Recommendations for evaluation and diagnosis of extra-glandular manifestations of primary Sjögren syndrome: results of an epidemiologic systematic review/ meta-analysis and a consensus guideline from the Brazilian society of rheumatology (hepatic, gastrointestinal and pancreatic)

Trevisani, Virginia Fernandes Moça; Pinheiro, Aysa César; Fialho, Sonia Cristina de Magalhães Souza; Fernandes, Marilena Leal Mesquita Silvestre; Pugliesi, Alisson; Pasoto, Sandra Gofinet; Lopes, Maria Lucia Lemos; Guedes, Lissiane Karine Noronha; Miyamoto, Samira Tatiyama; Santos, Laura Caldas dos; Appenzeller, Simone; Fidelix, Tania; Ribeiro, Sandra Lúcia Euzébio; Brito, Danielle Christinne Soares Egypto de; Libório-Kimura, Tatiana Nayara; Santos, Maria Carmen Lopes Ferreira Silva; Cantali, Diego Ustárroz; Gennari, Juliana D.’Agostino; Capobianco, Karina Gatz; Civile, Vinicius Tassoni; Pinto, Ana Carolina Pereira Nunes; Rocha, Aline Pereira da; Rocha Filho, César Ramos; Oliveira, Fabiola Reis; Valim, Valeria

doi:10.1186/s42358-022-00267-y

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Position Article and Guidelines • Adv. rheumatol. 62 • 2022 • https://doi.org/10.1186/s42358-022-00267-y copy

Recommendations for evaluation and diagnosis of extra-glandular manifestations of primary Sjögren syndrome: results of an epidemiologic systematic review/ meta-analysis and a consensus guideline from the Brazilian society of rheumatology (hepatic, gastrointestinal and pancreatic)

Authorship SCIMAGO INSTITUTIONS RANKINGS

Abstract

Sjogren's syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands and other organs, associated with sicca syndrome but also with systemic involvement with varying degrees of severity. Despite their importance, some systemic manifestations, mainly liver, gastrointestinal, and pancreatic are not routinely evaluated. To address these manifestations, the Sjögren's Syndrome Committee of the Brazilian Society of Rheumatology conducted a broad systematic review of the literature on studies investigating prevalence and diagnosis of these symptoms in Sjogren´s patients and made recommendations based on the findings. Agreement between the experts was achieved using the Delphi method. This is the second part of this guideline, providing 6 recommendations for liver, gastrointestinal, and pancreatic care of SS patients.

Keywords:
Sjögren's syndrome; Gastrointestinal diseases; Clinical guidelines

Parameter	Cut-off point	Score
Positive ANA or positive smooth muscle	≥1:40	+1
Positive ANA or positive smooth muscle	≥ 1:80	+2
or anti-LKM positive	≥ 1:40	+2
or anti-SLA/LP positive	Any value	+2
IgG or gamma-globulin	Above the normal limit	+1
	> 1.1 instead of the upper limit	+2
Liver biopsy (evidence of hepatitis is indispensable)	Compatible	+1
	Typical	+2
	Atypical	0
Viral hepatitis	Yes	0
	No	+2

References	Country	pSS (n)	PBC n (%)	AIH n (%)
Lindgren et al. [⁴⁴44 Lindgren S, Manthorpe R, Eriksson S. Autoimmune liver disease in patients with primary Sjogren's syndrome. J Hepatol. 1994;20:354–8.]	Sweden	45	4 (9%)	2 (4%)
Ramos-Casals et al. [⁷7 Ramos-Casals M, Sánchez-Tapias JM, Parés A, Forns X, Brito-Zerón P, Nardi N, et al. Characterization and differentiation of autoimmune versus viral liver involvement in patients with Sjögren's syndrome. J Rheumatol. 2006;33(8):1593–9.]	Spain	475	16 (4%)	8 (2%)
Montaño-Loza et al. [¹¹11 Montaño-Loza AJ, Crispín-Acuña JC, Remes-Troche JM, Uribe M. Abnormal hepatic biochemistries and clinical liver disease in patients with primary Sjögren's syndrome. Ann Hepatol. 2007;6(3):150–5.]	Mexico	95	5 (5%)	2 (2%)
Hatzis et al. [²⁶26 Hatzis GS, Fragoulis GE, Karatzaferis A, Delladetsima I, Barbatis C, Moutsopoulos HM. Prevalence and longterm course of primary biliary cirrhosis in primary Sjögren's syndrome. J Rheumatol. 2008;35(10):2012–6.]	Greece	410	27 (6.6%)	–
Karp et al. [³⁹39 Karp JK, Akpek EK, Anders RA. Autoimmune hepatitis in patients with primary Sjogren's syndrome: a series of two-hundred and two patients. Int J Clin ExpPathol. 2010;3(6):582–6.]	USA	58	–	1 (1.7%)

