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Endocrine, Metabolic & Immune Disorders - Drug Targets

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ISSN (Print): 1871-5303
ISSN (Online): 2212-3873

Case Report

Co-Existence of Sarcoidosis and Sjögren’s Syndrome with Hypercalcemia and Renal Involvement: A Case Report and Literature Review

Author(s): Jing Xu, Yumei Yang, Dandan Chen, Zhiqiang Lu, Junbo Ge, Xiaomu Li* and Xin Gao

Volume 21, Issue 4, 2021

Published on: 19 June, 2020

Page: [768 - 776] Pages: 9

DOI: 10.2174/1871530320666200619133654

Price: $65

Abstract

Background: Sarcoidosis and Sjögren's syndrome (SS) are chronic multi-system inflammatory diseases of unknown origin that most commonly attack the salivary glands. Both of the diseases have vague and non-specific symptoms, causing difficulties for the clinicians to distinguish between the two diseases. Main diagnostic criteria of SS exclude sarcoidosis. However, a co-existence of both diseases should be noted. Here, a case of co-existing sarcoidosis and Sjögren's syndrome is reported, complicated with severe hypercalcemia and renal failure, in addition to a literature review.

Case Presentation: A 71-year-old man visited our hospital complaining of daily progressive oral dryness, thirst, and blurred vision with a feeling of dry eyes for a one-year duration. His physical examination showed enlargement of both sides of cervical and supraclavicular lymph nodes. Lung auscultation showed decreased breath sounds with bibasilar inspiratory crackles. However, initial laboratory results revealed severe hypercalcemia with moderate hypercalciuria and renal failure. The final diagnosis was co-existing SS and sarcoidosis according to clinical, radiological, and laboratory data. The patient received oral prednisone therapy for 18 months. After a follow-up of years, the serum calcium concentration, renal function, and chest CT scan remained normal after prednisone treatment stopped for more than 18 months.

Conclusion: In the literature, calcium metabolic disorder and renal involvement have not been reported among patients with Sarcoidosis and Sjögren's syndrome, suggesting that calcium metabolic disorder may be underestimated. Serum and urine calcium concentration should be measured in addition to routine laboratory tests.

Keywords: Sjögren’s syndrome, sarcoidosis, hypercalcemia, renal failure, calcium concentration, serum, urine.

