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 Table of Contents  
IMAGES IN RHEUMATOLOGY
Year : 2017  |  Volume : 12  |  Issue : 1  |  Page : 54-55

Spontaneous pneumomediastinum in dermatomyositis


Department of Clinical Immunology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India

Date of Web Publication23-Feb-2017

Correspondence Address:
Durga Prasanna Misra
Department of Clinical Immunology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry - 605 006
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-3698.199129

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  Abstract 

Keywords: Dermatomyositis, pneumomediastinum, vasculitis


How to cite this article:
Jain A, Misra DP, Jain VK, Negi VS. Spontaneous pneumomediastinum in dermatomyositis. Indian J Rheumatol 2017;12:54-5

How to cite this URL:
Jain A, Misra DP, Jain VK, Negi VS. Spontaneous pneumomediastinum in dermatomyositis. Indian J Rheumatol [serial online] 2017 [cited 2017 Mar 28];12:54-5. Available from: http://www.indianjrheumatol.com/text.asp?2017/12/1/54/199129

Dermatomyositis is a rare systemic autoimmune disease characterized by proximal muscle weakness with pathognomonic rash. We report a patient who developed spontaneous pneumomediastinum while on treatment for dermatomyositis, a very unusual association, and discuss potential mechanisms for the same.

A young male presented with subacute onset inflammatory polyarthritis, proximal muscle weakness, exertional breathlessness, and ulcers on elbows, nasal bridge, and right middle finger with significant weight loss over 5 months. Examination revealed periorbital heliotrope rash, Gottron's papules, and vasculitic ulcers at the above-mentioned sites. He had significant neck, truncal, upper and lower limb proximal weakness with preserved reflexes, and normal sensory examination. Respiratory examination revealed bibasal fine crackles; other systems were unremarkable. Investigations revealed elevated serum creatine phosphokinase (347 IU/mL) and lactate dehydrogenase (438 IU/mL). Immunofluorescence for antinuclear antibody was negative. Immunoblot for myositis-specific and myositis-associated antibodies showed positivity for antibodies to SRP, PL7 and Ro52. Electromyogram was consistent with myopathy. Magnetic resonance imaging revealed short Tau inversion recovery hyperintensities in thigh muscles. Pulmonary function test showed restrictive pattern; high-resolution computerized tomography (HRCT) of chest suggested usual interstitial pneumonia pattern of interstitial lung disease (ILD). Screening for associated visceral malignancy was negative. With a diagnosis of dermatomyositis, he was treated with pulsed intravenous methylprednisolone for 3 days followed by high-dose oral glucocorticoids and monthly intravenous cyclophosphamide (750 mg/m 2). Over the next 10 days, his proximal muscle weakness improved with continued physiotherapy. However, on the 10th day of hospitalization, he suddenly became breathless at rest. New onset of breathlessness in a patient with dermatomyositis on immunosuppression made us consider chest infection (as a consequence of immunosuppression), respiratory muscle weakness (due to dermatomyositis), or heart failure (due to myocardial involvement). Examination revealed subcutaneous emphysema with clear lung fields and no evidence of heart failure. Plain radiograph of the neck (lateral view) showed air in the subcutaneous and prevertebral tissue planes [Figure 1]a. Chest X-ray [Figure 1]b and HRCT of thorax [Figure 2] revealed pneumomediastinum with pneumopericardium without pneumothorax. He had no risk factors for this such as prior instrumentation. Fiberoptic bronchoscopy, direct video laryngoscopy, barium contrast swallow, esophagoduodenoscopy, and contrast-enhanced computerized tomography of neck and thorax failed to reveal obvious site of air leak. Hence, the pneumomediastinum was attributed to dermatomyositis. He was treated conservatively with high flow oxygen and immunosuppression was continued. The pneumomediastinum improved over 3 weeks. He was continued on monthly pulses of intravenous cyclophosphamide with gradual tapering of oral glucocorticoids and had good clinical response over the 1st month of therapy.
Figure 1: (a) Plain radiograph of neck (lateral view) shows air in the subcutaneous and prevertebral planes (arrows). (b) Chest X-ray showing pneumomediastinum (arrow)

