Primary Respiratory Disease in Patients with Systemic Lupus Erythematosus: Data from the Spanish Rheumatology Society Lupus Registry (RELESSER) Cohort

Javier Narváez¹, Helena Borrell², Fernando Sánchez-Alonso³, Iñigo Rúa-Figueroa⁴, Francisco Javier López Longo⁵, María Galindo⁶, Jaime Calvo-Alén⁷, Jose Luis Andreu⁸, Mariano Andres⁹, Juan José Alegre¹⁰, Ricardo Blanco¹¹, Tatiana Cobo-Ibáñez¹², Gema Bonilla¹³, Alina Boteanu¹⁴, Elvira Diez Alvarez¹⁵, Antonio Fernandez-Nebro¹⁶, Mercedes Freire¹⁷, Marian Gantes¹⁸, Paloma Garcia de la Peña¹⁹, Rosario García-Vicuña²⁰, José Hernández Beiraín²¹, Maria Loreto Horcada²², Jesus Ibañez²³, Antonio Juan²⁴, Nuria Lozano-Rivas²⁵, José Luis Marenco de la Fuente²⁶, Rafael-Benito Melero González²⁷, Carlos Alberto Montilla-Morales²⁸, Mireia Moreno²⁹, Alejandro Olivé³⁰, Maria Teresa Oton Sanchez³¹, Angela Pecondon-Español³², Esther Ruiz Lucea³³, Ana Sánchez Atrio³⁴, Gregorio Santos-Soler³⁵, Francisco Toyos³⁶, Esther Uriarte Isacelaya³⁷, Tomas Ramón Vazquez Rodriguez³⁸, Joan Miquel Nolla² and JM Pego-Reigosa³⁹, ¹Rheumatology Department, Hospital de Bellvitge. Barcelona. Spain, L’Hospitalet de Llobregat, Spain, ²Rheumatology, Hospital Universitario de Bellvitge, Barcelona, Spain, ³Unidad de Investigación, Spanish Society of Rheumatology, Madrid, Spain, ⁴Rheumatology Division, Hospital Doctor Negrin, Las Palmas GC, Spain, ⁵Rheumatology, Hospital Gregorio Marañón, Madrid, Spain, ⁶Servicio de Reumatología, Hospital 12 de Octubre, Madrid, Spain, ⁷Rheumatology, Hospital de Sierrallana. Torrelavega. Cantabria. Spain, Alava, Spain, ⁸Rheumatology, Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain, ⁹RHEUMATOLOGY, Hospital general universitario de Alicante, Alicante, Spain, ¹⁰Sección de Reumatología Hospital Universitario Dr Peset Valencia, Valencia, Spain, ¹¹Rheumatology, Hospital Universitario Marqués de Valdecilla. IDIVAL. Santander. Universidad de Cantabria. Spain, Santander, Spain, ¹²Hospital Universitario Reina Sofía, Universidad Europea de Madrid, Madrid, Spain, ¹³Hospital Universitario La Paz, Madrid, Spain, ¹⁴Rheumatology, Hospital Universitario Ramon y Cajal, Madrid, Spain, ¹⁵Complejo Asistencial Universitario de León. León. Spain, León, Spain, ¹⁶Rheumatology, Regional Universitary Hospital of Málaga, Malaga, Spain, ¹⁷Servicio de Reumatología. Instituto de Investigación Biomédica de A Coruña (INIBIC). Complexo HospitalarioUniversitario de A Coruña (CHUAC), Sergas. Universidade da Coruña (UDC), A Coruña, Spain, ¹⁸Rheumatology, Hospital Universitario de Canarias, La Laguna; Tenerife, Spain, ¹⁹Rheumatology, Hospital Madrid Norte Sanchinarro, Madrid, Spain, ²⁰Rheumatology, Hospital Universitario de La Princesa. IIS La Princesa, Madrid, Spain, ²¹Rheumatology, Hospital Insular de Gran Canaria, Las palmas Gran Canarias, Spain, ²²Rheumatology, Hospital de Navarra, Pamplona, Spain, ²³Rheumatology, Hospital Povisa, Vigo, Spain, ²⁴Rheumatology, Hospital Son LLatzer, Palma de Mallorca, Spain, ²⁵Rheumatology, Hospital Virgen de la Arrixaca, murcia, Spain, ²⁶Rheumatology, Hospital de Valme, Seville, Spain, ²⁷Rheumatology, EOXI Vigo, Vigo, Spain, ²⁸Hospital Clínico Universitario de Salamanca, Salamanca, Spain, ²⁹Rheumatology, Parc Tauli Hospital Universitari, Sabadell, Spain, ³⁰Rheumatology, Hospital Germans Trias i Pujol, Badalona, Spain, ³¹Rheumatology Department. Hospital Universitario Puerta de Hierro Majadahonda, Majadahonda, Spain, ³²Rheumatology, Hospital Miguel Servet, Zaragoza, Spain, ³³Rheumatology, Hospital de Basurto, Bilbao, Spain, ³⁴University Hospital Príncipe de Asturias, Immune System Diseases, Rheumatology Department, Alcalá de Henares, Madrid, Spain, ³⁵Rheumatology, Hospital Marina Baixa, Villajoyosa (Alicante), Spain, ³⁶Rheumatology, Hospital Universitario Virgen de la Macarena, Sevilla, Spain, ³⁷Rheumatology, Hospital Universitario de Donosti, San Sebastian, Spain, ³⁸Rheumatology, Hospital Universitario Lucus Augusti, Lugo, Spain, ³⁹Rheumatology Section, Hospital de Meixoeiro, Pontevedra, Spain, Vigo, Spain