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Graphical Abstract

[1]
Baughman, R.P.; Lower, E.E.; du Bois, R.M. Sarcoidosis. Lancet, 2003, 361(9363), 1111-1118.
[http://dx.doi.org/10.1016/S0140-6736(03)12888-7] [PMID: 12672326]
[2]
Stefanski, A.L.; Tomiak, C.; Pleyer, U.; Dietrich, T.; Burmester, G.R.; Dörner, T. The diagnosis and treatment of Sjögren’s syndrome. Dtsch. Arztebl. Int., 2017, 114(20), 354-361.
[http://dx.doi.org/10.3238/arztebl.2017.0354] [PMID: 28610655]
[3]
Wirnsberger, R.M.; de Vries, J.; Wouters, E.F.; Drent, M. Clinical presentation of sarcoidosis in the Netherlands an epidemiological study. Neth. J. Med., 1998, 53(2), 53-60.
[http://dx.doi.org/10.1016/S0300-2977(98)00058-8] [PMID: 9766153]
[4]
Agrawal, V.; Crisi, G.M.; D’Agati, V.D.; Freda, B.J. Renal sarcoidosis presenting as acute kidney injury with granulomatous interstitial nephritis and vasculitis. Am. J. Kidney Dis., 2012, 59(2), 303-308.
[http://dx.doi.org/10.1053/j.ajkd.2011.09.025] [PMID: 22177616]
[5]
Hishida, E.; Masuda, T.; Akimoto, T.; Sato, R.; Wakabayashi, N.; Miki, A.; Otani, N.; Imai, T.; Sugase, T.; Takeda, S.; Muto, S.; Nagata, D. Renal failure found during the follow-up of sarcoidosis: the relevance of a delay in the diagnosis of concurrent hypercalcemia. Intern. Med., 2016, 55(14), 1893-1898.
[http://dx.doi.org/10.2169/internalmedicine.55.6194] [PMID: 27432099]
[6]
Korsten, P.; Tampe, B.; Konig, M.F.; Nikiphorou, E. Sarcoidosis and autoimmune diseases: differences, similarities and overlaps. Curr. Opin. Pulm. Med., 2018, 24(5), 504-512.
[http://dx.doi.org/10.1097/MCP.0000000000000500] [PMID: 29985181]
[7]
Mahévas, M.; Lescure, F.X.; Boffa, J.J.; Delastour, V.; Belenfant, X.; Chapelon, C.; Cordonnier, C.; Makdassi, R.; Piette, J.C.; Naccache, J.M.; Cadranel, J.; Duhaut, P.; Choukroun, G.; Ducroix, J.P.; Valeyre, D. Renal sarcoidosis: clinical, laboratory, and histologic presentation and outcome in 47 patients. Medicine (Baltimore), 2009, 88(2), 98-106.
[http://dx.doi.org/10.1097/MD.0b013e31819de50f] [PMID: 19282700]
[8]
Lois, M.; Roman, J.; Holland, W.; Agudelo, C. Coexisting Sjögren’s syndrome and sarcoidosis in the lung. Semin. Arthritis Rheum., 1998, 28(1), 31-40.
[http://dx.doi.org/10.1016/S0049-0172(98)80026-7] [PMID: 9726334]
[9]
Mansour, M.J.; Al-Hashimi, I.; Wright, J.M. Coexistence of Sjögren’s syndrome and sarcoidosis: a report of five cases. J. Oral Pathol. Med., 2007, 36(6), 337-341.
[http://dx.doi.org/10.1111/j.1600-0714.2007.00530.x] [PMID: 17559494]
[10]
van de Loosdrecht, A.; Kalk, W.; Bootsma, H.; Henselmans, J.M.; Kraan, J.; Kallenberg, C.G. Simultaneous presentation of sarcoidosis and Sjögren’s syndrome. Rheumatology (Oxford), 2001, 40(1), 113-115.
[http://dx.doi.org/10.1093/rheumatology/40.1.113] [PMID: 11157156]
[11]
Cummings, M.M.; Dunner, E.; Williams, J.H., Jr Epidemiologic and clinical observations in sarcoidosis. Ann. Intern. Med., 1959, 50(4), 879-890.
[http://dx.doi.org/10.7326/0003-4819-50-4-879] [PMID: 13637563]
[12]
Gillman, A.; Steinfort, C. Sarcoidosis in Australia. Intern. Med. J., 2007, 37(6), 356-359.
[http://dx.doi.org/10.1111/j.1445-5994.2007.01365.x] [PMID: 17535377]
[13]
Goldstein, R.A.; Israel, H.L.; Becker, K.L.; Moore, C.F. The infrequency of hypercalcemia in sarcoidosis. Am. J. Med., 1971, 51(1), 21-30.
