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GAZZETTA MEDICA ITALIANA ARCHIVIO PER LE SCIENZE MEDICHE

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Gazzetta Medica Italiana Archivio per le Scienze Mediche 2004 August;163(4):107-10

language: English

Sar­coi­do­sis com­pli­cat­ing ­long-stand­ing pri­mary ­Sjögren’s syn­drome. A ­case ­report

Fietta P. 1, Manganelli P. 1, Martella E. M. 2, Quaini F. 3

1 Unit of Rheu­ma­tol­o­gy and Inter­nal Med­i­cine ­Osteo-Artic­u­lar Depart­ment, Gen­er­al Hos­pi­tal of Par­ma, Ita­ly;
2 Sec­tion of His­tol­o­gy and Anat­o­mop­a­thol­o­gy Depart­ment of Labor­a­to­ry Pathol­o­gy, Uni­ver­sity of Par­ma, Par­ma, Ita­ly;
3 Depart­ment of Inter­nal Med­i­cine and Bio­med­i­cal Sci­enc­es, Uni­ver­sity of Par­ma, Par­ma, Ita­ly


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A 63-year-old wom­an, suf­fer­ing ­from long­stand­ing pri­mary ­Sjögren’s syn­drome (pSS), abrupt­ly devel­oped super­fi­cial lym­pha­de­no-­pathies. A ­lymph ­node biop­sy ­revealed efface­ment of the ­nodal archi­tec­ture by mul­ti­ple non­case­at­ing gra­nu­lo­mas, con­sti­tut­ed by epithe­li­oid and mul­ti­nu­cle­at­ed ­giant ­cells, sur­round­ed by a ­large num­ber of lym­pho­cytes ­with a pre­dom­i­nance of CD4+ T help­er ­cells. ­High res­o­lu­tion com­put­ed tomog­ra­phy of the ­chest ­showed mul­ti­ple bilat­er­al ­small subpleu­ral nod­ules and ­small are­as of con­sol­i­da­tion. The diag­no­sis of sar­coi­do­sis com­pli­cat­ing pSS was ­made. Cor­ti­cos­ter­oid ther­a­py led to a ­prompt and com­plete regres­sion of ­both lym­phad­e­nop­a­thies and pul­mo­nary find­ings. The appear­ance of lym­phad­e­nop­a­thies in pSS ­patients ­requires a care­ful clin­i­cal exam­ina­tion, due to the ­high ­risk of lym­pho­ma devel­op­ment; how­ev­er, the case ­here report­ed under­lines ­that sar­coi­do­sis has ­always to be includ­ed in the dif­fe­ren­tial diag­no­sis of ­lymph ­node enlarge­ment in pSS.

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