{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,1]],"date-time":"2026-03-01T06:56:26Z","timestamp":1772348186126,"version":"3.50.1"},"reference-count":23,"publisher":"PAGEPress Publications","issue":"2","license":[{"start":{"date-parts":[[2023,7,17]],"date-time":"2023-07-17T00:00:00Z","timestamp":1689552000000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Reumatismo"],"abstract":"<jats:p>Sjo\u0308gren\u2019s syndrome (SS) is a rare disease with the highest reported prevalence of 0.01-0.09%. Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) is another rare auto-immune disease (prevalence of 0.0009-0.01%). The co-occurrence of these two separate clinical entities in one patient might rarely be encountered as an overlap syndrome. Here, we present the case of a 60-year-old female patient who had complaints of headache, nausea, weakness, gritty sensation in her eyes, and dry mouth [unstimulated saliva production of 0.033 mL\/minute (normal; &gt;0.1 mL\/minute)] with a blood pressure of 190\/110 mmHg, hypertensive retinopathy, proteinuric kidney disease, positivity of myeloperoxidase anti-neutrophil cytoplasmic antibodies, anti-Ro-52, anti-Ro, and anti-La antibodies. Pauci-immune crescentic proliferative glomerulonephritis was found in a kidney biopsy and successfully treated with cyclophosphamide and methylprednisolone. The co-occurrence of these diseases was first reported in 1992 by Bo\u0308ttinger et al. Since then, nearly 37 cases of SS and AAV have been reported. By reporting this case of primary SS and AAV, we emphasize the importance of auto-antibody tests in searching for the etiology of patients with proteinuria.<\/jats:p>","DOI":"10.4081\/reumatismo.2023.1564","type":"journal-article","created":{"date-parts":[[2023,7,17]],"date-time":"2023-07-17T09:38:09Z","timestamp":1689586689000},"source":"Crossref","is-referenced-by-count":2,"title":["A case of Sj\u00f6gren syndrome and anti-neutrophil cytoplasmic antibody-associated vasculitis"],"prefix":"10.4081","volume":"75","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4957-465X","authenticated-orcid":false,"given":"K.","family":"Kaynar","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"B.","family":"G\u00fcvercin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0968-4253","authenticated-orcid":false,"given":"A.","family":"\u015eeng\u00f6r","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4882-352X","authenticated-orcid":false,"given":"S.","family":"Mungan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"2549","published-online":{"date-parts":[[2023,7,17]]},"reference":[{"key":"4506","unstructured":"Baer AN. Diagnosis and classification of Sj\u00f6gren\u2019s syndrome; 2022. Available from: https:\/\/www.uptodate.com\/contents\/diagnosis-and-classification-of-sjogrens-syndrome? Accessed: 26\/05\/2022."},{"key":"4507","unstructured":"Falk RJ, Merkel PA, King TE. Granulomatosis with polyangiitis and microscopic polyangiitis: clinical manifestations and diagnosis; 2022. Available from: https:\/\/www.uptodate.com\/contents\/granulomatosis-with-polyangiitis-and-microscopic-polyangiitis-clinical-manifestations-and-diagnosis? Accessed: 02\/06\/2022."},{"key":"4508","doi-asserted-by":"crossref","unstructured":"B\u00f6ttinger EP, Niles JL, Collins AB, McCluskey RT, Arnaout MA. Antineutrophil cytoplasmic autoantibody-associated vasculitis presenting as Sj\u00f6gren's syndrome. Arthritis Rheum 1992; 35: 1373-6.","DOI":"10.1002\/art.1780351120"},{"key":"4509","doi-asserted-by":"crossref","unstructured":"Guellec D, Cornec-Le Gall E, Groh M, Hachulla E, Karras A, Charles P, et al. ANCA-associated vasculitis in patients with primary Sj\u00f6gren's syndrome: detailed analysis of 7 new cases and systematic literature review. Autoimmun Rev 2015; 14: 742-50.","DOI":"10.1016\/j.autrev.2015.04.009"},{"key":"4510","unstructured":"Lu C, Wang R, Liu C, Zhou G. Antineutrophil cytoplasmic antibody-associated vasculitis accompanied by IgG4-tubulointerstitial nephritis with underlying Sj\u00f6gren syndrome: a case report and review of literature. Ann Clin Lab Sci 2021; 51: 883-9."