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Epidemiology of sarcoidosis in northern Spain, 1999-2019: A population-based study

  • Author Footnotes
    1 Dr. Raúl Fernández-Ramón and Jorge Javier Gaitán-Valdizán shared first authorship.
    Raúl Fernández-Ramón
    Footnotes
    1 Dr. Raúl Fernández-Ramón and Jorge Javier Gaitán-Valdizán shared first authorship.
    Affiliations
    Department of Ophthalmology. Hospital Universitario Marqués de Valdecilla, Santander, Spain
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  • Author Footnotes
    1 Dr. Raúl Fernández-Ramón and Jorge Javier Gaitán-Valdizán shared first authorship.
    Jorge J. Gaitán-Valdizán
    Footnotes
    1 Dr. Raúl Fernández-Ramón and Jorge Javier Gaitán-Valdizán shared first authorship.
    Affiliations
    Department of Ophthalmology. Hospital Universitario Marqués de Valdecilla, Santander, Spain
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  • Lara Sánchez-Bilbao
    Affiliations
    Department of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain
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  • José L. Martín-Varillas
    Affiliations
    Department of Rheumatology. Hospital Sierrallana, IDIVAL, Santander, Spain
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  • David Martínez-López
    Affiliations
    Department of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain
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  • Rosalía Demetrio-Pablo
    Affiliations
    Department of Ophthalmology. Hospital Universitario Marqués de Valdecilla, Santander, Spain
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  • M. Carmen González-Vela
    Affiliations
    Department of Pathology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain.
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  • José Cifrián
    Affiliations
    Department of Pneumology. Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain.
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  • Author Footnotes
    2 Dr. Santos Castañeda, Prof. Miguel A. González-Gay, and Dr. R Blanco shared senior authorship.
    Santos Castañeda
    Footnotes
    2 Dr. Santos Castañeda, Prof. Miguel A. González-Gay, and Dr. R Blanco shared senior authorship.
    Affiliations
    Department of Rheumatology. Hospital Universitario de La Princesa, Madrid

    Cátedra EPID-Future, UAM-Roche, Universidad Autónoma de Madrid (UAM), Madrid, Spain.
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  • Javier Llorca
    Affiliations
    University of Cantabria, Santander, Spain

    CIBER Epidemiología y Salud Pública, Madrid, Spain
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  • Author Footnotes
    2 Dr. Santos Castañeda, Prof. Miguel A. González-Gay, and Dr. R Blanco shared senior authorship.
    Miguel A. González-Gay
    Correspondence
    Corresponding authors: Dr. Ricardo Blanco OR Prof. Miguel Á. González-Gay, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, Avda. Valdecilla s/n., ES-39008, Santander. SPAIN
    Footnotes
    2 Dr. Santos Castañeda, Prof. Miguel A. González-Gay, and Dr. R Blanco shared senior authorship.
    Affiliations
    Department of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain

    Department of Medicine and Psychiatry, University of Cantabria, Santander, Spain.

    Cardiovascular Pathophysiology and Genomics Research Unit, School of Physiology, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa.
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  • Author Footnotes
    2 Dr. Santos Castañeda, Prof. Miguel A. González-Gay, and Dr. R Blanco shared senior authorship.
    Ricardo Blanco
    Correspondence
    Corresponding authors: Dr. Ricardo Blanco OR Prof. Miguel Á. González-Gay, Rheumatology Division, Hospital Universitario Marqués de Valdecilla, Avda. Valdecilla s/n., ES-39008, Santander. SPAIN
    Footnotes
    2 Dr. Santos Castañeda, Prof. Miguel A. González-Gay, and Dr. R Blanco shared senior authorship.
    Affiliations
    Department of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain
    Search for articles by this author
  • Author Footnotes
    1 Dr. Raúl Fernández-Ramón and Jorge Javier Gaitán-Valdizán shared first authorship.
    2 Dr. Santos Castañeda, Prof. Miguel A. González-Gay, and Dr. R Blanco shared senior authorship.

      Highlights

      • The annual incidence of sarcoidosis in north Spain (3.58/100,000 inhabitants) is comparable to that of other Mediterranean countries.
      • An upward trend in incidence has been detected over the last two decades.
      • No gender predominance was observed in the incidence of sarcoidosis.
      • Males have an earlier disease onset.
      • Löfgren's syndrome (an acute variant of sarcoidosis) and the mildest radiological stages are more frequent in women.

      Abstract

      Background

      The incidence of sarcoidosis varies widely worldwide. The aim of this study was to estimate the incidence of sarcoidosis in a population-based cohort from northern Spain.

