Fibrothorax and severe lung restriction secondary to lupus pleuritis and its successful treatment by pleurectomy

Section of Respirology, University of Manitoba, Winnipeg, Manitoba Correspondence and reprints: Dr S Sharma, BG034, 409 Tache Avenue, St Boniface Hospital, Winnipeg, Manitoba R2H 2A6. Telephone 204-237-2217, fax 204-231-1927, e-mail ssharma@sbgh.mb.ca S Sharma, R Smith, F Al-Hameed. Fibrothorax and severe lung restriction secondary to lupus pleuritis and its successful treatment by pleurectomy. Can Respir J 2002;9(5):335-337.

P ulmonary involvement may occur in 50% to 75% of patients with systemic lupus erythematosus (SLE) (1,2).The pleura is the most commonly affected site, and pleuritis and pleural effusion are the most common manifestations (3,4).
Most pleural effusions in patients with SLE resolve spontaneously; others require treatment with corticosteroids or a combination of corticosteroids and immunosuppressive agents.Although pleuritis may result in pleural thickening, progressive pleural fibrosis leading to fibrothorax has not been previously reported.A case of fibrothorax secondary to lupus pleuritis and its successful treatment by pleurectomy is reported.

CASE PRESENTATION
A 26-year-old woman presented with symptoms of arthralgia, alopecia, fatigue, weight loss and malar rash at the age of 19 years.Diagnosis of SLE was considered, and confirmed by low complement levels (C4), an antinuclear antibody titre of 1:10,240, and elevated single-and double-stranded DNA levels at 75 and 52 Bethesda units/mL, respectively.She was treated with prednisone and intervenous cyclophosphamide.Over the next one to two years, she developed pleuritic chest pain and exertional dyspnea, which progressed to the point that she became dyspneic at rest.There was no history of paroxysmal nocturnal dyspnea, palpitations, abdominal pain or peripheral edema.A physical examination showed her to be tachypneic at rest, with a respiratory rate of 32 breaths/min.Thoracic examination showed a marked reduction in thoracic excursion, especially of the right hemithorax; the results of a cardiovascular examination was normal.
The results of her pulmonary function tests were forced vital capacity (FVC) 1.06 L/s (24% predicted), forced expiratory volume in 1 s (FEV 1 ) 0.95 L/s (27% predicted), FEV 1 /FVC ratio 90%, total lung capacity 2.11 L (35% predicted), lung diffusing capacity for carbon monoxide 7.8 mL/min/mmHg (29% predicted), maximum inspiratory pressure 194 cm/H 2 O and maximal expiratory pressure 104 cm/H 2 O.A chest radiograph and computed tomography scan of the thorax revealed small right pleural effusion, pleural thickening and partial atelectasis of the right lower and right middle lobes (Figures 1 and 2).There was no evidence of interstitial lung disease.The 6 min walk test was discontinued because of dyspnea.A walking distance of 220 m was achieved; however, no desaturation was demonstrated.
Because the patient did not respond to medical therapy, surgical options were considered.A right thoracotomy, decortication of right visceral pleura and an open lung biopsy were performed.At the time of the surgery, dense adhesions were seen trapping the right lower lobe; thick visceral pleura limited the expansion of the right upper and middle lobes.A thick plural peel was excised by decortication.The lung biopsy confirmed plural fibrosis, but did not show evidence of lupus pneumonitis.Four months postoperatively, the patient's dyspnea markedly improved.The results of a pulmonary function test showed FVC 2.03 L/s (47% predicted), FEV 1 1.58 L/s (46% predicted), FEV 1 /FVC ratio 84%, total lung capacity 3.04 L (51% predicted) and lung diffusing capacity for carbon monoxide 12.5 mL/min/mmHg (47% predicted).The patient achieved 400 m on the 6 min walk test, and there was no desaturation and no dyspnea noted.Since the surgery, the patient has continued to be asymptomatic (Figure 3).

DISCUSSION
Pulmonary involvement occurs in more than one-half of patients with SLE during the course of their illness.Pulmonary manifestations include infectious pneumonia, pleuritis with or without effusion, acute lupus pneumonitis, alveolar hemorrhage, chronic interstitial pneumonitis or fibrosis, respiratory muscle weakness, pulmonary hypertension, pulmonary thromboembolism and upper airway dysfunction (1,2).The most common site of respiratory involvement in patients with SLE is the pleura.The presence of pleural effusion was noted in 7% of patients with SLE by echocardiography (3) and in 9% of patients with SLE by high resolution computed tomography scan (4).Effusions are typically small to moderate in size and are commonly bilateral, but may be unilateral.Pleural effusions in patients with SLE may resolve spontaneously or, alternatively, may respond well to therapy with corticosteroids (5).On rare occasions, chest tube drainage and pleurodesis with a sclerosing agent may be necessary in a few patients who are unresponsive to systemic anti-inflammatory therapy.Thickening of the visceral pleura has been previously reported in patients with SLE, but is rather rare (6,7).Progressive pleural fibrosis leading to fibrothorax has not been previously described in patients with SLE.We report, for the first time, the treatment of such a complication by decortication.
Bell and Lawrence (6) reported two cases of chronic pleurisy in patients with SLE treated with pleurectomy; these patients had unremitting pleural pain as the dominant symptom.Both of these patients failed conservative medical treatment, but pleurectomy resulted in a significant improvement of chronic pleural pain.In another article, Passero and Myers (8) reported two cases of hemopneumothorax in patients with SLE.Chest tube drainage failed to re-expand the lungs in these patients; therefore, surgical decortication led to the expansion of the collapsed lung and resolution of pneumothorax.Another case report in 1986 described the case of a 34-year-old man who developed recurrent, massive pleural effusions (9).Medical treatment with corticosteroids and azathioprine improved the manifestations of SLE, except for the pleural effusions.They were successfully treated by pleurectomy.In a similar report by Elborn et al (10), refractory massive pleural effusion was treated by pleurectomy.In our patient, severe restrictive pulmonary disease and disabling dyspnea resulted secondary to fibrothorax induced by lupus pleuritis.Aggressive medical therapy with corticosteroids and cyclophosphamide failed to improve the patient's symptoms.A pleurectomy resulted in a marked improvement in lung mechanics and resolution of symptoms.
Pleuritis and pleural effusion are the most common pulmonary manifestations of SLE.Although previously unreported, pleural thickening secondary to SLE may progress to fibrothorax.Previously published case series and the pres-ent report establish pleurectomy as a logical and definitive treatment for a variety of pleural disorders that may cause severe disability and are unresponsive to medical therapy.

Treatment of fibrothorax by pleurectomy
Can Respir J Vol 9 No 5 September/October 2002 337

Figure 1 )Figure 2 )
Figure 1) Chest radiograph showing marked restriction of right lung inflation during inspiration

Figure 3 )
Figure 3) Postpleurectomy chest radiograph demonstrating a marked improvement in right lung expansion