LETTER TO THE EDITOR

Antisynthetase Syndrome With Subcutaneous Emphysema and Pneumomediastinum To the Editor: ermatomyositis (DM) is a systemic disease characterized by the presence of myositis and typical skin manifestations. The lung is usually affected by interstitial pneumonitis.1 Pneumomediastinum is the presence of free air in the mediastinal structures and may occur spontaneously as a rare complication of DM. Its pathogenesis has not been clearly defined and is believed to be attributed to interstitial pneumonitis.2 Although it has been reported that pneumomediastinum in DM could be lethal, a review by Yoshida et al.3 showed that fatal outcome occurred in context of interstitial lung disease (ILD). We report a patient with spontaneous pneumomediastinum when starting with muscle weakness associated with cutaneous manifestations compatible with DM. A 50-year-old man with a history of hypothyroidism, type 2 diabetes (non–insulin dependent), and hypertension consulted for intermittent fever, weight loss, generalized muscle weakness, progressive dyspnea, and facial erythema associated with scaly papules on the fingers lasting more than 8 weeks. Physical examination revealed facial edema, heliotrope rash, shawl sign, Gottron papules, mechanic hands, and weakness of the flexor muscles of the neck, shoulders, and hip without compromise in swallowing. In addition, he presented bilateral supraclavicular swelling with crepitus on palpation and

D

FIGURE 2. Bibasal septal thickening with reticular pattern and patchy alveolar consolidation in posterior segments of both lungs (arrows). Axial bronchiectasis.

generalized hypoventilation associated with Velcro rales in both lung bases. The laboratory reported positive speckled and nucleolar antinuclear antibody 1/160, anti–JO-1 positive, slightly elevated muscle enzymes (creatine phosphokinase 309 IU/L and aspartate aminotransferase 74 IU/L) and acute phase reactants (erythrocyte sedimentation rate and C-reactive protein). Electromyogram supported a myopathic pattern, and chest radiograph showed bilateral interstitial infiltrates and subcutaneous emphysema. Chest computed tomography showed pulmonary interstitial involvement with little ground glass, pneumomediastinum associated with pericardial compromise, and muscle planes dissection from neck to anterior chest wall (Figs. 1 and 2). Respiratory functional test showed a moderate restrictive pattern and a fall in diffusion capacity for carbon monoxide. The bronchoscopy showed no alteration of the mucosa.

FIGURE 1. Pneumomediastinum extending from anterior cervical region to the chest wall (arrows). JCR: Journal of Clinical Rheumatology • Volume 20, Number 7, October 2014

Neoplasia was excluded by upper and lower endoscopy, abdomen/pelvis computed tomography, and tumor markers. A diagnosis of an antisynthetase syndrome in a patient with DM was made, and treatment was started with methylprednisone 1 mg/kg per day and azathioprine 2 mg/kg per day. Once the patient had no clinical and radiological subcutaneous emphysema (3 months of treatment), new respiratory function tests with diffusion capacity for carbon monoxide were performed, and normalization of spirometry was observed (after 6 months). Currently, the patient has no subcutaneous emphysema, and ILD is in remission. Dermatomyositis is commonly associated with pulmonary disease, including aspiration pneumonia, hypoventilation secondary to inflammation of the respiratory muscles, and ILD.4 Risk factors in patients with DM and pneumomediastinum include ILD, cutaneous vasculopathy, normal or slightly increased muscle enzymes, corticosteroid treatment, and young age.5 The development of spontaneous pneumomediastinum in DM is considered secondary to air leakage through the walls of the alveoli because of the formation of bullae in patients with pulmonary fibrosis.6 Kono et al.2 found significant association of pneumomediastinum with cutaneous vasculopathy, and bronchial necrosis was found in 1 patient, suggesting their relationship with vasculopathy and disruption of bronchial mucosa. On the other hand, ILD is not a necessary condition for the development of pneumomediastinum.7 Primary spontaneous pneumothorax rarely happens during strenuous exercise.8 Once sealed, the rate of reabsorption of air www.jclinrheum.com

Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.

401

JCR: Journal of Clinical Rheumatology • Volume 20, Number 7, October 2014

Letter to the Editor

in the pleural space is 1.24% of the volume of the pneumothorax in each 24 hours, so recovery can take 16 days for a 20% pneumothorax.9 The main risk of pneumothorax is discomfort and pain rather than death. Most patients could therefore safely undertake lung function testing 2 weeks after treatment. Pneumothorax is a relative contraindication for lung function testing.10 In summary, the presence of subcutaneous emphysema in DM is a rare complication and could be considered a marker of poor prognosis when associated with ILD. The degree of immunosuppression required depends on the existence of ILD and the location and severity of spontaneous emphysema. Early detection and treatment with corticosteroids and immunosuppressants can, as in our case, contribute to the improvement of this rare manifestation.

Juan Pablo Vinicki, MD División Reumatología, Hospital de Clínicas Universidad de Buenos Aires Argentina [email protected]

402

www.jclinrheum.com

Santiago Catalán Pellet, MD División Reumatología, Hospital de Clínicas Universidad de Buenos Aires Argentina

Alejandro Raimondi, MD División Neumonología, Hospital de Clínicas Universidad de Buenos Aires Argentina

Diana Dubinsky, MD Gustavo Nasswetter, MD División Reumatología, Hospital de Clínicas Universidad de Buenos Aires Argentina

REFERENCES 1. Lakhanpal S, Lie JT, Conn DL, et al. Pulmonary disease in polymyositis/dermatomyositis: a clinicopathological analysis of 65 autopsy cases. Ann Rheum Dis. 1987;46:23–29. 2. Kono H, Inokuma S, Nakayama H, et al. Pneumomediastinum in dermatomyositis: association with cutaneous vasculopathy. Ann Rheum Dis. 2000;59:372–376. 3. Yoshida K, Kurosaka D, Kingetsu I, et al. Pneumomediastinum in dermatomyositis itself is not a poor prognostic factor: report of a case and review of the literature. Rheumatol Int. 2008;28:913–917.

4. Selva-O’Callaghan A, Labrador-Horrillo M, Muñoz-Gall X, et al. Polymyositis/ dermatomyositis-associated lung disease: analysis of a series of 81 patients. Lupus. 2005; 14:534–542. 5. Korkmaz C, Ozkan R, Akay M, et al. Pneumomediastinum and subcutaneous emphysema associated with dermatomyositis. Rheumatology (Oxford). 2001;40:476–478. 6. Matsuoka S, Kurihara Y, Yagihashi K, et al. Thin-section CT assessment of spontaneous pneumomediastinum in interstitial lung disease: correlation with serial changes in lung parenchymal abnormalities. Respir Med. 2006; 100:11–19. 7. Carmody E, McNicholl J, Chadwick G, et al. Prolonged spontaneous pneumomediastinum in adult dermatomyositis. Ann Rheum Dis. 1987; 46:566. 8. Bense L, Wiman LG, Hedenstierna G. Onset of symptoms in spontaneous pneumothorax: correlations to physical activity. Eur Respir Dis. 1987;71:181e6. 9. Kircher LT, Swartzel RL. Spontaneous pneumothoraxand its treatment. JAMA. 1954; 155:24. 10. Cooper BG. An update on contraindications for lung function testing. Thorax. 2011;66:714–723.

© 2014 Lippincott Williams & Wilkins

Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.

Antisynthetase syndrome with subcutaneous emphysema and pneumomediastinum.

Antisynthetase syndrome with subcutaneous emphysema and pneumomediastinum. - PDF Download Free
656KB Sizes 0 Downloads 7 Views