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Abstract Number: 906

Sarcoidosis as a Systemic Disease. Clinical and Epidemiological Characterization of Systemic Phenotype in 1521 Patients

Soledad Retamozo1, Roberto Pérez-Alvarez 2, Carlos Feijoo-Massó 3, Begoña De Escalante 4, Andrés González-García 5, Joel Chara-Cervantes 6, Ricardo Gómez De La Torre 7, Miguel López Dupla 8, Ana Alguacil 9, Javier Rascón 10, Mercedes Pérez-Conesa 6, Mariona Bonet 11, Angel Robles 12, José Luis Callejas 13, Blanca Pinilla 14, Eva Fonseca Aizpuru 15, Patricia Perez Guerrero 16, Jose Salvador Garcia Morillo 17, Borja de Miguel 18, Miriam Akasbi 19, Silvia Ojea Varona 20, Gloria De La Red Bellvis 21, Eva Calvo Begueria 22, Jorge Francisco Gómez Cerezo 23, Cristina Soler i Ferrer 24, Enrique Peral Gutiérrez De Ceballos 25, Gracia Cruz-Caparrós 26, Sergio Rodríguez Fernández 27, Alberto Gato Diez 28, César Morcillo 29, Inmaculada Ojeda 30, M José Vives 31, María Penadés Vidal 32, Moisés De Vicente 33, Belchin Kostov 34, Lucio Pallarés 10, Pilar Brito-Zerón 35 and Manuel Ramos-Casals 36, 1Instituto De Investigaciones En Ciencias De La Salud, Univ. Nacional de Córdoba, Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto Universitario de Ciencias Biomédicas de Córdoba, Córdoba, Argentina., Cordoba, Argentina, 2Department of Internal Medicine, Hospital Alvaro Cunqueiro, Vigo, Spain., Vigo, Spain, 3Hospital Parc Tauli, Sabadell, Sabadell, Spain, 4Hospital Clinico, Zaragoza, Spain, Zaragoza, Spain, 5Hospital Ramon y Cajal, Madrid, Spain, Madrid, Spain, 6Hospital Universitario Miguel Servet, Zaragoza, Spain, Zaragoza, Spain, 7Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Spain, Oviedo, Spain, 8Systemic Autoimmune Diseases Unit, Hospital Joan XXIII, Tarragona, Spain., Tarragona, Spain, 9Hospital Virgen de la Salud, Toledo, Spain, Toledo, Spain, 10Hospital Son Espases, Palma de Mallorca, Spain, Palma de Mallorca, Spain, 11Department of Internal Medicine, Althaia, Xarxa Assistencial de Manresa, Spain, Manresa, Spain, 12Hospital La Paz, Madrid, Spain, Madrid, Spain, 13Hospital Clínico San Cecilio, Granada, Spain, Granada, Spain, 14Systemic Autoimmune Diseases Unit Medicine, Hospital Gregorio Marañón, Madrid, Spain, Madrid, Spain, 15Department of Internal Medicine, Hospital de Cabueñes, Gijón, Spain, Gijón, Spain, 16Department of Internal Medicine, Hospital Universitario Puerta del Mar, Cádiz, Spain, Cadiz, Spain, 17Department of Internal Medicine, Hospital Virgen del Rocio, Sevilla, Spain, Sevilla, Spain, 18Hospital 12 de Octubre, Madrid, Spain, Madrid, Spain, 19Hospital Infanta Leonor, Madrid, Madrid, Spain, 20Department of Internal Medicine, Hospital Clínico Universitario de Salamanca-IBSAL. Salamanca, Spain, Salamanca, Spain, 21Department of Internal Medicine, Hospital de Santa Coloma de Gramanet, Barcelona, Spain, Barcelona, Spain, 22Hospital Universitario Puerta del Mar, Cádiz, Spain, Cadiz, Spain, 23Hospital Infanta Sofía, Madrid, Spain, Madrid, Spain, 24Hospital de Sta Caterina, Girona, Spain, Girona, Spain, 25Department of Internal Medicine, Hospital Virgen Macarena, Sevilla, Spain, Sevilla, Spain, 26Department of Internal Medicine, Hospital de Poniente El Ejido, Almería, Spain, Almeria, Spain, 27Department of Internal Medicine, Hospital da Barbanza, A Coruña, Spain, A Coruña, Spain, 28Department of Internal Medicine, Complejo Hospitalario Albacete, Spain, Albacete, Spain, 29Systemic Autoimmune Diseases Unit, Hospital CIMA-Sanitas, Barcelona, Spain, Barcelona, Spain, 30Department of Internal Medicine, Hospital Valle del Guadiato, Córdoba, Spain, Cordoba, Spain, 31Parc Sanitari San Joan de Déu, Sant Boi, Barcelona, Spain, Barcelona, Spain, 32Department of Internal Medicine, Hospital De Manises, Valencia, Spain, Valencia, Spain, 33Department of Internal Medicine, Hospital Nuestra Señora del Prado, Talavera, Spain, Talavera, Spain, 34Primary Healthcare Transversal Research Group, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS). Primary Care Centre Les Corts, Consorci d’Atenció Primària de Salut Barcelona Esquerra (CAPSBE). Department of Statistics and Operations Research, Universitat Politècnica de Catalunya, Barcelona, Spain., Barcelona, Spain, 35Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, IDIBAPS-CELLEX, Department of Autoimmune Diseases, ICMiD, University of Barcelona, Hospital Clínic. Autoimmune Diseases Unit, Department of Medicine, Hospital CIMA- Sanitas, Barcelona, Spain., Barcelona, Spain, 36Department of Autoimmune Diseases, ICMiD. Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, IDIBAPS-CELLEX. Department of Medicine, University of Barcelona, Hospital Clínic, Barcelona, Spain., Barcelona, Spain