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: Lung Disease and systemic lupus erythematosus (SLE)

Session Information

Date: Monday, November 6, 2017

Title: Systemic Lupus Erythematosus – Clinical Aspects and Treatment Poster II: Damage and Comorbidities

Session Type: ACR Poster Session B

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

Background/Purpose: To assess the prevalence, risk factors and impact on mortality of primary respiratory disease in a large retrospective cohort.

Methods: All adult patients in the Spanish Rheumatology Society Lupus Registry (RELESSER) who meet ≥ 4 ACR-97 SLE criteria were retrospectively investigated for the presence of primary pleuropulmonary manifestations.

Results: A total of 3215 patients were included: 91% were female, their mean (SD) age at diagnosis was 37 yrs (13.5), and the median follow-up time was 136 (97.7) months.

At least one pleuropulmonary manifestation was present in 11.3% (365) of the patients (289 (79.2%) patients developed only one manifestation; the rest had two or more). The mean duration of SLE until the appearance of the first respiratory manifestation was 5.8 (SD: 8.3) years.

The most common manifestation was pleurisy, which occurred in 21.1% (680) of the patients, followed by acute lupus pneumonitis (or interstitial alveolitis/pneumonitis according with BILAG 2004 definition) in 3.6% (118); pulmonary thromboembolism (including lung infarction) 2.9% (95); primary pulmonary hypertension in 2.4% (79); diffuse interstitial lung disease (or pulmonary fibrosis according with SLICC ACR Damage Index 1996) 2% (65); alveolar hemorrhage (BILAG 2004 definition) 0.8% (28); and shrinking lung syndrome (BILAG 2004 definition) 0.8% (28).

Twenty six patients with acute lupus pneumonitis and six patients with alveolar hemorrhage finally developed interstitial lung disease/pulmonary fibrosis.

