Bilateral spontaneous pneumothorax in systemic lupus erythematosus and the role of low-dose intravenous immunoglobulin

Felix Liauw; Tiara Nien Paramita; Endah Citraresmi; Abdurrahman Hadi

doi:10.12701/jyms.2025.42.29

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Image vignette Pediatrics, Perinatology, and Child Health Bilateral spontaneous pneumothorax in systemic lupus erythematosus and the role of low-dose intravenous immunoglobulin: Felix Liauw¹, Tiara Nien Paramita², Endah Citraresmi², Abdurrahman Hadi³; Journal of Yeungnam Medical Science 2025;42:29.
DOI: https://doi.org/10.12701/jyms.2025.42.29
Published online: March 27, 2025

¹Division of Pediatric Emergency and Intensive Care, Harapan Kita National Women and Children Health Centre, Jakarta, Indonesia

²Division of Pediatric Allergy and Immunology, Harapan Kita National Women and Children Health Centre, Jakarta, Indonesia

³Division of Cardiovascular and Thoracic Surgery, Harapan Kita National Women and Children Health Centre, Jakarta, Indonesia

Corresponding author: Felix Liauw, MD Division of Pediatric Emergency and Intensive Care, Harapan Kita National Women and Children Health Centre, Jl. Letjen S. Parman Kav. 87, Slipi, Palmerah, West Jakarta 11420, Indonesia Tel: +62-5668284 ext. 6211 • E-mail: felixliauw@gmail.com

• Received: December 12, 2024 • Revised: March 20, 2025 • Accepted: March 22, 2025

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (https://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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An 11-year-old girl was diagnosed with systemic lupus erythematosus (SLE) in January 2021 based on clinical features including prolonged fever, hemolytic anemia, and immunological criteria (antinuclear antibody, 1:10,000; anti-Sjögren syndrome-related antigen A, +3; anti-Ro52, +3). Methylprednisolone and hydroxychloroquine were prescribed, with routine follow-up every 2 months. In January 2024, she presented to the emergency room with a 3-day history of dyspnea. Upon examination, the patient was irritable with tachycardia (206 beats/min), shock (blood pressure, 118/76 mmHg with a weak pulse and cold extremities), and chest retractions (respiratory rate, 72 breaths/min; oxygen saturation, 93%). Laboratory results revealed a normal complete blood count (hemoglobin, 13.1 g/dL; leukocyte count, 9,960/µL with 74.2% neutrophils; platelet count, 165,000/µL), elevated C-reactive protein level (129 mg/L), respiratory alkalosis (pH, 7.53; pCO₂, 18 mmHg; HCO₃^-, 15.3 mEq/L), normal complement levels (C3, 154 mg/dL; C4, 32 mg/dL), proteinuria (+2), hematuria (+2), and normal renal function (urea, 24 mg/dL; creatinine, 0.5 mg/dL). The Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) 2000 score was 9, indicating a mild to moderate flare (febrile, 1 point; proteinuria, 4 points; and hematuria, 4 points). Emergency chest radiography revealed left-sided tension pneumothorax (Fig. 1A). Pleural tapping was performed with intercostal drainage tube insertion. The patient’s respiratory function improved (Fig. 1B). A chest computed tomography scan showed left hydropneumothorax; active patchy consolidation in the left lung; multiple cavities in both lungs; and multiple lymphadenopathies in the mediastinum, bilateral axilla, and left supraclavicular regions (Fig. 2). On the 10th day, she developed severe chest pain and worsening dyspnea. Breath sounds were absent in the right lung, and emergency chest imaging revealed a right-sided tension pneumothorax (Fig. 1C). A right chest tube was inserted, leaving the patient with bilateral chest tubes (Fig. 1D). On the 14th day, she continued to experience dyspnea. Repeat chest radiography revealed worsening of the bilateral pneumothorax (Fig. 1E), and an open thoracotomy was planned. Before proceeding with surgery, the team administered intravenous immunoglobulin (IVIg) at 400 mg/kg. Remarkably, her condition improved within 48 hours (Fig. 1F). The patient was discharged several days later without chest tubes.

