Multisystem Sarcoidosis Combined with Sjögren's Syndrome: A Case
Report and Literature Review

Xiaoran      Li; Jing      Wu; Guangan      Ruan; Yu-Chen      Chen

doi:10.2174/1573405619666230215150154

Abstract

Background: Sarcoidosis is a systemic non-caseous necrotizing granulomatous disease with unknown etiology. It can involve multiple organs throughout the body, most commonly affecting lungs and/or bilateral hilar lymph nodes. Sjögren's syndrome is a multi-system autoimmune disease. The main clinical symptoms include dry mouth and dry eyes. The combination of the two diseases with the involvement of multiple systems is very rare, and the final diagnosis is mainly based on the comprehensive judgment of clinical history, imaging manifestations and pathological examination.

Case Presentation: We report a case of multiple sarcoidosis (lung, hilar, mediastinal, inguinal, liver, and spleen) with Sjögren syndrome. The patient had a dry mouth, dry eyes, and bilateral parotid gland enlargement. The first computed tomography (CT) scan of the chest and abdomen showed multiple nodules in the lungs, multiple enlarged lymph nodes, and low-density shadows in the liver and spleen. After a one-year interval, the re-examination showed that the lung lesions increased with bead-like changes, and the lymph nodes shrunk. Through pathological puncture and comprehensive judgment, considering the coexistence of the two diseases, the patient improved after hormone therapy and was finally diagnosed.

Conclusion: Multisystem sarcoidosis combined with Sjögren's syndrome has rarely been reported in the literature. This case has multiple imaging examinations, pathological data and a follow-up review after treatment. The dynamic changes in different periods will help us to better understand the situation of sarcoidosis and explore the connection between the two diseases so as to reduce misdiagnosis.

[1]
Amschler K, Seitz CS. Cutaneous manifestations of sarcoidosis. Z Rheumatol  2017; 76(5): 382-90.
 [http://dx.doi.org/10.1007/s00393-017-0290-8] [PMID:  28314915]

[2]
Wu CH, Chung PI, Wu CY, et al. Comorbid autoimmune diseases in patients with sarcoidosis: A nationwide case-control study in Taiwan. J Dermatol  2017; 44(4): 423-30.
 [http://dx.doi.org/10.1111/1346-8138.13654] [PMID:  27786368]

[3]
Seror R, Baron G, Camus M, et al. Development and preliminary validation of the Sjögren’s Tool for Assessing Response (STAR): A consensual composite score for assessing treatment effect in primary Sjögren’s syndrome. Ann Rheum Dis  2022; 81(7): 979-89.
 [http://dx.doi.org/10.1136/annrheumdis-2021-222054] [PMID:  35393271]

[4]
Brito-Zerón P, Pérez-Alvarez R, Feijoo-Massó C, et al. Coexistence of immune-mediated diseases in sarcoidosis. Frequency and clinical significance in 1737 patients. Joint Bone Spine  2021; 88(6): 105236.
 [http://dx.doi.org/10.1016/j.jbspin.2021.105236] [PMID:  34116201]

[5]
Batu ED, Demir S, Bilginer Y, Ozen S. Macrophage activation syndrome in pediatric Sjögren’s syndrome. Semin Arthritis Rheum  2022; 53: 151977.
 [http://dx.doi.org/10.1016/j.semarthrit.2022.151977] [PMID:  35182962]

[6]
Valeyre D, Prasse A, Nunes H, Uzunhan Y, Brillet PY, Müller-Quernheim J. Sarcoidosis. Lancet  2014; 383(9923): 1155-67.
 [http://dx.doi.org/10.1016/S0140-6736(13)60680-7] [PMID:  24090799]

[7]
Shi TY, Wen XH, Shi XH, et al. Associations between sarcoidosis, autoimmune diseases, and autoantibodies: A single-center retrospective study in China. Clin Exp Med  2022; 22(2): 277-83.
 [http://dx.doi.org/10.1007/s10238-021-00737-5] [PMID:  34191227]