Meeting: 2019 ACR/ARP Annual Meeting

Keywords: epidemiologic methods and Clinical, sarcoidosis

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Session Information

Date: Sunday, November 10, 2019

Session Title: 3S104: Miscellaneous Rheumatic & Inflammatory Disease I: Expanding the Spectrum of Clinical Features in Rheumatological Disorders (903–908)

Session Type: ACR Abstract Session

Session Time: 4:30PM-6:00PM

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.

Results: The cohort consisted of 1521 patients (81% biopsy-proven), including 877 (58%) women and 644 (42%) men, with a mean age at diagnosis of 47.4 ± 15.2 years; 234 (15%) patients were born outside Spain. With respect to the FDA ethnic classification, 1370 (90%) patients were classified as White, 80 (5%) as Hispanic, 47 (3%) as Black/African American and 24 (2%) as Asian. Thoracic involvement was present at diagnosis in 1409 (92%) patients, including 427 (29%) patients presenting with stage I, 619 (41%) with stage II, 288 (19%) with stage III and 53 (3%) presenting with stage IV. Extrathoracic disease was reported in 1096 (72%), and systemic disease (defined as at least 2 extrathoracic organs involved) in 523 (34%). According to the WASOG classification, the most frequently reported extrathoracic involvements at diagnosis were cutaneous in 558 (37%) patients, extrathoracic lymph nodes in 309 (20%), liver involvement in 189 (12%) and ocular involvement in 156 (10%) (Figure). Potentially life-threatening WASOG involvements were reported in frequencies less than 10%, including neurological involvement in 98 (6%) patients, kidney involvement in 58 (4%) or cardiac involvement in 30 (2%). Need for systemic therapy was required in 756 (50%) and aggressiveness of therapy in 124 (8%). Therapeutic approaches at diagnosis included the use of oral glucocorticosteroids in 731 (49%) patients, immunosuppressive agents in 118 (8%) and biological agents in 22 (1.5%).

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. However, extrathoracic disease was reported in 3 out of 4 patients, and one third of patients presented with at least 2 different extrathoracic organs involved. These findings underline the systemic clinical expression of sarcoidosis, being mandatory a multidisciplinary management of the disease.


Disclosure: S. Retamozo, None; R. Pérez-Alvarez, None; C. Feijoo-Massó, None; B. De Escalante, None; A. González-García, None; J. Chara-Cervantes, None; R. Gómez De La Torre, None; M. López Dupla, None; A. Alguacil, None; J. Rascón, None; M. Pérez-Conesa, None; M. Bonet, None; A. Robles, None; J. Callejas, None; B. Pinilla, None; E. Fonseca Aizpuru, None; P. Perez Guerrero, None; J. Garcia Morillo, None; B. de Miguel, None; M. Akasbi, None; S. Ojea Varona, None; G. De La Red Bellvis, None; E. Calvo Begueria, None; J. Gómez Cerezo, None; C. Soler i Ferrer, None; E. Gutiérrez De Ceballos, None; G. Cruz-Caparrós, None; S. Rodríguez Fernández, None; A. Gato Diez, None; C. Morcillo, None; I. Ojeda, None; M. Vives, None; M. Penadés Vidal, None; M. De Vicente, None; B. Kostov, None; L. Pallarés, None; P. Brito-Zerón, None; M. Ramos-Casals, None.

To cite this abstract in AMA style:

Retamozo S, Pérez-Alvarez R, Feijoo-Massó C, De Escalante B, González-García A, Chara-Cervantes J, Gómez De La Torre R, López Dupla M, Alguacil A, Rascón J, Pérez-Conesa M, Bonet M, Robles A, Callejas J, Pinilla B, Fonseca Aizpuru E, Perez Guerrero P, Garcia Morillo J, de Miguel B, Akasbi M, Ojea Varona S, De La Red Bellvis G, Calvo Begueria E, Gómez Cerezo J, Soler i Ferrer C, Gutiérrez De Ceballos E, Cruz-Caparrós G, Rodríguez Fernández S, Gato Diez A, Morcillo C, Ojeda I, Vives M, Penadés Vidal M, De Vicente M, Kostov B, Pallarés L, Brito-Zerón P, Ramos-Casals M. Sarcoidosis as a Systemic Disease. Clinical and Epidemiological Characterization of Systemic Phenotype in 1521 Patients [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/sarcoidosis-as-a-systemic-disease-clinical-and-epidemiological-characterization-of-systemic-phenotype-in-1521-patients/. Accessed January 30, 2023.
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