Session 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.
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 December 7, 2019.
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