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Subclinical Pulmonary Hypertension in Childhood Systemic Lupus Erythematosus Associated with Minor Disease Manifestations

Pediatric Cardiology volume 38, pages 234–239 (2017)Cite this article

Abstract

The aim of this study was to evaluate pulmonary hypertension (PH) in 852 childhood-onset systemic lupus erythematosus (cSLE) patients. This was a large multicenter study conducted in 10 Pediatric Rheumatology Services of São Paulo state, Brazil. PH was defined as systolic pulmonary artery pressure >35 mmHg and/or measurement of the mean pulmonary artery pressure >25 mmHg and/or diastolic pressure >15 mmHg by transthoracic echocardiogram. Demographic data, clinical manifestations, disease activity score (SLEDAI-2K), disease damage score (SLICC/ACR-DI) and treatments were also evaluated. Statistical analysis was performed using Bonferroni correction (p < 0.002). PH was observed in 17/852 (2%) cSLE patients. Effort dyspnea occurred in 3/17, chest pain in 1/17 and right ventricle dysfunction in 3/17 cSLE patients. None had pulmonary thromboembolism or antiphospholipid syndrome. Further comparison between 17 cSLE with PH and 85 cSLE control patients without PH with similar disease duration [15 (0–151) vs. 15 (0–153) months, p = 0.448], evaluated at the last visit, revealed higher frequencies of fever (47 vs. 9%, p < 0.001), reticuloendothelial manifestations (41 vs. 7%, p < 0.001) and serositis (35 vs. 5%, p = 0.001) in the former group. Frequencies of renal and neuropsychiatric involvements and antiphospholipid syndrome, as well as the median of SLEDAI-2K and SLICC/ACR-DI scores, were comparable in both groups (p > 0.002). Normal transthoracic echocardiography was evidenced in 9/17 (53%), with median cSLE duration of 17.5 months (1–40) after PH standard treatment. PH was a rare manifestation of cSLE occurring in the first two years of disease. The majority of patients were asymptomatic with mild lupus manifestations. The underlying mechanism seemed not to be related to pulmonary thromboembolism and/or antiphospholipid syndrome.

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Acknowledgements

Our gratitude to Ulysses Doria-Filho for the statistical analysis. The authors thank the following Pediatric Rheumatology Divisions and colleagues for including their patients: Pediatric Rheumatology Unit, FMUSP (Adriana Sallum, Adriana Almeida Jesus, Cristina Miuki Abe Jacob, Gabriela Blay, Gabriella Erlacher Lube de Almeida, Heloisa Helena de Souza Marques, João Domingos Montoni da Silva, Joaquim Carlos Rodrigues, Laila Pinto Coelho, Luciana dos Santos Henriques, Lucia Campos, Mariana Ferriani, Maria Helena Vaisbich, Nadia Emi Aikawa, Roberta Cunha, Victor Marques, Werther Brunow de Carvalho); Pediatric Rheumatology Unit, UNIFESP (Ana Sakamoto, Andreia Simões Lopes, Aline Alencar, Daniela Petry Piotto, Giampaolo Faquin, Gleice Clemente, Maria Odete Esteves Hilário, Octavio Augusto Bedin Peracchi, Vanessa Bugni); Division of Rheumatology, FMUSP (Juliane A Paupitz, Glauce Leão Lima); UNESP (Priscila R. Aoki, Juliana de Oliveira Sato, Silvana Paula Cardin, Taciana Albuquerque Pedrosa Fernandes), Irmandade da Santa Casa de Misericórdia de São Paulo (Andressa Guariento, Eunice Okuda), State University of Campinas (Maraísa Centeville, Renata Barbosa, Roberto Marini), Ribeirão Preto Medical School—University of São Paulo (Francisco Hugo Gomes, Paola Pontes Pinheiro, Luciana Carvalho, Virginia Ferriani), Hospital Infantil Darcy Vargas (Jonatas Libório, Luciana Tudech Pedro Paulo, Cássia Barbosa), Hospital Municipal Infantil Menino Jesus (Tânia Caroline Monteiro de Castro) and Pontifical Catholic University of Sorocaba (Valéria C. Ramos).

Fundings

This study was supported by Grants from Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq 301805/2013-0 to RMRP, 303752/2015-7 to MTT, 301479/2015-1 to CSM, 305068/2014-8 to EB and 303422/2015-7 to CAS), Federico Foundation (to EB, RMRP and CAS) and by Núcleo de Apoio à Pesquisa “Saúde da Criança e do Adolescente” da USP (NAP-CriAd) to CAS.

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Affiliations

  1. Pediatric Rheumatology Unit, Children’s Institute, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil

    Pedro Anuardo, Monica Verdier, Natali W. S. Gormezano, Gabriela R. V. Ferreira, Juliana C. O. A. Ferreira, Nadia E. Aikawa & Clovis A. Silva

  2. Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, Av. Dr. Arnaldo, nº 455, 3º andar, sala 3190 - Cerqueira César, São Paulo, SP, 05403-010, Brazil

    Natali W. S. Gormezano, Rosa M. R. Pereira, Nadia E. Aikawa, Eloisa Bonfá & Clovis A. Silva

  3. Pediatric Echocardiography Unit, Children’s Institute, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil

    Gabriela N. Leal & Alessandro Lianza

  4. Pediatric Rheumatology Unit, Universidade Federal de São Paulo, São Paulo, Brazil

    Maria Teresa Terreri & Claudio A. Len

  5. Faculdade de Medicina de Botucatu, São Paulo State University (UNESP), Botucatu, São Paulo, Brazil

    Claudia S. Magalhães

  6. State University of Campinas, Campinas, Brazil

    Simone Appenzeller

  7. Irmandade da Santa Casa de Misericórdia de São Paulo, São Paulo, Brazil

    Maria Carolina dos Santos & Silvana B. Sachetti

  8. Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, São Paulo, Brazil

    Gecilmara S. Pilleggi

  9. Hospital Menino Jesus, São Paulo, Brazil

    Simone Lotufo

Authors
  1. Pedro Anuardo
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  2. Monica Verdier
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  3. Natali W. S. Gormezano
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  4. Gabriela R. V. Ferreira
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  5. Gabriela N. Leal
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  6. Alessandro Lianza
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  7. Juliana C. O. A. Ferreira
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  8. Rosa M. R. Pereira
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  9. Nadia E. Aikawa
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  10. Maria Teresa Terreri
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  11. Claudia S. Magalhães
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  12. Simone Appenzeller
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  13. Maria Carolina dos Santos
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  14. Silvana B. Sachetti
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  15. Claudio A. Len
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  16. Gecilmara S. Pilleggi
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  17. Simone Lotufo
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  18. Eloisa Bonfá
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  19. Clovis A. Silva
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Correspondence to Clovis A. Silva.

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Anuardo, P., Verdier, M., Gormezano, N.W.S. et al. Subclinical Pulmonary Hypertension in Childhood Systemic Lupus Erythematosus Associated with Minor Disease Manifestations. Pediatr Cardiol 38, 234–239 (2017). https://doi.org/10.1007/s00246-016-1504-6

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  • DOI: https://doi.org/10.1007/s00246-016-1504-6

Keywords

  • Pulmonary hypertension
  • Childhood systemic lupus erythematosus
  • Lung
  • Multicenter cohort