In the multivariable analysis, the variables independently associated with the presence of pleuropulmonary manifestation were older age at disease onset (OR 1.03; 95% CI 1.02-1.04), higher SLEDAI scores (OR 1.03; 95% CI 1.00-1.07), the presence of Reynaud’s phenomenon (OR 1.41; 95% CI 1.09-1.84), secondary antiphospholipid syndrome (OR 2.20; 95% CI 1.63-2.96), anti-RNP positivity (OR 1.32; 95% CI 1.00-1.75), and the previous or concomitant presence of severe lupus nephritis (including classes III, IV, V and mixed III/IV + V) (OR 1.48; 95% CI 1.12-1.95), neuropsychiatric manifestations (OR 1.49; 95% CI 1.11-2.02), primary cardiac disease (OR 2.91; 95% CI 1.90-4.15), vasculitis (OR 1.81; 95% CI 1.25-2.62), hematological manifestations (OR 1.31; 95% CI 1.00-1.71) and gastrointestinal manifestations, excluding hepatitis (OR 2.05; 95% CI 1.14-3.66).

Sixty-one (1.89%) patients with pleuropulmonary manifestations died over the follow-up period. Although the mortality rate was low, the development of respiratory disease was associated with lower survival (survival rates 95.6% versus 82.2%; p=0.030). After adjusting for known confounders in the multivariable Cox regression model pleuropulmonary manifestations remained a risk factor for diminished survival (HR: 3.13; 95% CI 1.56–6.28, p=0.001).

Conclusion: Primary respiratory disease is not uncommon in patients with SLE and independently contributed to a decreased survival in these patients. This complication occurs mainly in patients with active and severe disease (with previous or concomitant major organ involvement other that lung) and seems to be associated with the positivity of antiphospholipid and anti-Sm antibodies.

Disclosure: J. Narváez, None; H. Borrell, None; F. Sánchez-Alonso, None; I. Rúa-Figueroa, None; F. J. López Longo, None; M. Galindo, None; J. Calvo-Alén, None; J. L. Andreu, None; M. Andres, None; J. J. Alegre, None; R. Blanco, None; T. Cobo-Ibáñez, None; G. Bonilla, None; A. Boteanu, None; E. Diez Alvarez, None; A. Fernandez-Nebro, None; M. Freire, None; M. Gantes, None; P. Garcia de la Peña, None; R. García-Vicuña, None; J. Hernández Beiraín, None; M. L. Horcada, None; J. Ibañez, None; A. Juan, None; N. Lozano-Rivas, None; J. L. Marenco de la Fuente, None; R. B. Melero González, None; C. A. Montilla-Morales, None; M. Moreno, None; A. Olivé, None; M. T. Oton Sanchez, None; A. Pecondon-Español, None; E. Ruiz Lucea, None; A. Sánchez Atrio, None; G. Santos-Soler, None; F. Toyos, None; E. Uriarte Isacelaya, None; T. R. Vazquez Rodriguez, None; J. M. Nolla, None; J. Pego-Reigosa, None.

To cite this abstract in AMA style:

Narváez J, Borrell H, Sánchez-Alonso F, Rúa-Figueroa I, López Longo FJ, Galindo M, Calvo-Alén J, Andreu JL, Andres M, Alegre JJ, Blanco R, Cobo-Ibáñez T, Bonilla G, Boteanu A, Diez Alvarez E, Fernandez-Nebro A, Freire M, Gantes M, Garcia de la Peña P, García-Vicuña R, Hernández Beiraín J, Horcada ML, Ibañez J, Juan A, Lozano-Rivas N, Marenco de la Fuente JL, Melero González RB, Montilla-Morales CA, Moreno M, Olivé A, Oton Sanchez MT, Pecondon-Español A, Ruiz Lucea E, Sánchez Atrio A, Santos-Soler G, Toyos F, Uriarte Isacelaya E, Vazquez Rodriguez TR, Nolla JM, Pego-Reigosa J. Primary Respiratory Disease in Patients with Systemic Lupus Erythematosus: Data from the Spanish Rheumatology Society Lupus Registry (RELESSER) Cohort [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/primary-respiratory-disease-in-patients-with-systemic-lupus-erythematosus-data-from-the-spanish-rheumatology-society-lupus-registry-relesser-cohort/. Accessed .

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