Rheumatology 2001; 40: 588-590
© 2001 British Society for Rheumatology
Paediatric Rheumatology |
Interstitial pneumonitis and air leakage in juvenile dermatomyositis
Paediatric Rheumatology/Series Editor: P. WooLetters to the Editor
Department of Pediatrics (MBC-58), King Faisal Specialist Hospital and Research Center, PO Box 3354, Riyadh 11211, Saudi Arabia
Interstitial pneumonitis and air leakage in juvenile dermatomyositis
SIR, Interstitial pneumonitis is a common pulmonary complication of dermatomyositis (DM) but associated air leakage is rarely described in juvenile dermatomyositis (JDM). We have recently looked after a child with severe JDM and associated pulmonary involvement complicated by recurrent spontaneous air leakage that was treated successfully with cyclosporin.
A 4-yr-old girl presented with a 1-month history of intermittent fever, skin rash and progressive generalized muscle weakness, associated with difficulty in swallowing and respiratory distress. Within 2 weeks she was unable to sit or stand and she became bedridden. Her weight was 12.8 kg (below the 5th percentile) and her height 98 cm (5th percentile). She was looking ill, with nasal speech and drooling of saliva. Her temperature was 38.5°C, heart rate 111 beats/min, respiratory rate 44/min and blood pressure 99/57 mmHg. She had a heliotrope rash over both eyelids and an ulcer at the right inner canthus, generalized subcutaneous oedema, and muscle tenderness with muscle strength grade 1. Lung examination showed bilateral basal crepitations and a normal cardiovascular system. Her abdomen was tender, with hepatomegaly 3 cm below the right costal margin and splenomegaly 2 cm below the left costal margin. The central nervous system revealed no sign of meningeal irritation; fundi and reflexes were normal. A diagnosis of JDM was made. Her initial investigation showed creatine kinase (CK) 1455 (24–195) U/l, lactate dehydrogenase 1834 (240–480) U/l. The chest X-ray was normal, an electromyographic study was consistent with myositis and muscle biopsy showed inflammatory myopathy. The antinuclear antibody titre was 1:160 with negative extractable nuclear antigens.
She was managed initially with i.v. methylprednisolone 30 mg/kg daily for 3 consecutive days then prednisone 2 mg/kg/day in two divided doses, after which she showed some improvement and started to move her legs, but she continued to have intermittent fever, tachycardia, tachypnoea and abdominal pain.
Screening for sepsis (blood, urine, and nasopharyngeal aspirate cultures) was negative. Because the response to treatment was not optimal, i.v. immunoglobulin 2 g/kg and weekly s.c. methotrexate (MTX) 10 mg (17 mg/m2) were added. A week later she deteriorated, with gastrointestinal bleeding, severe respiratory distress and hypoxia; chest X-ray showed pneumonic infiltration in the left lung. A computed tomography (CT) scan of the lung showed pneumomediastinum, bilateral basal atelectasis and emphysematous bullae in the left lung, which progressed to subcutaneous emphysema of the neck, bilateral pneumothorax and severe respiratory acidosis, which required bilateral chest tube insertion and admission to the intensive care unit.
In view of respiratory deterioration, i.v. cyclophosphamide (400 mg/m2) once every 2 weeks was started. There was no improvement, and the treatment was eventually discontinued because of severe leucopenia, which was managed with the use of granulocyte colony-stimulating factor. Bronchial alveolar lavage was negative for acid-fast bacilli, fungi and Pneumocystis carinii. Lung biopsy was performed, and histological examination was compatible with cytomegalovirus (CMV) interstitial pneumonia. Polymerase chain reaction (PCR), virus culture and serology for CMV were negative, and eye examination was normal. In spite of these results, ganciclovir and CMV immunoglobulin (Cytotec) were started, together with a prophylactic antibiotic against P. carinii infection. The patient's condition did not change, but she still required a high oxygen supply and there was persistent pulmonary air leakage. MTX was therefore discontinued after 4 weeks. Five daily sessions of plasmapheresis were given, which initially gave a better response in muscle strength but not the lung disease; the tachypnoea and respiratory acidosis persisted. As the patient did not respond to the current medication, cyclosporin 5 mg/kg was started, and she improved steadily. Progressive improvement was noted in muscle strength as well as lung disease (Fig. 1
). Gradual tapering of oral steroid and weaning from oxygen was begun. Repeated CT scanning of the lung showed significant improvement in lung parenchyma and air leakage (Fig. 2). However, the patient continued to have emphysematous bullae in the right lung. She was discharged after 4 months of hospital stay.
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The patient was readmitted after 2 weeks with recurrent right-sided pneumothorax and a collapsed left lung. Insertion of a right-sided chest tube failed to inflate her lung, so a right thoracotomy was done to excise the right-lobe bullae and pleurodesis was carried out. Four months after discharge she was able to walk, play and climb stairs independently. Currently, she is on prednisone 5 mg daily and cyclosporin 4 mg/kg, without significant side-effects but with mild hirsutism.
JDM is a multisystem disease characterized by vasculopathy of the skin and/or muscles causing symmetrical proximal muscle weakness and typical skin rash [1]. Pulmonary involvement is rarely reported in JDM. In contrast, up to 50% of adult patients with polymyositis and DM have been reported to have clinical lung disease [2]. Interstitial pneumonitis is the most common pulmonary complication of DM [3]. However; spontaneous air leakage (pneumothorax, pneumomediastinum and subcutaneous emphysema) has been reported rarely in both adult and paediatric patients with dermatomyositis [4, 5].
It is known that pulmonary involvement in DM is associated with a poor prognosis [6]. Mortality is very high in patients with JDM complicated by interstitial pneumonitis and air leakage [4, 5].
The exact mechanism responsible for the development of air leakage in DM is unknown. It may be secondary to interstitial pneumonitis, fibrosis or vasculopathy and associated infarction and necrosis of the blood vessels of the lung parenchyma and pleura [5, 7, 8]. Other underlying causes that may be responsible for these complications are viral infection and immune responses to latent viruses or viral products such as CMV. Such mechanisms may contribute to the pathogenesis of interstitial pneumonitis and its sequelae [9]. In our patient, lung biopsy revealed intracellular inclusion bodies compatible with CMV but PCR, conventional virus culture and serology for CMV were negative. Although there is no recognized effective therapy for interstitial pneumonitis and its sequelae in DM patients, different modalities of therapy have been tried [5, 7, 10]. Cyclosporin has been used with promising results in refractory JDM [11]; it may be the preferred treatment in steroid-resistant DM with interstitial pneumonitis, especially in the presence of side-effects of other immunosuppressive drugs [12]. In our patient, the institution of cyclosporin therapy may have contributed to the improvement of the myositis as well as to the interstitial pneumonitis.
In conclusion, interstitial pneumonitis is an uncommon complication of JDM, and may be associated with air leakage. Cyclosporin may be effective in interstitial pneumonitis associated with JDM.
Notes
Correspondence to: S. Al-Mayouf. 
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