[http://dx.doi.org/10.1016/0002-9343(71)90320-2] [PMID: 5570317]
[14]
Kim, D.S. Sarcoidosis in Korea: report of the second nationwide survey. Sarcoidosis Vasc. Diffuse Lung Dis., 2001, 18(2), 176-180.
[PMID: 11436538]
[15]
Sharma, O.P.; Vitamin, D. Vitamin D, calcium, and sarcoidosis. Chest, 1996, 109(2), 535-539.
[http://dx.doi.org/10.1378/chest.109.2.535] [PMID: 8620732]
[16]
Rizzato, G.; Fraioli, P.; Montemurro, L. Nephrolithiasis as a presenting feature of chronic sarcoidosis. Thorax, 1995, 50(5), 555-559.
[http://dx.doi.org/10.1136/thx.50.5.555] [PMID: 7597671]
[17]
Vitali, C.; Bombardieri, S.; Moutsopoulos, H.M.; Balestrieri, G.; Bencivelli, W.; Bernstein, R.M.; Bjerrum, K.B.; Braga, S.; Coll, J.; de Vita, S. Preliminary criteria for the classification of Sjögren’s syndrome. Results of a prospective concerted action supported by the European Community. Arthritis Rheum., 1993, 36(3), 340-347.
[http://dx.doi.org/10.1002/art.1780360309] [PMID: 8452579]
[18]
Akiyama, Y.; Suzuki, T.; Tanaka, M.; Katagiri, T.; Ishibashi, T.; Imai, F.; Ohno, S.; Doi, Y. A case of sarcoidosis associated with Sjögren’s syndrome. Arerugi, 1992, 41(10), 1500-1506.
[PMID: 1482299]
[19]
Araki, T.; Katsura, H.; Motegi, T.; Yamada, K.; Uehara, T.; Gotoh, R.; Kida, K.; Esaki, Y. Two elderly patients with sarcoidosis and Sjögren’s syndrome. Nippon Ronen Igakkai Zasshi, 2001, 38(2), 229-234.
[http://dx.doi.org/10.3143/geriatrics.38.229] [PMID: 11305039]
[20]
Belgodere, X.; Viraben, R.; Gorguet, B.; Allaouchiche, B.; Lieutaud, O.; Maestracci, D. Guess what! Cutaneous sarcoidosis, Sjögren’s syndrome and autoimmune thyroiditis associated with hepatitis C virus infection. Eur. J. Dermatol., 1999, 9(3), 235-236.
[PMID: 10408919]
[21]
Deheinzelin, D.; de Carvalho, C.R.; Tomazini, M.E.; Barbas Filho, J.V.; Saldiva, P.H. Association of Sjögren’s syndrome and sarcoidosis. Report of a case. Sarcoidosis, 1988, 5(1), 68-70.
[PMID: 3381023]
[22]
Justiniani, F.R. Sarcoidosis complicating primary Sjögren’s syndrome. Mt. Sinai J. Med., 1989, 56(1), 59-61.
[PMID: 2784185]
[23]
Koarada, S.; Uchida, M.; Tada, Y.; Ushiyama, O.; Suzuki, N.; Ohta, A.; Nagasawa, K. A case of Sjögren’s syndrome complicated by polymyositis and sarcoidosis with HLA-B7 and DR 8: common causes of susceptibility for these diseases. Jpn. J. Clin. Immunol., 2000, 23(2), 141-147.
[http://dx.doi.org/10.2177/jsci.23.141] [PMID: 10863330]
[24]
Kohsokabe, S.; Fujita, J.; Imai, T.; Tanaka, M.; Kawahara, Y.; Koide, Y. A case of Sjögren syndrome associated with sarcoidosis. Ryumachi, 1992, 32(3), 230-236.
[PMID: 1523525]
[25]
Kondo, H.; Sakai, S.; Sakai, Y. Autoimmune haemolytic anaemia, Sjögren’s syndrome and idiopathic thrombocytopenic purpura in a patient with sarcoidosis. Acta Haematol., 1993, 89(4), 209-212.
[http://dx.doi.org/10.1159/000204526] [PMID: 8213004]
[26]
Koopmans, P.P.; Bodeutsch, C.; de Wilde, P.C.; Boerbooms, A.M. Primary Sjögren’s syndrome presenting as a case of sarcoidosis and a suspected pancreatic tumour. Ann. Rheum. Dis., 1990, 49(6), 407-409.
[http://dx.doi.org/10.1136/ard.49.6.407] [PMID: 2383067]
[27]
Melsom, R.D.; Speight, P.M.; Ryan, J.; Perry, J.D. Sarcoidosis in a patient presenting with clinical and histological features of primary Sjögren’s syndrome. Ann. Rheum. Dis., 1988, 47(2), 166-168.