      Methods

      Patients diagnosed with sarcoidosis at Marqués de Valdecilla University Hospital, corresponding to the central Cantabria that encompasses Santander city and the surroundings, between January 1999 and December 2019were assessed. The diagnosis of sarcoidosis was established according to ATS/ERS/WASOG criteria as follows: compatible clinical and radiological presentation, histopathologic confirmation, and exclusion of other granulomatous diseases. Demographic and clinical data were collected. The incidence of sarcoidosis between 1999-2019 was estimated by sex, age, and year of diagnosis.

      Results

      A total of 234 patients were included, with a male/female ratio of 0.81. The mean age of the cohort at diagnosis was 48.43 ± 14.83 years and 129 (55.1%) were women. Incidence during the period of study was 3.58 per 100,000 populations (95% confidence interval: 3.13 – 4.07). No gender predominance was observed. An increase in age at diagnosis over time was found in the linear regression analysis. Thoracic affection was found in 180 patients (76.9%). Most common extra-thoracic areas affected were skin (34.2%), joints (30.8%) and eyes (15.4%).

      Conclusions

      The incidence of sarcoidosis estimated in this study was similar to that of other Mediterranean countries. No gender predominance was observed. Consistent with previous studies, male presented an incidence peak 10 years earlier than female. A second peak between ages 60-69 years was identified in both sexes.