[8]
Santiago T, Santiago M, Rovisco J, et al. Coexisting primary Sjögren’s syndrome and sarcoidosis: Coincidence, mutually exclusive conditions or syndrome? Rheumatol Int  2014; 34(11): 1619-22.
 [http://dx.doi.org/10.1007/s00296-014-3024-0] [PMID:  24770372]

[9]
Drosos AA, Voulgari PV, Psychos DN, Tsifetaki N, Bai M. Sicca syndrome in patients with sarcoidosis. Rheumatol Int  1999; 18(5-6): 177-80.
 [http://dx.doi.org/10.1007/s002960050081] [PMID:  10399792]

[10]
Dilektasli AG, Cetinoglu ED, Durmus E, Uzaslan E, Karadag M. Sarcoidosis and Sjögren’s syndrome: Mimicry or coexistence? Kaohsiung J Med Sci  2015; 31(10): 548-9.
 [http://dx.doi.org/10.1016/j.kjms.2015.07.006] [PMID:  26520695]

[11]
Huitema MP, Mathijssen H, Bakker ALM, et al. Four-year survival rate in pulmonary sarcoidosis with extensive pulmonary hypertension screening. Respir Med  2022; 195: 106762.
 [http://dx.doi.org/10.1016/j.rmed.2022.106762] [PMID:  35227546]

[12]
Hansen SR, Hetta AK, Omdal R. Primary Sjögren’s syndrome and sarcoidosis: Coexistence more than by chance? Scand J Rheumatol  2008; 37(6): 485-6.
 [http://dx.doi.org/10.1080/03009740802220075] [PMID:  18830902]

[13]
Lopes AI, Machado-Neves R, Honavar M, Pereira PR. The role of minor salivary glands’ biopsy in the diagnosis of Sjögren’s syndrome and other systemic diseases. Eur J Intern Med  2021; 94: 69-72.
 [http://dx.doi.org/10.1016/j.ejim.2021.07.012] [PMID:  34384684]

[14]
Xu J, Yang Y, Chen D, et al. Co-Existence of sarcoidosis and Sjögren’s Syndrome with hypercalcemia and renal involvement: A case report and literature review. Endocr Metab Immune Disord Drug Targets  2021; 21(4): 768-76.
 [http://dx.doi.org/10.2174/1871530320666200619133654] [PMID:  32560621]

[15]
Ahmad Y, Shahril NS, Hussein H, Said MS. Case review of sarcoidosis resembling sjogren’s syndrome. J Clin Med Res  2010; 2(6): 284-8.
 [http://dx.doi.org/10.4021/jocmr482w] [PMID:  22043264]

[16]
Mansour MJ, Al-Hashimi I, Wright JM. Coexistence of Sjögren’s syndrome and sarcoidosis: A report of five cases. J Oral Pathol Med  2007; 36(6): 337-41.
 [http://dx.doi.org/10.1111/j.1600-0714.2007.00530.x] [PMID:  17559494]

[17]
Mansour MJ, He C, Al-Farra ST, et al. Sarcoidosis and Sjögren’s syndrome: Clinical and salivary evaluation. J Oral Pathol Med  2013; 42(8): 594-9.
 [http://dx.doi.org/10.1111/jop.12057] [PMID:  23480241]

[18]
Amlani B, Elsayed G, Barvalia U, et al. Treatment of primary sjögren’s syndrome-related interstitial lung disease: A retrospective cohort study. Sarcoidosis Vasc Diffuse Lung Dis  2020; 37(2): 136-47.
 [http://dx.doi.org/10.36141/svdld.v37i2.8461] [PMID:  33093777]

Rights & Permissions Print Cite

Journal Information

For Authors

For Editors

For Reviewers

Explore Articles

For Visitors

DOI https://dx.doi.org/10.2174/1573405619666230215150154	Print ISSN 1573-4056
Publisher Name Bentham Science Publisher	Online ISSN 1875-6603

Current Medical Imaging

Multisystem Sarcoidosis Combined with Sjögren's Syndrome: A Case Report and Literature Review

Abstract Play Pause

Related Books

Abstract