right lower leg.The results of Schirmer’s test were positive,despite the absence of dry eyes.Labial salivary gland biopsy revealed lymphocytic infiltration and chronic inflammation with a focus score of 2.The patient was diagnosed with SS.She underwent resection of one cutaneous nodule,and histopathological analysis identified the nodule as MALT lymphoma.Her dry mouth symptoms improved,and the nodules decreased after 6 mo of treatment with hydroxychloroquine sulfate and chemotherapy(thalidomide,cyclophosphamide,and dexamethasone).CONCLUSION Lymphoma is a severe complication of SS,shown by the reported unique case of cutaneous MALT lymphoma with SS.

Identification and functional analysis of shared gene signatures between systemic lupus erythematosus and Sjogren's syndrome

Background:Systemic lupus erythematosus(SLE)is an autoimmune disease that can affect multiple systems.Sjogren's syndrome(SS)is an autoimmune disease that may be primary SS(pSS)or occur together with other autoimmune diseases,including SLE.This study aimed to explore the shared gene signatures in SLE and pSS.Methods:Gene expression data sets of SLE(GSE50772 and GSE81622)and pSS(GSE84844 and GSE48378)were obtained and analyzed for differentially expressed genes(DEGs)in peripheral blood mononuclear cells(PBMCs).A protein–protein interaction(PPI)network was constructed.Gene ontology(GO)and KEGG pathway enrichment analysis were carried out for the DEGs.Results:We screened 232 and 110 DEGs from the SLE and pSS data sets,respectively.We found 32 shared DEGs,which were all upregulated in patients compared with controls.Among these 32 DEGs,11 genes showed a more than twofold change in all data sets(IFI27,IFI44L,RSAD2,IFIT1,IFI44,USP18,IFI6,HERC5,EPSTI1,OAS1,and OAS3).PPI analysis showed that 29 genes interacted with each other.GO analysis showed that these 32 shared DEGs were mainly enriched in biological processes associated with the type Ⅰ interferon signaling pathway,defense response to viruses,response to viruses,negative regulation of viral genome replication,and the immune response.Kyoto Encyclopedia of Genes and Genomes pathway analysis showed that these 32 DEGs were related to virus infection.Conclusion:This study showed that alterations to biological processes associated with the response to virus infection play critical roles in both SLE and pSS.