References	Country	PBC n	pSS n (%)
Marasini et al. [²²22 Marasini B, Gagetta M, Rossi V, Ferrari P. Rheumatic disorders and primary biliary cirrhosis: na appraisal of 170 Italian patients. Ann Rheum Dis. 2001;60(11):1046–9.]	Italy	170	6 (3.5%)
Valera et al. [⁴⁶46 Valera JM, Smok SG, Poniachik TJ, Oksenberg RD, Silva PG, Ferrario BM, et al. Primary biliarycirrhosis: a thirteen years experience. Ver Med Chil. 2006;134(4):469–74. https://doi.org/10.4067/s0034-98872006000400010.(inSpanish). https://doi.org/10.4067/s0034-9887200600... ]	Chile	115	38%
Wang et al. [³²32 Wang Q, Yang F, Miao Q, Krawitt EL, Gershwin ME, Ma X. The clinical phenotypes of autoimmune hepatitis: a comprehensive review. J Autoimmun. 2016;66:98–107. https://doi.org/10.1016/j.jaut.2015.10.006. https://doi.org/10.1016/j.jaut.2015.10.0... ]	China	322	121 (36.2%)

References	Study type	n	Dysphagia (%)	GI motility disorder
Kjellen et al. [⁴⁸48 Kjellen G, Fransson SG, Lindstrom F, Sokjer H, Tibbling L. Esophageal function, radiography, and dysphagia in Sjogren's syndrome. Dig Dis Sci. 1986;31:225–9.]	Case–control	22	73	No
Grande et al. [⁵²52 Grande L, Lacima G, Ros E, Font J, Pera C. Esophageal motor function in primary Sjogren's syndrome. Am J Gastroenterol. 1993;88:378–81.]	Cross-sectional	20	75	No
Palma et al. [⁵¹51 Palma R, Freire A, Freitas J, Morbey A, Costa T, Saraiva F. Esophageal motility disorders in patients with Sjogren's syndrome. Dig Dis Sci. 1994;39:758–61.]	Case–control	21	70.6	No
Anselmino et al. [⁵⁴54 Anselmino M, Zaninotto G, Costantini M, Ostuni P, Ianniello A, Boccú C, et al. Esophageal motor function in primary Sjögren's syndrome: correlation with dysphagia and xerostomia. Dig Dis Sci. 1997;42(1):113–8.]	Cross-sectional	27	74	Yes
Rosztoczy et al. [⁵⁵55 Rosztoczy A, Kovacs L, Wittmann T, Lonovics J, Pokorny G. Manometric assessment of impaired esophageal motor function in primary Sjögren's syndrome. Clin Exp Rheumatol. 2001;19:147–52.]	Case–control	25	92	Yes
Volter et al. [⁵⁰50 Volter F, Fain O, Mathieu E, Thomas M. Esophageal function and Sjogren's syndrome. Dig Dis Sci. 2004;49:248–53.]	Case–control	21	33	No
Turk et al. [⁵³53 Turk T, Pirildar T, Tunc E, Bor S, Doganavsargil E. Manometric assessment of esophageal motility in patients with primary Sjogren's syndrome. Rheumatol Int. 2005;25:246–9.]	Case–control	40	65	Yes
Mandl et al. [⁴⁹49 Mandl T, Ekberg O, Manthorpe R, Jacobsson LT. Dysphagia and dysmotility of the pharynx and oesophagus in patients with primary Sjogren's syndrome. Scand J Rheumatol. 2007;36(5):394–401.]	Case–control	22	65	No

References	Country	Study type	pSS, n	Acute pancreatitis (%)
Ramos Casals et al. [⁷7 Ramos-Casals M, Sánchez-Tapias JM, Parés A, Forns X, Brito-Zerón P, Nardi N, et al. Characterization and differentiation of autoimmune versus viral liver involvement in patients with Sjögren's syndrome. J Rheumatol. 2006;33(8):1593–9.]	Spain	Retrospective Cohort	1010	5 (0.5%)
Chang et al. [⁸⁶86 Chang C-C, Chang Y-S, Wang S-H, et al. Primary Sjogren's syndrome and the risk of acute pancreatitis: a nationwide cohort study. BMJ Open. 2017;7: e014807. https://doi.org/10.1136/bmjopen-2016-014807. https://doi.org/10.1136/bmjopen-2016-014... ]	Taiwan	Retrospective Cohort	9468	44 (0.46%)

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