Recent guidelines have provided strategies for managing pneumothorax in adults; however, these strategies may differ in pediatric patients. In the present case, a chest tube was placed because of a tension pneumothorax, which caused respiratory distress. The recurrent pneumothorax was suspected to have resulted from an airway leak. Surgical intervention or video-assisted thoracoscopic surgery is often recommended for the treatment of bilateral pneumothorax. Nonsurgical approaches such as observation, supplemental oxygen, needle aspiration, and treatment of the underlying disease can be considered depending on the clinical presentation and size of the pneumothorax [1,2]. The team offered IVIg to this patient as an adjunctive therapy, which is occasionally considered a supplemental treatment for autoimmune diseases [3]. In the present case, the patient demonstrated normal diuresis and renal function; however, urinalysis revealed proteinuria and hematuria, which may be indicative of lupus nephritis. The mechanism of action of IVIg in SLE remains unclear; however, it is believed to enhance immune function and protect against the destruction of platelets and erythrocytes. Unfortunately, serum immunoglobulin G levels were not measured. An IVIg dose of 2 g/kg is considered to exert an immunomodulatory effect; however, this treatment is not covered by national insurance in some countries, such as Indonesia. Studies in adults have suggested that administering high-dose IVIg (2 g/kg) has a positive impact on SLE flares, particularly those involving hematological and cardiac issues, as well as on managing secondary or concomitant infection [4-6]. In the present case, a low dose of IVIg (400 mg/kg) was administered, which yielded favorable outcomes. A study conducted in Israel found clinical improvement and decreased SLEDAI scores in 62 patients with SLE treated with approximately 500 mg/kg IVIg. However, the study noted that thrombocytopenia, vasculitis, alopecia, and proteinuria did not improve significantly with low-dose IVIg [7]. Thus, the optimal dose of IVIg for SLE remains unclear and requires further investigation. In conclusion, low-dose IVIg therapy may be a viable treatment option for SLE-associated pneumothorax, potentially avoiding surgical intervention.

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Ethics statement

This study was exempt from review by the Institutional Review Board (IRB) of Harapan Kita National Women and Children Health Centre (IRB No: IRB/61/10/ETIK/2024). Written informed consent was obtained from the patient’s parents for participation in the study.

Conflicts of interest

No potential conflicts of interest relevant to this article were disclosed.

Funding

None.

Author contributions

Conceptualization: all authors; Formal analysis: FL, EC; Validation: FL, TNP, AH; Investigation: FL; Supervision: EC; Writing–original draft: FL, TNP, EC; Writing–review & editing: all authors.

Fig. 1.

Pre- and post-thoracentesis images of the lungs. (A) Left-sided tension pneumothorax. (B) Expanded image of the left lung after chest tube insertion. (C) Right-sided tension pneumothorax. (D) Expanded image of the right lung after chest tube insertion. (E) Air entrapment persists on both sides. (F) Improvement in both pneumothoraces after intravenous immunoglobulin.

Fig. 2.

Chest computed tomography scan. (A) Hydropneumothorax. (B) Multiple regions of patchy consolidation (arrows). (C) Multiple cavities (arrows).

References

1. Yousuf S, Cardenas S, Rezaee F. Pediatric pneumothorax: case studies and review of current literature. Respir Med Case Rep 2021;34:101548.Article PubMed PMC
2. Burnand K. Spontaneous pneumothorax [Internet]. London: British Association of Paediatric Surgeons; published 2020, updated 2024 [cited 2024 Dec 12]. https://www.baps.org.uk/subspecialties/thoracic/spontaneous-pneumothorax/.
3. Shoenfeld Y, Katz U. IVIg therapy in autoimmunity and related disorders: our experience with a large cohort of patients. Autoimmunity 2005;38:123–37.Article PubMed
4. Camara I, Sciascia S, Simoes J, Pazzola G, Salas V, Karim Y, et al. Treatment with intravenous immunoglobulins in systemic lupus erythematosus: a series of 52 patients from a single centre. Clin Exp Rheumatol 2014;32:41–7.PubMed
5. Toubi E, Kessel A, Shoenfeld Y. High-dose intravenous immunoglobulins: an option in the treatment of systemic lupus erythematosus. Hum Immunol 2005;66:395–402.Article PubMed
6. Nieto-Aristizábal I, Martínez T, Urbano MA, Posso-Osorio I, Plata IF, Garcia-Robledo JE, et al. Treatment with intravenous immunoglobulins in systemic lupus erythematosus: a single-center experience with 63 patients. Lupus 2019;28:1566–70.Article PubMed PDF
7. Sherer Y, Kuechler S, Jose Scali J, Rovensky J, Levy Y, Zandman-Goddard G, et al. Low dose intravenous immunoglobulin in systemic lupus erythematosus: analysis of 62 cases. Isr Med Assoc J 2008;10:55–7.PubMed

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Figure

Bilateral spontaneous pneumothorax in systemic lupus erythematosus and the role of low-dose intravenous immunoglobulin

Fig. 1. Pre- and post-thoracentesis images of the lungs. (A) Left-sided tension pneumothorax. (B) Expanded image of the left lung after chest tube insertion. (C) Right-sided tension pneumothorax. (D) Expanded image of the right lung after chest tube insertion. (E) Air entrapment persists on both sides. (F) Improvement in both pneumothoraces after intravenous immunoglobulin.

Fig. 2. Chest computed tomography scan. (A) Hydropneumothorax. (B) Multiple regions of patchy consolidation (arrows). (C) Multiple cavities (arrows).

Fig. 1.

Fig. 2.

Bilateral spontaneous pneumothorax in systemic lupus erythematosus and the role of low-dose intravenous immunoglobulin