Background:Patients with primary Sjogren’s syndrome (pSS) are at high risk of lymphoma. Signs of lymphomas in ACA+pSS were not widely reported, the descriptions were single [1, 2]. According to pSS treatment recommendations [3, 4, 5, 6], glandular forms require only symptomatic treatment, in the absence of systemic manifestations systemic therapy is not required.Objectives:to evaluate the incidence of lymphomas in ACA+pSS; to compare clinical and laboratory manifestations in 2 groups of ACA+pSS: with and without lymphomas.Methods:we examined 119 ACA+pSS patients. We evaluated both glandular and systemic manifestations. We diagnosed lymphomas based on study of biopsy specimens of affected organs.Results:MALT-lymphomas diagnosed in 19 (16%) ACA+pSS patients. Persistent enlargement of parotid salivary glands, decreased C4-complement, decreased CD19+cells in peripheral blood, cryoglobulinemic vasculitis, lymphoid infiltration of minor salivary glands (MSG) more than 200 cells in focus, severe xerostomia and xerophthalmia more often detected in patients with ACA+pSS+lymphomas. RF and antiRo were found in only 25% of patients with lymphomas and its frequency did not differ between groups. Only 20% of patients with lymphoma had monoclonal immunoglobulins secretion and its frequency also did not differ between groups. Anemia, leukopenia, thrombocytopenia, increased ESR, hypergammaglobulinemia, increased levels of immunoglobulins were found in the study groups with the same frequency. There were no differences in the frequency of detection of recurrent parotitis, lymphadenopathy, Raynaud phenomenon, arthritis/arthralgia, pleuritis/pericarditis, neuropathy, nephritis, hypergammaglobulinemic purpura.Conclusion:in the present study in patients with lymphomas, the course of pSS was characterized by minimal systemic manifestations and low immunological activity, but severe glandular manifestations with the development of late stage damages of salivary and lacrimal glands, severe lymphoid infiltration of MSG, which led to the frequent occurrence of MALT-lymphomas. Thus, in patients with pSS, regardless of the type of detected antibodies (antiRo/La, ACA, RF or others), regardless of the presence or absence of systemic manifestations, damage of salivary and lacrimal gland progresses, which in some cases leads to the development of lymphomas, therefore, therapy that can prevent this complication should be initiated immediately after diagnosis of pSS is confirmed. The signs of lymphoproliferation detected in the present study should be evaluated in all ACA+pSS patients for early diagnosis of lymphoma.References:[1]Baldini C, Mosca M, Della Rossa A, Pepe P, Notarstefano C, Ferro F, Luciano N, Talarico R, Tani C, Tavoni AG, Bombardieri S. Overlap of ACA-positive systemic sclerosis and Sjögren’s syndrome: a distinct clinical entity with mild organ involvement but at high risk of lymphoma[2]Dhiraj Gulati & Irving Kushner & Elizabeth File & Marina Magrey. Primary Sjogren’s syndrome with anticentromere antibodies—a clinically distinct subset. ClinRheumatol (2010) 29:789–791 DOI 10.1007/s10067-009-1359-9.[3]F. Vivino et al. Sjogren’s syndrome: An update on disease pathogenesis, clinical manifestations and treatment. Clinical Immunology 203 (2019) 81–121.https://doi.org/10.1016/j.clim.2019.04.009[4]Xavier Mariette et al. Primary Sjögren’s Syndrome. N Engl J Med 2018; 378:931-9. DOI: 10.1056/NEJMcp1702514[5]Xiaoyun Chen et al. Advances in the diagnosis and treatment of Sjogren’s syndrome. Clinical Rheumatology (2018) 37:1743–1749.https://doi.org/10.1007/s10067-018-4153-8[6]Ana-Luisa Stefanski et al. The Diagnosis and Treatment of Sjögren’s Syndrome. Deutsches Ärzteblatt International | Dtsch Arztebl Int 2017; 114: 354–61Disclosure of Interests:None declared
Current State of Knowledge on Primary Sjögren’s Syndrome, an Autoimmune Exocrinopathy