      Keywords

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      References

      1. Statement on Sarcoidosis.
        Am J Respir Crit Care Med [Internet]. 1999; 160: 736-755https://doi.org/10.1164/ajrccm.160.2.ats4-99
        • Valeyre D
        • Prasse A
        • Nunes H
        • Uzunhan Y
        • Brillet P-Y
        • Sarcoidosis Muller-Quernheim J.
        Lancet (London, England). 2014; 383: 1155-1167https://doi.org/10.1016/S0140-6736(13)60680-7
        • Gerke AK.
        Morbidity and mortality in sarcoidosis.
        Curr Opin Pulm Med. 2014; 20: 472-478https://doi.org/10.1097/MCP.0000000000000080
        • Baughman RP
        • Field S
        • Costabel U
        • Crystal RG
        • Culver DA
        • Drent M
        • et al.
        Sarcoidosis in America: analysis based on health care use.
        Ann Am Thorac Soc. 2016; 13: 1244-1252https://doi.org/10.1513/AnnalsATS.201511-760OC
        • Rybicki BA
        • Major M
        • Popovich JJ
        • Maliarik MJ
        • Iannuzzi MC.
        Racial differences in sarcoidosis incidence: a 5-year study in a health maintenance organization.
        Am J Epidemiol. 1997; 145: 234-241https://doi.org/10.1093/oxfordjournals.aje.a009096
        • Milman N
        • Selroos O.
        Pulmonary sarcoidosis in the Nordic countries 1950-1982. epidemiology and clinical picture.
        Sarcoidosis. 1990; 7: 50-57
        • Arkema E V
        • Grunewald J
        • Kullberg S
        • Eklund A
        • Askling J.
        Sarcoidosis incidence and prevalence: a nationwide register-based assessment in Sweden.
        Eur Respir J. 2016; 48: 1690-1699https://doi.org/10.1183/13993003.00477-2016
        • Pietinalho A
        • Hiraga Y
        • Hosoda Y
        • Lofroos AB
        • Yamaguchi M
        • Selroos O.
        The frequency of sarcoidosis in Finland and Hokkaido, Japan. a comparative epidemiological study.
        Sarcoidosis. 1995; 12: 61-67
        • Gribbin J
        • Hubbard RB
        • Le Jeune I
        • Smith CJP
        • West J
        • Tata LJ
        Incidence and mortality of idiopathic pulmonary fibrosis and sarcoidosis in the UK.
        Thorax. 2006; 61: 980-985https://doi.org/10.1136/thx.2006.062836
        • Baughman RP
        • Teirstein AS
        • Judson MA
        • Rossman MD
        • Yeager HJ
        • Bresnitz EA
        • et al.
        Clinical characteristics of patients in a case control study of sarcoidosis.
        Am J Respir Crit Care Med. 2001; 164 (Pt 1): 1885-1889https://doi.org/10.1164/ajrccm.164.10.2104046
        • Erdal BS
        • Clymer BD
        • VO Yildiz
        • Julian MW
        • Crouser ED.
        Unexpectedly high prevalence of sarcoidosis in a representative U.S. metropolitan population.
        Respir Med. 2012; 106: 893-899https://doi.org/10.1016/j.rmed.2012.02.007
        • Yoon H
        • Song J
        • Kim Y.
        Sarcoidosis in South Korea: Analysis of prevalence and incidence by a nationwide population-based study.
        Am J Respir Crit Care Med [Internet]. 2018; 197 (MeetingAbstracts): 1-8
        • Gerke AK.
        Morbidity and mortality in sarcoidosis.
        Curr Opin Pulm Med. 2014; 20: 472-478https://doi.org/10.1097/MCP.0000000000000080
        • Cozier YC
        • Berman JS
        • Palmer JR
        • Boggs DA
        • Serlin DM
        • Rosenberg L.
        Sarcoidosis in black women in the United States: data from the Black Women's Health Study.
        Chest. 2011; 139: 144-150https://doi.org/10.1378/chest.10-0413
        • Gorham ED
        • Garland CF
        • Garland FC
        • Kaiser K
        • Travis WD
        • Centeno JA.
        Trends and occupational associations in incidence of hospitalized pulmonary sarcoidosis and other lung diseases in Navy personnel: a 27-year historical prospective study, 1975-2001.
        Chest. 2004; 126: 1431-1438https://doi.org/10.1378/chest.126.5.1431
        • Park JE
        • Kim YS
        • Kang MJ
        • Kim CJ
        • Han CH
        • Lee SM
        • et al.
        Prevalence, incidence, and mortality of sarcoidosis in Korea, 2003-2015: a nationwide population-based study.
        Respir Med. 2018; 144S: S28-S34https://doi.org/10.1016/j.rmed.2018.03.028
        • Morimoto T
        • Azuma A
        • Abe S
        • Usuki J
        • Kudoh S
        • Sugisaki K
        • et al.
        Epidemiology of sarcoidosis in Japan.
        Eur Respir J. 2008; 31: 372-379https://doi.org/10.1183/09031936.00075307
        • Haraldsdottir SO
        • Jorundsdottir KB
        • Yngvason F
        • Bjornsson J
        • Gislason T.
        [Sarcoidosis in Iceland 1981-2003].
        Laeknabladid. 2007; 93: 105-109
        • Byg K-E
        • Milman N
        • Hansen S.
        Sarcoidosis in Denmark 1980-1994. a registry-based incidence study comprising 5536 patients. Sarcoidosis.
        Vasc Diffus lung Dis Off J WASOG.20. 2003: 46-52
        • Kowalska M
        • Niewiadomska E
        • Zejda JE.
        Epidemiology of sarcoidosis recorded in 2006-2010 in the Silesian voivodeship on the basis of routine medical reporting.
