Differentiated Phenotypes at Diagnosis of Sarcoidosis According to the Scadding Classification: Analysis in 1230 Patients

Soledad Retamozo^1,2,3, Roberto Pérez-Alvarez⁴, Pilar Brito-Zerón^5,6, Belchin Kostov⁷, Carlos Feijoo Massó⁸, Guadalupe Fraile⁹, A. González-García¹⁰, Ricardo Gómez De La Torre¹¹, Begoña De-Escalante¹², Miguel Lopez-Dupla¹³, Ana Alguacil¹⁴, Joel Chara-Cervantes¹⁵, M. Pérez-Conesa¹⁶, Francisco-Javier Rascón¹⁷, Jose Salvador Garcia Morillo¹⁸, Patricia Perez Guerrero¹⁹, Eva Fonseca Aizpuru²⁰, Miriam Akasbi²¹, Mariona Bonet²², José Luis Callejas²³, Gloria De La Red Bellvis²⁴, Eva Calvo Begueria²⁵, Cristina Soler i Ferrer²⁶, Enrique Peral Gutiérrez De Ceballos²⁷, J. F. Gómez-Cerezo²⁸, Gracia Cruz Caparrós²⁹, Sergio Rodríguez Fernández³⁰, Blanca Pinilla³¹, Alberto Gato Diez³², A. Rolo³³, César Morcillo⁶, Angel Robles³⁴, Inmaculada Ojeda³⁵, M. J. Vives³⁶, Borja de-Miguel³⁷, María Penadés Vidal³⁸, Moisés De Vicente³⁹, Xavier Bosh⁴⁰, Marta Pérez-de-Lis⁴¹, A. González-García⁹, Carmen Yllera Gutiérrez⁴², B. Gracia-Tello⁴³, M. Pedrosa⁴⁴, A. Perez-Gonzalez⁴, Carles Tolosa⁴⁵, Antoni Sisó-Almirall⁴⁶, Lucio Pallarés⁴⁷ and Manuel Ramos-Casals⁵, ¹Instituto de Investigaciones en Ciencias de la Salud, Universidad Nacional de Córdoba, Consejo Nacional de Investigaciones Científicas y Técnicas (INICSA-UNC-CONICET), Cordoba, Argentina, 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, ³Rheumatology Unit, Hospital Privado Universitario de Córdoba, Institute University of Biomedical Sciences University of Córdoba (IUCBC), Cordoba, Argentina, ⁴Department of Internal Medicine, Hospital Alvaro Cunqueiro, Vigo, Vigo, Spain, ⁵Laboratory of Autoimmune Diseases Josep Font, IDIBAPS, Department of Autoimmune Diseases, ICMiD, Hospital Clinic, Barcelona, Barcelona, Spain, ⁶Systemic Autoimmune Diseases Unit, Hospital CIMA-Sanitas, Barcelona, Barcelona, Spain, ⁷Primary Care Research Group, IDIBAPS, Centre d’Assistència Primària ABS Les Corts, GESCLINIC, Barcelona, Barcelona, Spain, ⁸Department of Internal Medicine, Hospital Parc Taulí, Sabadell, Sadabell, Spain, ⁹Hospital Ramon y Cajal, Madrid, Madrid, Spain, ¹⁰Internal Medicine, Hospital Ramón y Cajal, Madrid, Madrid, Spain, ¹¹Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Oviedo, Spain, ¹²Hospital Clínico, Zaragoza, Zaragoza, Spain, ¹³Systemic Autoimmune Diseases Unit, Hospital Joan XXIII, Tarragona, Tarragona, Spain, ¹⁴Hospital Virgen de la Salud, Toledo, Toledo, Spain, ¹⁵Hospital Universitario Miguel Servet, Zaragoza, Zaragoza, Spain, ¹⁶Hospital Miguel Servet, Zaragoza, Zaragoza, Spain, ¹⁷Hospital Son Espases, Palma de Mallorca, Palma de Mallorca, Spain, ¹⁸Department of Internal Medicine, Hospital Virgen del Rocio, Sevilla, Sevilla, Spain, ¹⁹Department of Internal Medicine, Hospital Universitario Puerta del Mar, Cádiz, Cadiz, Spain, ²⁰Department of Internal Medicine, Hospital de Cabueñes, Gijón, Gijón, Spain, ²¹Department of Internal Medicine, Hospital Infanta Leonor, Madrid, Madrid, Spain, ²²Department of Internal Medicine, Althaia, Xarxa Assistencial de Manresa, Manresa, Spain, ²³Hospital Clínico San Cecilio, Granada, Granada, Spain, ²⁴Department of Internal Medicine, Hospital de Santa Coloma de Gramanet, Barcelona, Barcelona, Spain, ²⁵Hospital Universitario Puerta del Mar, Cádiz, Cádiz, Spain, ²⁶Hospital de Sta Caterina, Girona, Girona, Spain, ²⁷Department of Internal Medicine, Hospital Virgen Macarena, Sevilla, Sevilla, Spain, ²⁸Hospital Infanta Sofía, Madrid, Madrid, Spain, ²⁹Department of Internal Medicine, Hospital de Poniente El Ejido, Almería, Almería, Spain, ³⁰Department of Internal Medicine, Hospital da Barbanza, A Coruña, A Coruña, Spain, ³¹Systemic Autoimmune Diseases Unit Medicine, Hospital Gregorio Marañón, Madrid, Madrid, Spain, ³²Department of Internal Medicine, Complejo Hospitalario Albacete, Albacete, Albacete, Spain, ³³Hospital Salamanca, Salamanca, Salamanca, Spain, ³⁴Hospital La Paz, Madrid, Madrid, Spain, ³⁵Department of Internal Medicine, Hospital Valle del Guadiato, Córdoba, Cordoba, Spain, ³⁶Parc Sanitari San Joan de Déu, Sant Boi, Barcelona, Spain, ³⁷Hospital 12 de Octubre, Madrid, Madrid, Spain, ³⁸Department of Internal Medicine, Hospital De Manises, Valencia, Valencia, Spain, ³⁹Department of Internal Medicine, Hospital Nuestra Señora del Prado, Talavera, Talavera, Spain, ⁴⁰Department of Internal Medicine, ICMiD, Hospital Clinic, Barcelona, Barcelona, Spain, ⁴¹Servicio de Anestesiologia y Reanimación. Complejo Hospitalario Universitario de A Coruña, A Coruña, A Coruña, Spain, ⁴²Hospital Universitario Central de Asturias, Oviedo, Oviedo, Spain, ⁴³Hospital Clinico, Zaragoza, Zaragoza, Spain, ⁴⁴Hospìtal Parc Taulí, Sadabell, Sabadell, Spain, ⁴⁵Department of Internal Medicine, Corporación Sanitaria Universitaria Parc Taulí, Barcelona, Spain, ⁴⁶Grup de Recerca Transversal en Atenció Primària, IDIBAPS, Barcelona, Barcelona, Spain, ⁴⁷Department of Internal Medicine, Hospital Son Espases. Palma de Mallorca, Palma de Mallorca, Spain

