How Does Sarcoidosis Present in Spain? Characteristics at Diagnosis of 979 Patients from the Sarcogeas-SEMI Registry

Pilar Brito-Zerón^1,2, Belchin Kostov³, Marta Pérez de Lis⁴, Guadalupe Fraile⁵, Ricardo Gómez De La Torre⁶, María Roca Herrera⁷, Begoña De Escalante Yangüela⁸, Ana Alguacil⁹, Mercedes Pilar Perez Conesa¹⁰, Francisco Javier Rascón¹¹, Jose Salvador Garcia Morillo¹², Carlos Feijoo Massó¹³, Eva Fonseca Aizpuru¹⁴, Mariona Bonet¹⁵, Naya Faro Minguez¹⁶, Gloria De La Red Bellvis¹⁷, Eva Calvo Begueria¹⁸, Albert Gómez Lozano¹⁹, Enrique Peral Gutiérrez De Ceballos²⁰, Jorge Francisco Gómez Cerezo²¹, Gracia Cruz Caparrós²², Patricia Perez Guerrero²³, Sergio Rodríguez Fernández²⁴, Alberto Gato Diez²⁵, Neera Toledo Samaniego²⁶, Miriam Akasbi²⁷, Angel Robles²⁸, Inmaculada Ojeda²⁹, Maria José Vives³⁰, María Penadés Vidal³¹, César Morcillo³², Moisés De Vicente³³, Soledad Retamozo^2,34, Lucio Pallarés¹¹, Manuel Ramos-Casals³⁵, Roberto Pérez-Alvarez⁴ and SARCOGEAS Registry, GEAS-SEMI, ¹Autoimmune Diseases Unit, Department of Medicine, Hospital CIMA- Sanitas, Barcelona., Bacelona, Spain, ²Laboratory of Systemic Autoimmune Diseases “Josep Font”, CELLEX, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Department of Systemic Autoimmune Diseases, ICMID, Hospital Clinic, Barcelona, Barcelona, Spain, ³Primary Care Research Group, IDIBAPS, Centre d’Assistència Primària ABS Les Corts, CAPSE, Barcelona, Barcelona, Spain, ⁴Department of Internal Medicine, Hospital Alvaro Cunqueiro, Vigo, Vigo, Spain, ⁵Department of Internal Medicine, Hospital Ramón y Cajal, Madrid, Spain, Madrid, Spain, ⁶Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Oviedo, Spain, ⁷Department of Internal Medicine, Hospital Universitari Joan XXIII, Tarragona, Tarragona, Spain, ⁸Department of Internal Medicine, Hospital Clínico, Zaragoza, Zaragoza, Spain, ⁹Department of Internal Medicine, Hospital Virgen de la Salud, Toledo, Toledo, Spain, ¹⁰Department of Internal Medicine, Hospital Universitario Miguel Servet, Zaragoza, Zaragoza, Spain, ¹¹Department of Internal Medicine, Hospital Son Espases. Palma de Mallorca, Palma de Mallorca, Spain, ¹²Department of Internal Medicine, Hospital Virgen del Rocio, Sevilla, Sevilla, Spain, ¹³Department of Internal Medicine, Hospital Parc Taulí, Sabadell, Sadabell, Spain, ¹⁴Department of Internal Medicine, Hospital de Cabueñes, Gijón, Gijón, Spain, ¹⁵Department of Internal Medicine, Althaia, Xarxa Assistencial de Manresa, Manresa, Spain, ¹⁶Department of Internal Medicine, Hospital Clínico San Cecilio, Granada, Granada, Spain, ¹⁷Department of Internal Medicine, Hospital de Santa Coloma de Gramanet, Barcelona, Barcelona, Spain, ¹⁸Department of Internal Medicine, Hospital General San Jorge, Huesca, Huesca, Spain, ¹⁹Department of Internal Medicine, Hospital de Santa Caterina, Girona, Girona, Spain, ²⁰Department of Internal Medicine, Hospital Virgen Macarena, Sevilla, Sevilla, Spain, ²¹Department of Internal Medicine, Hospital Infanta Sofía, San Sebastián, San Sebastian, Spain, ²²Department of Internal Medicine, Hospital de Poniente El Ejido, Almería, Almería, Spain, ²³Department of Internal Medicine, Hospital Universitario Puerta del Mar, Cádiz, Cadiz, Spain, ²⁴Department of Internal Medicine, Hospital da Barbanza, A Coruña, A Coruña, Spain, ²⁵Department of Internal Medicine, Complejo Hospitalario Albacete, Albacete, Albacete, Spain, ²⁶Department of Internal Medicine, Hospital Gregorio Marañón, Madrid, Madrid, Spain, ²⁷Department of Internal Medicine, Hospital Infanta Leonor, Madrid, Madrid, Spain, ²⁸Department of Internal Medicine, Hospital La Paz, Madrid, Madrid, Spain, ²⁹Department of Internal Medicine, Hospital Valle del Guadiato, Córdoba, Cordoba, Spain, ³⁰Department of Internal Medicine, Parc Sanitari San Joan de Déu, San Boi de Llobregat, Barcelona, Spain, ³¹Department of Internal Medicine, Hospital De Manises, Valencia, Valencia, Spain, ³²Department of Medicine, Hospital CIMA-Sanitas, Barcelona, Barcelona, Spain, ³³Department of Internal Medicine, Hospital Nuestra Señora del Prado, Talavera, Talavera, Spain, ³⁴Rheumatology Unit, Hospital Privado Centro Médico de Córdoba, Argentina, Córdoba, Argentina, ³⁵Laboratory of Systemic Autoimmune Diseases “Josep Font”, CELLEX, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Department of Systemic Autoimmune Diseases, ICMID, Hospital Clinic, Barcelona, Spain, Barcelona, Spain

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords: data collection, epidemiologic methods and sarcoidosis

Session Information

Date: Sunday, November 13, 2016

Title: Miscellaneous Rheumatic and Inflammatory Diseases - Poster I

Session Type: ACR Poster Session A

Session Time: 9:00AM-11:00AM

Background/Purpose: To characterize the main epidemiological, clinical and radiological features at presentation of sarcoidosis in a large multicenter cohort from Southern Europe.