        Ann Agric Environ Med. 2014; 21: 55-58
        • Deubelbeiss U
        • Gemperli A
        • Schindler C
        • Baty F
        • Brutsche MH.
        Prevalence of sarcoidosis in Switzerland is associated with environmental factors.
        Eur Respir J. 2010; 35: 1088-1097https://doi.org/10.1183/09031936.00197808
        • Duchemann B
        • Annesi-Maesano I
        • Jacobe de Naurois C
        • Sanyal S
        • Brillet P-Y
        • Brauner M
        • et al.
        Prevalence and incidence of interstitial lung diseases in a multi-ethnic county of Greater Paris.
        Eur Respir J. 2017; 50https://doi.org/10.1183/13993003.02419-2016
        • Karakatsani A
        • Papakosta D
        • Rapti A
        • Antoniou KM
        • Dimadi M
        • Markopoulou A
        • et al.
        Epidemiology of interstitial lung diseases in Greece.
        Respir Med [Internet]. 2009; 103: 1122-1129https://doi.org/10.1016/j.rmed.2009.03.001
        • Škopljanac I
        • Miše K
        • Šegrt I
        • Ilak D.
        Sarcoidosis in South Croatia, 30-year follow up.
        Eur Respir J [Internet]. 2017 Sep 1; 50 (suppl): PA2599https://doi.org/10.1183/1393003.congress-2017.PA2599
        • Garcia Martin C
        • Masa JF
        • Verea H
        • Fontan J
        • Mosquera JA
        • Galdo F
        • et al.
        [Sarcoidosis. epidemiological, clinical and diagnostic aspects in 40 patients].
        Rev Clin Esp. 1985; 176: 32-34
        • Perez Arellano JL
        • Ramos Guevara RM
        • Lopez Ochoa FJ
        • Alcazar Montero MC
        • de Leon Gomez B
        • Flores MT
        • et al.
        [Sarcoidosis. retrospective study of 30 cases in Salamanca and comparison with other national series].
        Rev Clin Esp. 1991; 189: 159-162
        • Villarroya Lequericaonandia ME
        • Fernández Jorge MA
        • Ibáñez Gómez J
        Sarcoidosis: análisis retrospectivo de 14 casos.
        Atención Primaria. 1992; 9: 436-438
        • Fite E
        • Alsina JM
        • Mana J
        • Pujol R
        • Ruiz J
        • Morera J.
        Epidemiology of sarcoidosis in Catalonia: 1979-1989.
        Sarcoidosis, Vasc Diffus lung Dis Off J WASOG.13. 1996: 153-158
        • Alcoba Leza M
        • Perez-Simon MR
        • Guerra Laso JM
        • Carro Fernandez JA
        • Alonso Fernandez MA
        • Blanco Iglesias B
        • et al.
        [Sarcoidosis in a sanitary area at Leon (Spain). Epidemiology and clinical features].
        An Med Interna. 2003; 20: 617-620
        • Fernández González S
        • López González R.
        Epidemiología, formas de presentación, estadio radiológico y métodos diagnósticos de la sarcoidosis en el área de León (2001-2008).
        Rev Clin Esp. 2011; 211: 291-297https://doi.org/10.1016/j.rce.2011.03.001
        • Costabel U
        • Hunninghake GW
        on behalf of the Sarcoidosis Statement Committee. ATS/ERS/WASOG statement on sarcoidosis.
        Eur Respir J. 1999; 14: 735https://doi.org/10.1034/j.1399-3003.1999.14d02.x
        • Brown F
        • Tanner LS.
        Lofgren Syndrome.
        Treasure Island, FL2020
        • Judson MA
        • Costabel U
        • Drent M
        • Wells A
        • Maier L
        • Koth L
        • et al.
        The WASOG Sarcoidosis Organ Assessment Instrument: an update of a previous clinical tool.
        Sarcoidosis, Vasc Diffus lung Dis Off J WASOG.31. 2014: 19-27
        • Ungprasert Patomong
        • Crowson Cynthia S
        • Matteson EL
        Influence of gender on epidemiology and clinical manifestations of sarcoidosis: a population-based retrospective cohort study 1976-2013.
        Lung. 2017; 195: 87-91https://doi.org/10.1007/s00408-016-9952-6
        • Baughman RP
        • Field S
        • Costabel U
        • Crystal RG
        • Culver DA
        • Drent M
        • et al.
        Sarcoidosis in America. analysis based on health care use.
        Ann Am Thorac Soc. 13. 2016: 1244-1252https://doi.org/10.1513/AnnalsATS.201511-760OC
        • Ungprasert P
        • Crowson CS
        • Matteson EL.
        Epidemiology and clinical characteristics of sarcoidosis: an update from a population-based cohort study from Olmsted County, Minnesota.
        Reumatismo. 2017; 69: 16-22https://doi.org/10.4081/reumatismo.2017.965
        • Ungprasert P
        • Carmona EM
        • Utz JP
        • Ryu JH
        • Crowson CS
        • Matteson EL.
        Epidemiology of Sarcoidosis 1946-2013: a population-based study.
        Mayo Clin Proc. 2016; 91: 183-188https://doi.org/10.1016/j.mayocp.2015.10.024
        • Kreider ME
        • Christie JD
        • Thompson B
        • Newman L
        • Rose C
        • Barnard J
        • et al.
        Relationship of environmental exposures to the clinical phenotype of sarcoidosis.
        Chest. 2005; 128: 207-215https://doi.org/10.1378/chest.128.1.207
        • Cozier YC
        • Berman JS
        • Palmer JR
        • Boggs DA
        • Wise LA