[http://dx.doi.org/10.1136/ard.47.2.166] [PMID: 3355252]
[28]
Newball, H.H.; Brahim, S.A. Chronic obstructive airway disease in patients with Sjögren’s syndrome. Am. Rev. Respir. Dis., 1977, 115(2), 295-304.
[PMID: 842943]
[29]
Radenne, F.; Tillie-Leblond, I.; Maurage, C.A.; Rémy-Jardin, M.; Wallaert, B.; Tonnel, A.B. Sjögren’s syndrome and necrotizing sarcoid-like granulomatosis. Rev. Mal. Respir., 1999, 16(4), 554-557.
[PMID: 10549066]
[30]
Ramos-Casals, M.; Brito-Zerón, P.; García-Carrasco, M.; Font, J. Sarcoidosis or Sjögren syndrome? Clues to defining mimicry or coexistence in 59 cases. Medicine (Baltimore), 2004, 83(2), 85-95.
[http://dx.doi.org/10.1097/01.md.0000121237.98962.1e] [PMID: 15028962]
[31]
Theander, E.; Hansen, O.; Jacobsson, L.; Manthorpe, R. AV-block III in a patient with sarcoidosis mimicking Sjögren’s syndrome. Scand. J. Rheumatol., 1999, 28(6), 377-379.
[http://dx.doi.org/10.1080/03009749950155382] [PMID: 10665745]
[32]
Tokuyasu, H.; Harada, T.; Touge, H.; Kawasaki, Y.; Maeda, R.; Isowa, N.; Ohnuma, H.; Miura, H.; Shimizu, E. Primary Sjögren’s syndrome complicated by sarcoidosis. Intern. Med., 2008, 47(23), 2049-2052.
[http://dx.doi.org/10.2169/internalmedicine.47.1526] [PMID: 19043259]
[33]
Turiaf, J.; Battesti, J.P.; Rain, J.D.; Basset, F.; Marc, A. Gougerot-Sjögren syndrome in patients with sarcoidosis Nouv. Presse Med., 1976, 5(10), 625-627.
[PMID: 1264589]
[34]
Gilboe, I.M.; Kvien, T.K.; Uhlig, T.; Husby, G. Sicca symptoms and secondary Sjögren’s syndrome in systemic lupus erythematosus: comparison with rheumatoid arthritis and correlation with disease variables. Ann. Rheum. Dis., 2001, 60(12), 1103-1109.
[http://dx.doi.org/10.1136/ard.60.12.1103] [PMID: 11709451]
[35]
Manoussakis, M.N.; Georgopoulou, C.; Zintzaras, E.; Spyropoulou, M.; Stavropoulou, A.; Skopouli, F.N.; Moutsopoulos, H.M. Sjögren’s syndrome associated with systemic lupus erythematosus: clinical and laboratory profiles and comparison with primary Sjögren’s syndrome. Arthritis Rheum., 2004, 50(3), 882-891.
[http://dx.doi.org/10.1002/art.20093] [PMID: 15022331]
[36]
Nossent, J.C.; Swaak, A.J. Systemic lupus erythematosus VII: frequency and impact of secondary Sjøgren’s syndrome. Lupus, 1998, 7(4), 231-234.
[http://dx.doi.org/10.1191/096120398678920046] [PMID: 9643312]
[37]
Mattey, D.L.; González-Gay, M.A.; Hajeer, A.H.; Dababneh, A.; Thomson, W.; García-Porrúa, C.; Ollier, W.E. Association between HLA-DRB1*15 and secondary Sjögren’s syndrome in patients with rheumatoid arthritis. J. Rheumatol., 2000, 27(11), 2611-2616.
[PMID: 11093441]
[38]
Uhlig, T.; Kvien, T.K.; Jensen, J.L.; Axéll, T. Sicca symptoms, saliva and tear production, and disease variables in 636 patients with rheumatoid arthritis. Ann. Rheum. Dis., 1999, 58(7), 415-422.
[http://dx.doi.org/10.1136/ard.58.7.415] [PMID: 10381485]
[39]
Alarcón-Segovia, D.; Ibánez, G.; Hernández-Ortíz, J.; Velázquez-Forero, F.; González-Jiménez, Y. Sjögren’s syndrome in progressive systemic sclerosis (scleroderma). Am. J. Med., 1974, 57(1), 78-85.
[http://dx.doi.org/10.1016/0002-9343(74)90771-2] [PMID: 4134767]
[40]
