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 Table of Contents  
Year : 2017  |  Volume : 1  |  Issue : 3  |  Page : 95-98

Lupus nephritis with spontaneously resolved bilateral pneumothorax and mediastinal emphysema

1 Department of Internal Medicine, Rashid Hospital, Dubai, United Arab Emirates
2 Department of Medicine, London Health Sciences Centre, Western University, Ontario, Canada

Date of Web Publication16-Feb-2018

Correspondence Address:
Salman Abdulaziz
Department of Internal Medicine, Rashid Hospital, PO Box 4545, Dubai
United Arab Emirates
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/sccj.sccj_21_17

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We report a 29-year-old female patient known to have lupus nephritis on immunosuppression and peritoneal dialysis who developed large bilateral spontaneous pneumothorax and mediastinal emphysema. The patient showed signs of tension pneumothorax and pneumomediastinum; however, she adamantly refused chest tubes insertion. Recovery was noted within 18 h of high-flow oxygen with normalization of chest image.

Keywords: Bilateral pneumothorax, lupus nephritis, mediastinal emphysema, pneumomediastinum

How to cite this article:
Abdulaziz S, Al-Thenayan E. Lupus nephritis with spontaneously resolved bilateral pneumothorax and mediastinal emphysema. Saudi Crit Care J 2017;1:95-8

How to cite this URL:
Abdulaziz S, Al-Thenayan E. Lupus nephritis with spontaneously resolved bilateral pneumothorax and mediastinal emphysema. Saudi Crit Care J [serial online] 2017 [cited 2022 Dec 8];1:95-8. Available from: https://www.sccj-sa.org/text.asp?2017/1/3/95/225730

  Introduction Top

Systemic lupus erythematosus (SLE) is an autoimmune disease with the involvement of multiple organs.[1] The respiratory system manifestations can be present in 30%–50% of lupus patients.[2] The first case of SLE with pneumothorax was reported in 1971 by Sawkar and Easom.[3] Herein, we describe the first case of bilateral spontaneous pneumothorax and pneumomediastinum associated with SLE that recovered without surgical intervention.

  Case Report Top

A 29-year-old female patient presented to the emergency room with a history of progressive shortness of breath for 4 days after excessive bouts of coughing, followed by a sudden sharp central chest pain with radiation to the back for 1 day. The patient had no history of fever or trauma; however, she had a history of forceful vomiting before admission. She had SLE with cerebritis and nephritis on oral mycophenolate mofetil and prednisone. She had previously been admitted to an Intensive Care Unit (ICU) 8 years before this presentation for pulmonary hemorrhage and was treated by pulse steroids and cyclophosphamide. During the previous admission, she required prolonged mechanical ventilation and percutaneous tracheostomy for several weeks. Peritoneal dialysis (PD) was started to treat the worsening kidney function in the last 8 months before this presentation.

She neither had a history of recently active SLE nor was she known to be smoker or to have a history of drug intoxication or similar illness in the past.

On examination, the patient was found to have sinus tachycardia and hypotension, as the presenting blood pressure was 70/40 mm Hg. She was tachypneic and hypoxic, with a respiratory rate of 26/min and oxygen saturation on room air of 67%. She was afebrile. Subcutaneous crepitus was palpable on the neck, indicating subcutaneous emphysema. The trachea was central, with hyperresonance on percussion note and reduced breath sounds by auscultation of both sides of the chest. The abdominal examination showed the PD catheter in place, otherwise normal.

Her supine chest X-ray showed subcutaneous emphysema in the neck and pneumomediastinum [Figure 1]. Chest computed tomography (CT) scan showed an intact tracheostomy scar, normal lung parenchyma, mediastinal emphysema, and large bilateral pneumothorax [Figure 2] and [Figure 3]. Abdomen CT scan confirmed the intact PD catheter in situ with no air in the peritoneal cavity [Figure 4]. Gastrografin swallow study showed no signs of esophageal perforation [Figure 5]. [Table 1] shows her laboratory findings at presentation.
Figure 1: Chest radiograph showing subcutaneous emphysema and pneumomediastinum

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Figure 2: Chest computed tomography scan showing pneumomediastinum, bilateral pneumothorax and subcutaneous emphysema. The lung parenchyma is normal in both sides

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Figure 3: Virtual bronchoscopic image showing intact tracheostomy scar

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Figure 4: Gastrograffin swallow study excluded esophageal perforation

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Figure 5: Abdominal computed tomography scan ruled out contrast leak from the peritoneal catheter and confirms in situ placement

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Table 1: Laboratory findings of the patient at presentation to the emergency department

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She was put on nonrebreather mask oxygen and fluid resuscitation through femoral central venous catheter in the emergency room. Broad-spectrum antibiotics (tazocin and vancomycin) were started to cover for possible mediastinal sepsis, and stress dose of hydrocortisone was added. She was admitted to the ICU and remained fasting. The patient refused chest tube insertion; however, she was maintained on 100% oxygen with close monitoring. She had clinical improvement after 18 h and recession of the chest X-ray findings. The patient was discharged home 3 days later with close follow-up after complete resolution of the pneumomediastinum, pneumothorax, and subcutaneous emphysema evident by repeated CT chest [Figure 6].
Figure 6: Chest computed tomography scan showing recovery from pneumomediastinum, pneumothorax and subcutaneous emphysema

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After discharge from the hospital, she presented to the outpatient clinic 2 weeks after discharge for a follow-up with no shortness of breath or chest pain. The patient had no recurrence of pneumothorax or pneumomediastinum for the 2 years after discharge.

