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

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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