Brandt, J.; Maier, T.; Rudwaleit, M.; Kühl, U.; Hiepe, F.; Sieper, J.; Braun, J. Co-occurrence of spondyloarthropathy and connective tissue disease: development of Sjögren’s syndrome and mixed connective tissue disease (MCTD) in a patient with ankylosing spondylitis. Clin. Exp. Rheumatol., 2002, 20(1), 80-84.
[PMID: 11892716]
[41]
Ramos-Casals, M.; Nardi, N.; Brito-Zerón, P.; Aguiló, S.; Gil, V.; Delgado, G.; Bové, A.; Font, J. Atypical autoantibodies in patients with primary Sjögren syndrome: clinical characteristics and follow-up of 82 cases. Semin. Arthritis Rheum., 2006, 35(5), 312-321.
[http://dx.doi.org/10.1016/j.semarthrit.2005.12.004] [PMID: 16616154]
[42]
Miyata, M.; Takase, Y.; Kobayashi, H.; Kokubun, M.; Yoshimura, A.; Katsuura, Y.; Nishimaki, T.; Kasukawa, R. Primary Sjögren’s syndrome complicated by sarcoidosis. Intern. Med., 1998, 37(2), 174-178.
[http://dx.doi.org/10.2169/internalmedicine.37.174] [PMID: 9550600]
[43]
Vitali, C.; Bombardieri, S.; Jonsson, R.; Moutsopoulos, H.M.; Alexander, E.L.; Carsons, S.E.; Daniels, T.E.; Fox, P.C.; Fox, R.I.; Kassan, S.S.; Pillemer, S.R.; Talal, N.; Weisman, M.H. European study group on classification criteria for Sjögren’s syndrome. Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by the American-European consensus group. Ann. Rheum. Dis., 2002, 61(6), 554-558.
[http://dx.doi.org/10.1136/ard.61.6.554] [PMID: 12006334]
[44]
Khan, A.A.; Hanley, D.A.; Rizzoli, R.; Bollerslev, J.; Young, J.E.; Rejnmark, L.; Thakker, R.; D’Amour, P.; Paul, T.; Van Uum, S.; Shrayyef, M.Z.; Goltzman, D.; Kaiser, S.; Cusano, N.E.; Bouillon, R.; Mosekilde, L.; Kung, A.W.; Rao, S.D.; Bhadada, S.K.; Clarke, B.L.; Liu, J.; Duh, Q.; Lewiecki, E.M.; Bandeira, F.; Eastell, R.; Marcocci, C.; Silverberg, S.J.; Udelsman, R.; Davison, K.S.; Potts, J.T., Jr; Brandi, M.L.; Bilezikian, J.P. Primary hyperparathyroidism: review and recommendations on evaluation, diagnosis, and management. A Canadian and international consensus. Osteoporos. Int., 2017, 28(1), 1-19.
[http://dx.doi.org/10.1007/s00198-016-3716-2] [PMID: 27613721]
[45]
Lebacq, E.; Desmet, V.; Verhaegen, H. Renal involvement in sarcoidosis. Postgrad. Med. J., 1970, 46(538), 526-529.
[http://dx.doi.org/10.1136/pgmj.46.538.526] [PMID: 5481106]
[46]
Bell, N.H.; Stern, P.H.; Pantzer, E.; Sinha, T.K.; DeLuca, H.F. Evidence that increased circulating 1 alpha, 25-dihydroxyvitamin D is the probable cause for abnormal calcium metabolism in sarcoidosis. J. Clin. Invest., 1979, 64(1), 218-225.
[http://dx.doi.org/10.1172/JCI109442] [PMID: 312811]
[47]
Papapoulos, S.E.; Clemens, T.L.; Fraher, L.J.; Lewin, I.G.; Sandler, L.M.; O’Riordan, J.L. 1, 25-dihydroxycholecalciferol in the pathogenesis of the hypercalcaemia of sarcoidosis. Lancet, 1979, 1(8117), 627-630.
[http://dx.doi.org/10.1016/S0140-6736(79)91076-6] [PMID: 85869]
[48]
Zeimer, H.J.; Greenaway, T.M.; Slavin, J.; Hards, D.K.; Zhou, H.; Doery, J.C.; Hunter, A.N.; Duffield, A.; Martin, T.J.; Grill, V. Parathyroid-hormone-related protein in sarcoidosis. Am. J. Pathol., 1998, 152(1), 17-21.
[PMID: 9422518]
[49]
Holick, M.F. Vitamin D deficiency. N. Engl. J. Med., 2007, 357(3), 266-281.
[http://dx.doi.org/10.1056/NEJMra070553] [PMID: 17634462]
[50]
Göbel, U.; Kettritz, R.; Schneider, W.; Luft, F. The protean face of renal sarcoidosis. J. Am. Soc. Nephrol., 2001, 12(3), 616-623.
[PMID: 11181812]

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