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Figure 2: High-resolution computerized tomography of chest showing air inside the mediastinum and subcutaneous tissue

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  Discussion Top


Spontaneous pneumomediastinum is a rare complication associated with dermatomyositis (estimated prevalence 2.2%),[1] reported in both adults and children.[2] It has also been rarely reported in association with other connective tissue diseases such as lupus or polyarteritis nodosa.[3] It may precede the onset of clinical muscle weakness in dermatomyositis (i.e., clinically amyopathic dermatomyositis).[4] Patients with cutaneous ulcers, normally or mildly elevated CPK levels, concomitant ILD, prior reduction of vital capacity of lungs, and those receiving high-dose intravenous corticosteroids are thought to be at a greater risk of developing this rare complication.[1],[4] A recent association of pneumomediastinum with anti-melanoma differentiation antigen 5 antibody has been reported.[4] Our patient had cutaneous vasculitic ulcers with ILD and had been treated with pulsed intravenous methylprednisolone, also had a prior reduction of vital capacity due to ILD, hence was predisposed to develop pneumomediastinum. Up to one-fourth of patients with dermatomyositis developing pneumomediastinum may succumb within the 1st month after pneumomediastinum.[4] Fortunately, our patient recovered completely.

Various hypotheses have been proposed to explain pneumomediastinum in dermatomyositis. Association with ILD has led to the suggestion of increased alveolar pressures in already diseased lungs causing rupture of previously existing bullae.[1],[5] However, our patient did not have any such bullae on the CT of the thorax before the onset of pneumomediastinum. Association with cutaneous vasculitic ulcers has lent credence to the hypothesis that involvement of small vessels may result in ischemic necrosis of the bronchial wall resulting in air leak.[1],[5] The link with high-dose steroid therapy has warranted the theory that weakening of the alveolar wall due to corticosteroids may cause subsequent air leak.[1] Our patient had cutaneous vasculitic ulcers; hence, it is reasonable to hypothesize that systemic vasculitis, a key pathogenic mechanism in dermatomyositis,[6] may have predisposed toward air leak from alveolar sacs leading on to pneumomediastinum. Our case illustrates a rare but potentially fatal complication of dermatomyositis, which fortunately had a favorable outcome.

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

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Le Goff B, Chérin P, Cantagrel A, Gayraud M, Hachulla E, Laborde F, et al. Pneumomediastinum in interstitial lung disease associated with dermatomyositis and polymyositis. Arthritis Rheum 2009;61:108-18.  Back to cited text no. 1
    
2.
Dogra S, Suri D, Shah R, Rawat A, Singh S, Sodhi KS. Spontaneous pneumomediastinum: A rare complication of juvenile dermatomyositis. Int J Rheum Dis 2012;15:e131-3.  Back to cited text no. 2
    
3.
Zhang L, Shen M, Zhang F, Tang F. Survival analysis and risk factors for mortality in connective tissue disease-associated pneumomediastinum. Rheumatol Int 2014;34:1657-63.  Back to cited text no. 3
    
4.
Ma X, Chen Z, Hu W, Guo Z, Wang Y, Kuwana M, et al. Clinical and serological features of patients with dermatomyositis complicated by spontaneous pneumomediastinum. Clin Rheumatol 2016;35:489-93.  Back to cited text no. 4
    
5.
Neves Fde S, Shinjo SK, Carvalho JF, Levy-Neto M, Borges CT. Spontaneous pneumomediastinum and dermatomyositis may be a not so rare association: Report of a case and review of the literature. Clin Rheumatol 2007;26:105-7.  Back to cited text no. 5
    
6.
Lahoria R, Selcen D, Engel AG. Microvascular alterations and the role of complement in dermatomyositis. Brain 2016;139(Pt 7):1891-903.  Back to cited text no. 6
    


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