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Rheumatology 2001; 40: 476-478
© 2001 British Society for Rheumatology
Letters to the Editor |
Pneumomediastinum and subcutaneous emphysema associated with dermatomyositis
Division of Rheumatology, Department of Internal Medicine and
1Department of Radiology, Medical Faculty, University of Osmangazi, Eski
ehir, Turkey
SIR, Dermatomyositis (DM) is a generalized disorder characterized by myositis and typical cutaneous findings. The lung is frequently affected, usually by interstitial pneumonitis [1]. Spontaneous pneumomediastinum has also been reported as a rare complication in DM [2–4]. Here we describe a patient with DM who developed interstitial pneumonitis complicated by pneumomediastinum and subcutaneous emphysema during steroid therapy.
A 28-yr-old man was seen in another hospital in December 1999 with complaints of general fatigue, anorexia, fever, symmetrical arthritis of the hands and erythema of the fingers and face. Laboratory examination showed haemoglobin 9 g/dl, white blood cell (WBC) count 8300/mm3, erythrocyte sedimentation rate (ESR) 30 mm/h, aspartate transaminase (AST) 93 –U/l (normally <37), alanine transaminase (ALT) 49 U/l (normally <42), lactate dehydrogenase (LDH) 1227 U/l (normal range 225–450), creatine kinase (CK) 287 U/l (normal range 24–190). Tests for antinuclear antibodies (ANA), anti-Jo-1 and rheumatoid factor were negative. Electroneuromyography showed myopathic abnormalities; biopsy specimens of the right deltoid muscle revealed degeneration of muscle fibres and a mild degree of mononuclear cell infiltration. Chest radiography showed a mild alveolar and interstitial pattern in the lower lobes of both lungs. Therefore, the diagnosis of DM and associated interstitial lung disease was made. Prednisolone 60 mg/day was started and the patient's arthritis, fever and fatigue normalized, but after 2 weeks of prednisolone administration the patient developed acute cough, dyspnoea and cyanosis. He was admitted to our centre in February 2000. Physical examination disclosed characteristic skin abnormalities consisting of heliotrope rash, periungual erythema and an erythematous rash over the dorsal sides of the hands, and Gottron's papules. Fine crackles were heard in the lower lungs bilaterally and subcutaneous emphysema of the neck and chest was noted. There was symmetrical proximal muscle weakness of the limbs. Laboratory findings were as follows: haemoglobin 13.6 g/dl, WBC count 10100/mm3, ESR 25 mm/h, CK 79 U/l (normal range 16–190), LDH 1450 U/l (normal range 240–480), AST 48 U/l (normal range 7–39), ALT 128 U/l (normal range 2–40). Tests for ANA, anti-DNA, Sm, RNP, Ro, La and Jo-1 were negative. Arterial blood gases at rest were as follows: PO2 38.9 mmHg, PCO2 27.5 mmHg, pH 7.51. Pulmonary function tests and bronchoalveolar lavage were not performed because they were thought to be dangerous. Electroneuromyography showed myopathic changes, and biopsy of the right quadriceps showed degeneration and regeneration of myofibres and a mild degree of mononuclear cell infiltration. Chest radiography showed the presence of mediastinal and subcutaneous emphysema. High-resolution computed tomography (CT) scanning of the chest revealed air in the spinal area, around the trachea, in the anterior mediastinum and the subcutaneous regions of the chest and neck in addition to an alveolar and interstitial pattern in the lower lobes of both lungs (Fig. 1
).
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Continuous oxygen supplementation was started. The patient was treated with i.v. cyclophosphamide 1 g monthly in addition to prednisolone 50 mg/day. Because of steroid-induced diabetes mellitus, the dose of steroid was tapered to 10 mg/day within a month. Complete resolution of the pneumomediastinum and subcutaneous emphysema was observed after 12 weeks. Muscle weakness and skin findings improved gradually. The need for oxygen supplementation at rest disappeared completely. At the sixth month of follow-up, muscle enzyme levels were normal. Arterial blood gases at rest were as follows: PO2 75.6 mmHg, PCO2 30 mmHg, pH 7.41. High-resolution CT scanning showed complete resolution of the alveolar pattern, pneumomediastinum and subcutaneous emphysema, but interstitial marking continued. No recurrence of the pneumomediastinum has been observed.
Polymyositis and DM are commonly associated with pulmonary disease, including the usual interstitial pneumonitis, diffuse alveolar damage and bronchiolitis obliterans with organizing pneumonia [5]. The risk factors for the pneumomediastinum in patients with DM include interstitial lung disease [4, 6], cutaneous vasculopathy [7, 9], mild or absent CK elevation [3, 7, 8], younger age [7] and steroid treatment [6, 7]. Our patient was young and his CK levels were within the normal limits when the pneumomediastinum developed. Although we did not observe any signs of vasculopathy, such as periungual infarction, pitting scars and erosive or ulcerative lesions, the role of vasculitis as a pathogenic mechanism in this complication cannot be excluded. When the pneumomediastinum developed, the patient had been using prednisolone for 2 weeks. There have been several reports of patients who presented with classical cutaneous features of DM without CK elevation and who developed progressive interstitial pneumonitis during corticosteroid therapy in spite of improvement of muscle weakness and rash [6]. Yamanishi et al. [6] suggested that the weakening effects of steroids on the interstitial tissues of the lungs might cause pneumomediastinum. Alternatively, fibrosis itself leads to cyst formation and the rupture of such cysts can result in pneumomediastinum. On the other hand, interstitial fibrosis is not an essential underlying condition for the development of pneumomediastinum, as some cases with pneumomediastinum reported in the literature had no findings of interstitial lung disease [2, 3, 9].
In conclusion, patients with DM who have cutaneous vasculitis, mild or absent CK elevation, young age and steroid treatment should be monitored carefully for the occurrence of pneumomediastinum and interstitial pneumonitis.
Notes
Correspondence to: C. Korkmaz, Vinelik mah, Alifuat Güven Cad., Akasya sok, 11/11 Eskiehir, 26020 Turkey. 
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