Methods: In January 2016, the Autoimmune Diseases Study Group (GEAS-SEMI) created a national registry (SARCOGEAS) of patients with sarcoidosis. Sarcoidosis was diagnosed in agreement with the criteria proposed by the American Thoracic Society/European Respiratory Society/World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) 1999 statement on sarcoidosis. Organ involvement was retrospectively determined in each patient at the time of diagnosis using the 2014 WASOG organ assessment instrument. Ethnicity was defined according to the FDA classification. Two outcomes (association with neoplasia and death) were assessed at the end of follow-up.

Results: The cohort consisted of 979 patients (85% biopsy-proven), including 562 (57%) women and 417 (43%) men, with a mean age at diagnosis of 47.02 ± 15.44 years. One hundred twenty-three (13%) patients were born outside Spain. With respect to the FDA ethnic classification, 858 (88%) patients were classified as White, 83 (8%) as Hispanic, 21 (2%) as Black/African American and 17 (2%) as Asian. Thoracic involvement was present at diagnosis in 910 (93%) patients. With respect to diagnostic tests, data on the radiographic stage at diagnosis was available in all but 15 patients, with stage II (38%) and stage I (30%) being the most frequently-reported patterns. According to the WASOG classification, the most frequently reported extrathoracic involvements at diagnosis were cutaneous in 334 (34%) patients, extrathoracic lymph nodes in 179 (18%), liver involvement in 120 (12%) and ocular involvement in 109 (11%). Potentially life-threatening WASOG involvements were reported in frequencies less than 10%, including neurological involvement in 67 (7%) patients, kidney involvement in 46 (5%) or cardiac involvement in 18 (2%). Therapeutic approaches included the use of oral glucocorticosteroids in 552 (56%) patients, immunosuppressive agents in 76 (8%, mainly methotrexate in 35 patients, azathioprine and 21 and mycophenolate in 7) and biological agents in 13 (1%, including infliximab in 9 cases, adalimumab in 4, rituximab in 2, etanercept in 1 and bevacizumab in 1). After a mean follow-up of 87.9 months, neoplasia was reported in 112 (11%) patients and death in 91 (9%).

Conclusion: This is one of the largest series of sarcoidosis reported out of the US, predominantly composed by White patients in nearly 90% of cases. Clinical presentation is dominated by adenopathies (both thoracic and extrathoracic) and cutaneous features (erythema nodosum), with lower frequencies in the main extrathoracic involvements than that reported in US and Japanese series.

Disclosure: P. Brito-Zerón, None; B. Kostov, None; M. Pérez de Lis, None; G. Fraile, None; R. Gómez De La Torre, None; M. Roca Herrera, None; B. De Escalante Yangüela, None; A. Alguacil, None; M. P. Perez Conesa, None; F. J. Rascón, None; J. S. Garcia Morillo, None; C. Feijoo Massó, None; E. Fonseca Aizpuru, None; M. Bonet, None; N. Faro Minguez, None; G. De La Red Bellvis, None; E. Calvo Begueria, None; A. Gómez Lozano, None; E. Peral Gutiérrez De Ceballos, None; J. F. Gómez Cerezo, None; G. Cruz Caparrós, None; P. Perez Guerrero, None; S. Rodríguez Fernández, None; A. Gato Diez, None; N. Toledo Samaniego, None; M. Akasbi, None; A. Robles, None; I. Ojeda, None; M. J. Vives, None; M. Penadés Vidal, None; C. Morcillo, None; M. De Vicente, None; S. Retamozo, None; L. Pallarés, None; M. Ramos-Casals, None; R. Pérez-Alvarez, None.

To cite this abstract in AMA style:

Brito-Zerón P, Kostov B, Pérez de Lis M, Fraile G, Gómez De La Torre R, Roca Herrera M, De Escalante Yangüela B, Alguacil A, Perez Conesa MP, Rascón FJ, Garcia Morillo JS, Feijoo Massó C, Fonseca Aizpuru E, Bonet M, Faro Minguez N, De La Red Bellvis G, Calvo Begueria E, Gómez Lozano A, Peral Gutiérrez De Ceballos E, Gómez Cerezo JF, Cruz Caparrós G, Perez Guerrero P, Rodríguez Fernández S, Gato Diez A, Toledo Samaniego N, Akasbi M, Robles A, Ojeda I, Vives MJ, Penadés Vidal M, Morcillo C, De Vicente M, Retamozo S, Pallarés L, Ramos-Casals M, Pérez-Alvarez R. How Does Sarcoidosis Present in Spain? Characteristics at Diagnosis of 979 Patients from the Sarcogeas-SEMI Registry [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/how-does-sarcoidosis-present-in-spain-characteristics-at-diagnosis-of-979-patients-from-the-sarcogeas-semi-registry/. Accessed .

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