https://doi.org/10.4081/reumatismo.2023.1564
A case of Sjögren syndrome and anti-neutrophil cytoplasmic antibody-associated vasculitis
Submitted: 8 February 2023
Accepted: 26 May 2023
Published: 17 July 2023
Accepted: 26 May 2023
Abstract Views: 641
PDF: 445
Publisher's note
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
Authors
Sjögren’s 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; >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 Böttinger 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.
Baer AN. Diagnosis and classification of Sjögren’s syndrome; 2022. Available from: https://www.uptodate.com/contents/diagnosis-and-classification-of-sjogrens-syndrome? Accessed: 26/05/2022.
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.
Böttinger EP, Niles JL, Collins AB, McCluskey RT, Arnaout MA. Antineutrophil cytoplasmic autoantibody-associated vasculitis presenting as Sjögren's syndrome. Arthritis Rheum 1992; 35: 1373-6.
DOI: https://doi.org/10.1002/art.1780351120
Guellec D, Cornec-Le Gall E, Groh M, Hachulla E, Karras A, Charles P, et al. ANCA-associated vasculitis in patients with primary Sjögren's syndrome: detailed analysis of 7 new cases and systematic literature review. Autoimmun Rev 2015; 14: 742-50.
DOI: https://doi.org/10.1016/j.autrev.2015.04.009
Lu C, Wang R, Liu C, Zhou G. Antineutrophil cytoplasmic antibody-associated vasculitis accompanied by IgG4-tubulointerstitial nephritis with underlying Sjögren syndrome: a case report and review of literature. Ann Clin Lab Sci 2021; 51: 883-9.
Iwafuchi Y, Morioka T, Oyama Y, Narita I. A case of smoldering antineutrophil cytoplasmic antibody-associated vasculitis development during the course of primary Sjögren's syndrome. CEN Case Rep 2022; 11: 247-53.
DOI: https://doi.org/10.1007/s13730-021-00663-1
Fuchs PS, Lötscher J, Berkemeier CM, Hirsiger JR, Ghosh A, Li QZ, et al. Co-occurrence of ANCA-associated vasculitis and Sjögren's syndrome in a patient with acromegaly: a case report and retrospective single-center review of acromegaly patients. Front Immunol 2020; 11: 613130.
DOI: https://doi.org/10.3389/fimmu.2020.613130
Martín-Nares E, Zuñiga-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: https://doi.org/10.1007/s10067-018-4212-1
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: https://doi.org/10.3390/jcm8081218
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: https://doi.org/10.1155/2016/9070487
Kubota K, Ueno T, Mise K, Hazue R, Suwabe T, Kikuchi K, et al. ANCA-associated vasculitis in a patient with systematic sclerosis and Sjögren's syndrome: a case report. Case Rep Nephrol Dial 2015; 5: 113-7.
DOI: https://doi.org/10.1159/000381946
Ghorbel IB, Belfeki N, Salem TB, Hamzaoui A, Khanfir M, Lamloum M, et al. Microscopic polyangiitis associated with primary biliary cirrhosis, Sjögren's syndrome and Hashimoto's thyroiditis. Saudi J Kidney Dis Transpl 2015; 26: 359-62.
DOI: https://doi.org/10.4103/1319-2442.152515
Radhakrishnan J. Kidney disease in Sjögren’s syndrome;2022. Available from: https://www.uptodate.com/contents/kidney-disease-in-sjogrens-syndrome? Accessed: 30/05/2022.
Rodríguez-Muguruza S, Holgado S, Olivé A. Pulmonary hyalinizing granuloma associated with Sjögren syndrome and ANCA MPO vasculitis. Joint Bone Spine 2015; 82: 71-2.
DOI: https://doi.org/10.1016/j.jbspin.2014.06.005
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: https://doi.org/10.1136/annrheumdis-2021-221796
Carvalho CRR, Kairalla RA, Baldi BG. Interstitial lung disease associated with Sjögren syndrome: clinical manifestations, evaluation, and diagnosis; 2023. Available from: https://www.uptodate.com/contents/interstitial-lung-disease-associated-with-sjogren-syndrome-clinical-manifestations-evaluation-and-diagnosis? Accessed: 26/04/2023.
Uffmann M, Kiener HP, Bankier AA, Baldt MM, Zontsich T, Herold CJ. Lung manifestation in asymptomatic patients with primary Sjögren syndrome: assessment with high resolution CT and pulmonary function tests. J Thorac Imaging 2001; 16: 282-9.
DOI: https://doi.org/10.1097/00005382-200110000-00009
Guillot X, Solau-Gervais E, Coulon A, Debiais F. Sjögren's syndrome with ANCA-associated crescentic extramembranous glomerulonephritis. Joint Bone Spine 2009; 76: 188-9.
DOI: https://doi.org/10.1016/j.jbspin.2008.05.010
Hernández JL, Rodrigo E, De Francisco AL, Val F, González-Macías J, Riancho JA. ANCA-associated pauci-immune crescentic glomerulonephritis complicating Sjögren's syndrome. Nephrol Dial Transplant 1996; 11: 2313-5.
DOI: https://doi.org/10.1093/oxfordjournals.ndt.a027157
Kamachi M, Migita K, Tominaga M, Ichinose Y, Nakamura H, Origuchi T, et al. Sjögren's syndrome complicated by MPO-ANCA positive crescentic glomerulonephritis. Nephrol Dial Transplant 1999; 14: 1033-4.
DOI: https://doi.org/10.1093/ndt/14.4.1033
Kawashiri SY, Tamai M, Yamasaki S, Nakamura H, Kakugawa T, Sakamoto N, et al. A case of Sjögren syndrome with pulmonary amyloidosis complicating microscopic polyangiitis. Mod Rheumatol 2011; 21: 646-50.
DOI: https://doi.org/10.1007/s10165-011-0443-6
Tatsumi H, Tateno S, Hiki Y, Kobayashi Y. Crescentic glomerulonephritis and primary Sjögren's syndrome. Nephron 2000; 86: 505-6.
DOI: https://doi.org/10.1159/000045843
Wang WJ, Wu HS, Chu TS. Anti-neutrophil cytoplasmic antibody-associated pauci-immune crescentic glomerulonephritis complicating Sjögren's syndrome. J Formos Med Assoc 2011; 110: 473-7.
DOI: https://doi.org/10.1016/S0929-6646(11)60070-3
How to Cite
Kaynar, K., Güvercin, B., Şengör, A., & Mungan, S. (2023). A case of Sjögren syndrome and anti-neutrophil cytoplasmic antibody-associated vasculitis. Reumatismo, 75(2). https://doi.org/10.4081/reumatismo.2023.1564
Copyright (c) 2023 the Author(s)
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Authors who publish with this journal agree to the following terms:
- Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
- Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.
- Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work.