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords: Phenotypes and sarcoidosis

Session Information

Date: Tuesday, October 23, 2018

Title: Miscellaneous Rheumatic and Inflammatory Diseases Poster III: Sarcoid, Inflammatory Eye Disease, and Autoinflammatory Disease

Session Type: ACR Poster Session C

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

Background/Purpose: To analyse the correlation between Scadding radiological stages and the main phenotypic features at diagnosis (epidemiology, clinical patterns at presentation, extrathoracic disease and need for initial systemic therapy) in one of the largest cohorts of patients with sarcoidosis reported from Southern Europe.

Methods: The SARCOGEAS-Study Group was formed in 2015 including a multicenter database of consecutive patients diagnosed with sarcoidosis according to the WASOG 1999 criteria. Extrathoracic disease at diagnosis was defined according to the 2014 WASOG organ assessment instrument and the extrathoracic clusters proposed by Schupp et al.

Results: 1230 patients were finally analysed (712 women, mean age 47.3 yrs). Scadding radiologic stage at diagnosis consisted of stage 0 in 98 (8%) patients, stage I in 395 (32%), stage II in 500 (41%), stage III in 195 (16%) and stage IV in 42 (3%) patients. Epidemiologically, age at diagnosis was clearly linked with the Scadding stage, with younger mean ages being reported for stages I and II (p=0.001). With respect to extrathoracic disease, the highest frequencies of the organ-by-organ WASOG involvements are reported in stage 0 (skin, liver, spleen, ENT, nervous system and bone marrow), stage I (salivary glands, bone/joint) and stage IV (extra-thoracic lymph node, eye, kidney, calcium-vitamin D and heart) (FIGURE 1). The three SchappÕs extrathoracic clusters are overrepresented in stage 0. Patients with stage IV had the highest frequencies of both need of and aggressiveness of therapy, while patients with stage I had the lowest frequencies. Pulmonary fibrosis was related to a higher mean age at diagnosis (55 vs 47 yrs, p<0.001), a higher mean number of extrathoracic organs involved (1.5 vs 1.1, p=0.045), a higher frequency of calcium/vitamin D WASOG involvement (17% vs 7%, p=0.047) and a higher frequency of need for therapy (81% vs 49%, p<0.001); no statistically-significant differences were found between the stages II and III.

Conclusion: We found a significant association between Scadding stages and sarcoidosis phenotype at the time of diagnosis, including epidemiological profile, extrathoracic involvement and initial therapeutic management. However, the key determinant in phenotyping the disease at diagnosis was the involvement of each organ more than the classification using the Scadding stages.

Figure 1.

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

To cite this abstract in AMA style:

Retamozo S, Pérez-Alvarez R, Brito-Zerón P, Kostov B, Feijoo Massó C, Fraile G, González-García A, Gómez De La Torre R, De-Escalante B, Lopez-Dupla M, Alguacil A, Chara-Cervantes J, Pérez-Conesa M, Rascón FJ, Garcia Morillo JS, Perez Guerrero P, Fonseca Aizpuru E, Akasbi M, Bonet M, Callejas JL, De La Red Bellvis G, Calvo Begueria E, Soler i Ferrer C, Peral Gutiérrez De Ceballos E, Gómez-Cerezo JF, Cruz Caparrós G, Rodríguez Fernández S, Pinilla B, Gato Diez A, Rolo A, Morcillo C, Robles A, Ojeda I, Vives MJ, de-Miguel B, Penadés Vidal M, De Vicente M, Bosh X, Pérez-de-Lis M, González-García A, Yllera Gutiérrez C, Gracia-Tello B, Pedrosa M, Perez-Gonzalez A, Tolosa C, Sisó-Almirall A, Pallarés L, Ramos-Casals M. Differentiated Phenotypes at Diagnosis of Sarcoidosis According to the Scadding Classification: Analysis in 1230 Patients [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/differentiated-phenotypes-at-diagnosis-of-sarcoidosis-according-to-the-scadding-classification-analysis-in-1230-patients/. Accessed .

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