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Refractory chylous pleural effusion with systemic lupus erythematosus

Surgical treatment when steroid/immunosuppressant resistant

Therapierefraktärer chylöser Pleuraerguss bei systemischem Lupus erythematodes

Operative Behandlung bei Steroid‑/Immunsuppressivaresistenz

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Abstract

We analyzed four cases of systemic lupus erythematosus (SLE) with chylous pleural effusion treated in our hospital from June 2013 to May 2017. The three females and one male ranged in age from 22 to 33 years. The length of disease course was 20 to 120 months. Treatment with glucocorticoids and immunosuppressants (cyclophosphamide or cyclosporine A) did not reduce chylous pleural effusion. Lymphatic obstruction was found in all patients after direct lymphangiography and the pleural effusion was significantly reduced after surgery. A literature search identified six previously reported patients with SLE and chylous pleural effusion. Five were sensitive to classic immunosuppression treatment, probably due to early intervention. However, as shown here, the thoracic duct can be involved in SLE, leading to an intractable chylous pleural effusion with no response to medical treatment. Surgery would be a better choice when lymphatic mechanical obstruction is found.

Zusammenfassung

Die Autoren werteten 4 Fälle von systemischem Lupus erythematodes (SLE) mit chylösem Pleuraerguss aus, die zwischen Juni 2013 und Mai 2017 in der Klinik der Autoren behandelt worden waren. Die Patienten (3 w., 1 m.) waren zwischen 22 und 33 Jahre alt. Die Dauer des Krankheitsverlaufs betrug 20–120 Monate. Durch Behandlung mit Glukokortikoiden und Immunsuppressiva (Cyclophosphamid oder Ciclosporin) verminderte sich der chylöse Pleuraerguss nicht. In der direkten Lymphangiographie wurden bei allen Patienten lymphatische Obstruktionen festgestellt, nach operativer Therapie war der Pleuraerguss jeweils signifikant reduziert. Eine Literaturrecherche ergab 6 bereits dokumentierte Fälle von Patienten mit SLE und chylösem Pleuraerguss. Fünf sprachen auf eine klassische Immunsuppressionstherapie an, wahrscheinlich aufgrund einer frühzeitigen Intervention. Wie hier gezeigt, kann der Ductus thoracicus jedoch bei SLE mitbetroffen sein, und es kann zu einem hartnäckigen chylösen Pleuraerguss ohne Ansprechen auf eine medikamentöse Behandlung kommen. Bei Vorliegen einer lymphatischen mechanischen Obstruktion stellt eine Operation daher die bessere Wahl dar.

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Authors and Affiliations

  1. Department of Respiratory Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No. 1 Shuaifuyuan, 100730, Beijing, Dongcheng District, China

    Peng Song & Li Zhang

  2. Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

    JingCheng Zhang

  3. Department of Respiratory Medicine, Henan Provincial Peoples Hospital, Zhengzhou, China

    CongCong Shang

Authors
  1. Peng Song
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  2. JingCheng Zhang
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  3. CongCong Shang
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  4. Li Zhang
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Correspondence to Li Zhang.

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P. Song, J. Zhang, C. Shang, and L. Zhang declare that they have no competing interests.

This article does not contain any studies with human participants or animals performed by any of the authors.

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U. Müller-Ladner, Bad Nauheim

U. Lange, Bad Nauheim

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Song, P., Zhang, J., Shang, C. et al. Refractory chylous pleural effusion with systemic lupus erythematosus. Z Rheumatol 78, 797–802 (2019). https://doi.org/10.1007/s00393-018-0545-z

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  • DOI: https://doi.org/10.1007/s00393-018-0545-z

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