},{"key":"4511","doi-asserted-by":"crossref","unstructured":"Iwafuchi Y, Morioka T, Oyama Y, Narita I. A case of smoldering antineutrophil cytoplasmic antibody-associated vasculitis development during the course of primary Sj\u00f6gren's syndrome. CEN Case Rep 2022; 11: 247-53.","DOI":"10.1007\/s13730-021-00663-1"},{"key":"4512","doi-asserted-by":"crossref","unstructured":"Fuchs PS, L\u00f6tscher J, Berkemeier CM, Hirsiger JR, Ghosh A, Li QZ, et al. Co-occurrence of ANCA-associated vasculitis and Sj\u00f6gren's syndrome in a patient with acromegaly: a case report and retrospective single-center review of acromegaly patients. Front Immunol 2020; 11: 613130.","DOI":"10.3389\/fimmu.2020.613130"},{"key":"4513","doi-asserted-by":"crossref","unstructured":"Mart\u00edn-Nares E, Zu\u00f1iga-Tamayo D, Hinojosa-Azaola A. Prevalence of overlap of antineutrophil cytoplasmic antibody associated vasculitis with systemic autoimmune diseases: an unrecognized example of poliautoimmunity. Clin Rheumatol 2019; 38: 97-106.","DOI":"10.1007\/s10067-018-4212-1"},{"key":"4514","doi-asserted-by":"crossref","unstructured":"Guibert F, Garnier AS, Wacrenier S, Piccoli G, Djema A, Gansey R, et al. Patients with ANCA-associated glomerulonephritis and connective tissue diseases: a comparative study from the Maine-Anjou AAV registry. J Clin Med 2019; 8: 1218.","DOI":"10.3390\/jcm8081218"},{"key":"4515","doi-asserted-by":"crossref","unstructured":"Yeturi S, Cronin M, Robin A, Lorna C, Rosenthal AK. Pauci-immune crescentic glomerulonephritis in connective tissue disease. Case Rep Rheumatol 2016; 2016: 9070487.","DOI":"10.1155\/2016\/9070487"},{"key":"4516","doi-asserted-by":"crossref","unstructured":"Kubota K, Ueno T, Mise K, Hazue R, Suwabe T, Kikuchi K, et al. ANCA-associated vasculitis in a patient with systematic sclerosis and Sj\u00f6gren's syndrome: a case report. Case Rep Nephrol Dial 2015; 5: 113-7.","DOI":"10.1159\/000381946"},{"key":"4517","doi-asserted-by":"crossref","unstructured":"Ghorbel IB, Belfeki N, Salem TB, Hamzaoui A, Khanfir M, Lamloum M, et al. Microscopic polyangiitis associated with primary biliary cirrhosis, Sj\u00f6gren's syndrome and Hashimoto's thyroiditis. Saudi J Kidney Dis Transpl 2015; 26: 359-62.","DOI":"10.4103\/1319-2442.152515"},{"key":"4518","unstructured":"Radhakrishnan J. Kidney disease in Sj\u00f6gren\u2019s syndrome;2022. Available from: https:\/\/www.uptodate.com\/contents\/kidney-disease-in-sjogrens-syndrome? Accessed: 30\/05\/2022."},{"key":"4519","doi-asserted-by":"crossref","unstructured":"Rodr\u00edguez-Muguruza S, Holgado S, Oliv\u00e9 A. Pulmonary hyalinizing granuloma associated with Sj\u00f6gren syndrome and ANCA MPO vasculitis. Joint Bone Spine 2015; 82: 71-2.","DOI":"10.1016\/j.jbspin.2014.06.005"},{"key":"4520","doi-asserted-by":"crossref","unstructured":"Suppiah R, Robson JC, Grayson PC, Ponte C, Craven A, Khalid S, et al. 2022 American college of rheumatology\/European alliance of associations for rheumatology classification criteria for microscopic polyangiitis. Arthritis Rheumatol 2022; 74: 400-6.","DOI":"10.1136\/annrheumdis-2021-221796"},{"key":"4521","unstructured":"Carvalho CRR, Kairalla RA, Baldi BG. Interstitial lung disease associated with Sj\u00f6gren syndrome: clinical manifestations, evaluation, and diagnosis; 2023. 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ANCA-associated pauci-immune crescentic glomerulonephritis complicating Sj\u00f6gren's syndrome. Nephrol Dial Transplant 1996; 11: 2313-5.","DOI":"10.1093\/oxfordjournals.ndt.a027157"},{"key":"4525","doi-asserted-by":"crossref","unstructured":"Kamachi M, Migita K, Tominaga M, Ichinose Y, Nakamura H, Origuchi T, et al. Sj\u00f6gren's syndrome complicated by MPO-ANCA positive crescentic glomerulonephritis. Nephrol Dial Transplant 1999; 14: 1033-4.","DOI":"10.1093\/ndt\/14.4.1033"},{"key":"4526","doi-asserted-by":"crossref","unstructured":"Kawashiri SY, Tamai M, Yamasaki S, Nakamura H, Kakugawa T, Sakamoto N, et al. A case of Sj\u00f6gren syndrome with pulmonary amyloidosis complicating microscopic polyangiitis. Mod Rheumatol 2011; 21: 646-50.","DOI":"10.1007\/s10165-011-0443-6"},{"key":"4527","doi-asserted-by":"crossref","unstructured":"Tatsumi H, Tateno S, Hiki Y, Kobayashi Y. Crescentic glomerulonephritis and primary Sj\u00f6gren's syndrome. Nephron 2000; 86: 505-6.","DOI":"10.1159\/000045843"},{"key":"4528","doi-asserted-by":"crossref","unstructured":"Wang WJ, Wu HS, Chu TS. Anti-neutrophil cytoplasmic antibody-associated pauci-immune crescentic glomerulonephritis complicating Sj\u00f6gren's syndrome. J Formos Med Assoc 2011; 110: 473-7.","DOI":"10.1016\/S0929-6646(11)60070-3"}],"container-title":["Reumatismo"],"original-title":[],"link":[{"URL":"https:\/\/www.reumatismo.org\/index.php\/reuma\/article\/download\/1564\/981","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.reumatismo.org\/index.php\/reuma\/article\/download\/1564\/981","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,7,17]],"date-time":"2023-07-17T09:38:18Z","timestamp":1689586698000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.reumatismo.org\/index.php\/reuma\/article\/view\/1564"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,7,17]]},"references-count":23,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2023,7,17]]}},"URL":"https:\/\/doi.org\/10.4081\/reumatismo.2023.1564","relation":{},"ISSN":["2240-2683","0048-7449"],"issn-type":[{"value":"2240-2683","type":"electronic"},{"value":"0048-7449","type":"print"}],"subject":[],"published":{"date-parts":[[2023,7,17]]}}}