  Discussion Top

SLE can affect multiple organs.[1] Pleurisy is the most common involvement of the respiratory system, while pneumothorax is very rare manifestation of lupus.[2],[4],[5]

Pneumothorax, air in the pleural sac can be explained in SLE patients by alveolar rupture secondary to interstitial pneumonitis, which leads to parenchymal cysts that degenerate later on, the rupture of subpleural cysts and recurrent infection with Moraxella catarrhalis.[4],[6] One case report of pneumothorax secondary to nocardia infection in SLE patient that was explained by the formation of a bronchopleural fistula.[7]

Cyclophosphamide had led to pulmonary fibrosis and pleural thickening and consequent pneumothorax, with few patients receiving repeated doses.[8]

Tissue fragility secondary to prolonged steroid treatment was associated with few cases of pneumothorax in lupus through the antagonism of the growth factors effect, which might be the case in our patient.[8]

Pneumomediastinum, due to high intrathoracic pressure against closed glottis, i.e., coughing and vomiting, might be complicated by pneumothorax through air dissection to the pleural space.[9] On the other hand, case reports of pneumomediastinum are related to poor technique of manipulation of the PD catheter and displacement of the later in different patients.[10],[11] However, our patient denied any history of inappropriate techniques in dealing with the PD catheter and CT scan of the abdomen, and this confirmed the PD catheter in situ.

Bilateral spontaneous pneumothorax incidence is 1%–4%, as reported by Graf-Deuel and Knoblauch in their case series.[12] Surgical intervention, especially with underlying pleura-pulmonary disease, is strongly recommended for those cases, as they might fail the conservative management.[13]

The international guidelines for the management of pneumothorax indicate the need for chest tube insertion if there are any signs of respiratory distress, hemodynamic compromise, or bilateral involvement.[13]

Sawkar and Easomrecommend surgical intervention for pneumothorax if the patient has a rheumatologic disease to prevent recurrence.[3]

Pleurectomy, pleural abrasion, partial lobectomy, or video-assisted thoracoscopy were effective in managing SLE patients who had recurrent pneumothorax, especially when considering the risk of steroids to induce another event or delay the healing of wound after inserting a chest tube.[8]

Our patient had a bilateral pneumothorax and pneumomediastinum due to different triggering factors such as coughing, vomiting, and steroids, which could make alveoli more fragile to tension on their wall. Fortunately, she had spontaneous recovery on 100% oxygen without surgical intervention.

  Conclusion Top

Pneumothorax is a rare manifestation of SLE. Spontaneous resolution of large pneumothorax is possible and should be explored more fully. Surgical intervention is a valuable option when SLE patients have significant pleural or lung parenchymal changes.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Tan EM, Cohen AS, Fries JF, Masi AT, McShane DJ, Rothfield NF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1982;25:1271-7.  Back to cited text no. 1
Pines A, Kaplinsky N, Olchovsky D, Rozenman J, Frankl O. Pleuropulmonary manifestations of systemic lupus erythematosus: Clinical features of its subgroups. Prognostic and therapeutic implications. Chest 1985;88:129-35.  Back to cited text no. 2
Sawkar LA, Easom HF. Recurrent spontaneous pneumothoraces in systemic lupus erythematosus. Chest 1971;60:604-5.  Back to cited text no. 3
Wilhelm M, Van Why SK. Pneumothoraces complicating systemic lupus erythematosus with nephritis. Pediatr Nephrol 2002;17:261-3.  Back to cited text no. 4
Masuda A, Tsushima T, Shizume K, Mochizuki T, Isono K, Demura H, et al. Recurrent pneumothoraces and mediastinal emphysema in systemic lupus erythematosus. J Rheumatol 1990;17:544-8.  Back to cited text no. 5
Richards AJ, Talbot IC, Swinson DR, Hamilton EB. Diffuse pulmonary fibrosis and bilateral pneumothoraces in systemic lupus erythematosus. Postgrad Med J 1975;51:851-5.  Back to cited text no. 6
Leong KP, Tee NW, Yap WM, Chee TS, Koh ET. Nocardiosis in patients with systemic lupus erythematosus. The Singapore Lupus Study Group. J Rheumatol 2000;27:1306-12.  Back to cited text no. 7
Tanaka N, Kusunoki Y, Kaneko K, Yamamoto T, Kaburaki M, Muraoka S, et al. Systemic lupus erythematosus complicated by recurrent pneumothorax: Case report and literature review. Nihon Rinsho Meneki Gakkai Kaishi 2010;33:162-8.  Back to cited text no. 8
Abolnik I, Lossos IS, Breuer R. Spontaneous pneumomediastinum. A report of 25 cases. Chest 1991;100:93-5.  Back to cited text no. 9
Cancarini GC, Manili L, Cristinelli MR, Bracchi M, Carli O, Maiorca R, et al. Pneumoperitoneum and pneumomediastinum in a CAPD patient with peritonitis. Perit Dial Int 1997;17:389-91.  Back to cited text no. 10
Zbroch E, Malyszko J, Kamocki Z, Myśliwiec M. Pneumomediastinum and subcutaneous emphysema – Unusual complications of automated peritoneal dialysis. Perit Dial Int 2003;23:97-8.  Back to cited text no. 11
Graf-Deuel E, Knoblauch A. Simultaneous bilateral spontaneous pneumothorax. Chest 1994;105:1142-6.  Back to cited text no. 12
MacDuff A, Arnold A, Harvey J; BTS Pleural Disease Guideline Group. Management of spontaneous pneumothorax: British thoracic society pleural disease guideline 2010. Thorax 2010;65 Suppl 2:ii18-31.  Back to cited text no. 13


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]

  [Table 1]

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