        • Rosenberg L.
        Reproductive and hormonal factors in relation to incidence of sarcoidosis in US Black women: the Black Women's Health Study.
        Am J Epidemiol. 2012; 176: 635-641https://doi.org/10.1093/aje/kws145
        • Sawahata M
        • Sugiyama Y
        • Nakamura Y
        • Nakayama M
        • Mato N
        • Yamasawa H
        • et al.
        Age-related and historical changes in the clinical characteristics of sarcoidosis in Japan.
        Respir Med. 2015; 109: 272-278https://doi.org/10.1016/j.rmed.2014.12.012
        • Yanardağ H
        • Pamuk ON
        • Karayel T.
        Cutaneous involvement in sarcoidosis: analysis of the features in 170 patients.
        Respir Med. 2003; 97: 978-982https://doi.org/10.1016/s0954-6111(03)00127-6
        • Newman LS
        • Rose CS
        • Maier LA.
        N Engl J Med. 1997; 336: 1224-1234https://doi.org/10.1056/NEJM199704243361706
        • Judson MA
        • Boan AD
        • Lackland DT.
        The clinical course of sarcoidosis: presentation, diagnosis, and treatment in a large white and black cohort in the United States. Sarcoidosis.
        Vasc Diffus lung Dis Off J WASOG. 29. 2012: 119-127
        • Levitt DG
        • Levitt MD.
        Protein losing enteropathy: comprehensive review of the mechanistic association with clinical and subclinical disease states.
        Clin Exp Gastroenterol. 2017; 10: 147-168https://doi.org/10.2147/CEG.S136803
        • Pietinalho A
        • Ohmichi M
        • Lofroos AB
        • Hiraga Y
        • Selroos O.
        The prognosis of pulmonary sarcoidosis in Finland and Hokkaido, Japan. a comparative five-year study of biopsy-proven cases. Sarcoidosis.
        Vasc Diffus lung Dis Off J WASOG. 17. 2000: 158-166
        • Yigla M
        • Badarna-Abu-Ria N
        • Tov N
        • Ravell-Weiller D
        • Rubin A-HE
        Sarcoidosis in northern Israel; clinical characteristics of 120 patients. Sarcoidosis.
        Vasc Diffus lung Dis Off J WASOG. 19. 2002: 220-226
        • Behbehani N
        • JayKrishnan B
        • Khadadah M
        • Hawa H
        • Farah Y.
        Clinical presentation of sarcoidosis in a mixed population in the middle east.
        Respir Med. 2007; 101: 2284-2288https://doi.org/10.1016/j.rmed.2007.06.025
        • Kumar R
        • Goel N
        • Gaur SN.
        Sarcoidosis in north Indian population: a retrospective study.
        Indian J Chest Dis Allied Sci. 2012; 54: 99-104
        • Markevitz N
        • Epstein Shochet G
        • Levi Y
        • Israeli-Shani L
        • Shitrit D
        Sarcoidosis in Israel: Clinical outcome status, organ involvement, and long-term follow-up.
        Lung. 2017; 195: 419-424https://doi.org/10.1007/s00408-017-0015-4
        • Cardoso A V.
        • Mota PC
        • Melo N
        • Guimarães S
        • Souto Moura C
        • Jesus JM
        • et al.
        Analysis of sarcoidosis in the Oporto region (Portugal).
        Rev Port Pneumol (English Ed [Internet]. 2017; 23: 251-258https://doi.org/10.1016/j.rppnen.2017.05.004
        • Mañá J
        • Rubio-Rivas M
        • Villalba N
        • Marcoval J
        • Iriarte A
        • Molina-Molina M
        • et al.
        Multidisciplinary approach and long-term follow-up in a series of 640 consecutive patients with sarcoidosis.
        Med (United States).96. 2017 Jul 1https://doi.org/10.1097/MD.0000000000007595
        • Schupp JC
        • Freitag-Wolf S
        • Bargagli E
        • Mihailović-Vučinić V
        • Rottoli P
        • Grubanovic A
        • et al.
        Phenotypes of organ involvement in sarcoidosis.
        Eur Respir J. 2018; 51: 1-11https://doi.org/10.1183/13993003.00991-2017
        • Pérez-Alvarez R
        • Brito-Zerón P
        • Kostov B
        • Feijoo-Massó C
        • Fraile G
        • Gómez-de-la-Torre R
        • et al.
        Systemic phenotype of sarcoidosis associated with radiological stages. Analysis of 1230 patients.
        Eur J Intern Med. 2019; 69 (https://doi.org/10.1016/j.ejim.2019.08.025): 77-85
        • Thomeer M
        • Demedts M
        • Vandeurzen K.
        Registration of interstitial lung diseases by 20 centres of respiratory medicine in flanders.
        Acta Clin Belg. 2001; 56: 163-172https://doi.org/10.1179/acb.2001.026
        • Gillman A
        • Steinfort C.
        Sarcoidosis in Australia.
        Intern Med J. 2007; 37 (https://doi.org/10.1111/j.1445-5994.2007.01365.x.): 356-359
        • Musellim B
        • Kumbasar OO
        • Ongen G
        • Cetinkaya E
        • Turker H
        • Uzaslan E
        • et al.
        Epidemiological features of Turkish patients with sarcoidosis.
        Respir Med. 2009; 103: 907-912https://doi.org/10.1016/j.rmed.2008.12.011
        • Fidler LM
        • Balter M
        • Fisher JH
        • To T
        • Stanbrook MB
        • Gershon A
        Epidemiology and health outcomes of sarcoidosis in a universal healthcare population: a cohort study.
        Eur Respir J. 2019; 54 (https://doi.org/10.1183/13993003.00444-2019)