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Year : 2015  |  Volume : 3  |  Issue : 1  |  Page : 17-19

Fatal recurrence of pneumothorax in an adult dermatomyositis

Department of General Medicine, IPGMER and SSKM Hospital, Kolkata, West Bengal, India

Date of Web Publication12-Dec-2014

Correspondence Address:
Rakesh Agarwal
243, G.T Road (N), Laxmi Niketan, Flat 2E, Liluah, Howrah 711 204, Kolkata, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2320-8775.146846

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Dermatomyositis (DM) is an uncommon idiopathic inflammatory myopathy with characteristic cutaneous involvement that affects children and adults. Association of lung cancer and interstitial lung disease with DM has been well established, but spontaneous pneumothorax is rare. Recurrent spontaneous pneumothorax as a fatal complication in adult DM has not been described until date. We report a rare case of the patient with adult DM who presented with severe dyspnea. Chest X-rays revealed it to be a case of spontaneous pneumothorax. She had a history of a similar episode 15 days back. In the hospital, she was treated with intercostal drain placement, but succumbed despite vigorous resuscitative effort. Our case attempts to highlights the importance of recognizing recurrent pneumothorax as a potential serious complication of the disease.

Keywords: Dermatomyositis, recurrent pneumothorax dermatomyositis, spontaneous pneumothorax

How to cite this article:
Agarwal R, Lahiri D, Biswas A, Mukhopadhyay J, Roy MK. Fatal recurrence of pneumothorax in an adult dermatomyositis. J Assoc Chest Physicians 2015;3:17-9

How to cite this URL:
Agarwal R, Lahiri D, Biswas A, Mukhopadhyay J, Roy MK. Fatal recurrence of pneumothorax in an adult dermatomyositis. J Assoc Chest Physicians [serial online] 2015 [cited 2022 Jun 25];3:17-9. Available from: https://www.jacpjournal.org/text.asp?2015/3/1/17/146846

  Introduction Top

The prevalence of the inflammatory myopathies was estimated at 1 in 100,000. [1] Dermatomyositis (DM) is an uncommon idiopathic inflammatory myopathy with characteristic cutaneous involvement that affects children and adults. [1] Pulmonary involvement is present in approximately 10% of cases in the form of interstitial lung disease and lung cancer. However, spontaneous pneumothorax is relatively unfamiliar in adult DM. Our case is probably the first case to describe recurrent pneumothorax in adult DM as a fatal complication.

  Case report Top

A 54-year-old female presented with sudden onset breathlessness for 6 h before admission. There was no history of chest pain, cough or haemoptysis. She was on oral steroid therapy in the form of prednisolone 50 mg/day for DM with her disease activity. She was diagnosed to be a case of DM at the age of 53 years based on her serum creatine phosphokinase (CPK) level, which was 4332 U/L (normal 90-190 U/L), Muscle biopsy findings (from left deltoid muscle) of perifascicular atrophy (typical of DM) and positive anti-Ro 52 antibody. Her latest CPK level was 35 U/L.

On examination, she was in obvious distress, tachypnoeic (respiratory rate 32/min) and cyanotic. Her heart rate was 120/min. She had typical heliotrope rash around her eyelids [Figure 1] and erythematous rashes over the anterior chest wall. She was afebrile and had an intact sensorium. Chest percussion revealed hyper resonant notes on the right side in infraclavicular and mammary region. Auscultation findings were corroborative with diminished breath sounds on the right hemi thorax. Chest skiagram showed right sided pneumothorax involving approximately 50% of right hemithorax [Figure 2]. Her arterial blood gas analysis revealed respiratory acidosis.
Figure 1: Image of the patient showing heliotrope rash

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Figure 2: Chest X-ray showing massive right sided pneumothorax

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Routine blood examination was as follows - hemoglobin 8.1 gm%, Total leucocyte count 8700/cubic mm, differential count N65, L22, E5, M8, B0, platelets 1.8 lac/cu.mm. Urea 34 mg/dL, creatinine 1.0 mg/dL, random blood sugar 102 mg/dl. Sodium 134 mEq/L, potassium 3.8 mEq/L. Liver function tests were within normal limits. Electrocardiogram and thyroid profile were not contributory. Anti Ro 52 antibody was strongly positive while anti smRNP, SS-A/B, Jo-1 and antinuclear antibody were negative. Erythrocyte sedimentation rate was 48 mm/1 st h.

On detailed history taking, it was revealed that she suffered a similar episode of shortness of breath only 15 days before this current episode. She was treated at another hospital by putting a chest drain, following which her physical condition improved. The drain was removed in due course, and she was discharged in a stable condition. Her histopathological examination report done 1 year back revealed picture consistent with DM.

Immediately an intercostal drain was inserted, and pneumothorax drained [Figure 3]. However, the patient passed away the next day due to respiratory failure despite vigorous resuscitative attempts.
Figure 3: Image showing right sided pneumothorax (resolving) with intercostal drain placement

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  Discussion Top

Dermatomyositis is a clinico-pathological entity characterized by a distinctive cutaneous rash accompanying, or more often preceding, inflammatory myopathy. [1] Besides skin and muscle, the disease can also involve other internal organs, especially the lungs. Pulmonary dysfunction can occur in up to 10% cases of DM and may be due to weakness of the thoracic muscles, interstitial lung disease, or drug-induced pneumonitis, which may cause dyspnea, nonproductive cough, and aspiration pneumonia. [1] However recurrent spontaneous pneumothorax has only been rarely if ever described in the case of adult DM. Moreover, it has never been recognized as a fatal complication of DM till now.

Several pulmonary diseases can be associated with spontaneous pneumothorax. These may include asthma, chronic bronchitis, emphysema, bronchiectasis, pneumonia, abscess, primary interstitial lung disease. [2] Among connective tissue diseases, spontaneous pneumothorax has been rarely reported as a complication of rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), scleroderma, and childhood DM. [3],[4],[5] Rupture of a pleural necrobiotic nodule or sub pleural bleb has been described to cause pneumothorax in RA, SLE and scleroderma. [3],[4],[5] Sub pleural pulmonary infarctions from the diffuse vasculopathy have been implicated in causing pneumothorax in childhood DM. [6]

Bradley described a case of spontaneous pneumomediastinum and pneumothorax in a case of adult DM , but his case had a good prognosis. [7] Jansen et al. described a patient with DM and incomplete signs of myositis who developed pneumomediastinum, pneumothorax and massive subcutaneous emphysema. [8] Kim et al. reported a rare case of the patient with DM who developed spontaneous pneumomediastinum and subcutaneous emphysema who was successfully treated and recovered. [9] However none of these cases developed recurrent pneumothorax, and all had a good prognosis.

This case illustrates that adult DM may be associated with spontaneous pneumothorax, probably by the same mechanism as described in SLE or childhood DM. Our case highlights the importance of recognizing recurrent pneumothorax as a rare fatal complication of adult DM. More studies are however needed to fully elucidate its mechanism and outcome.

  References Top

Dalakas MC. Polymyositis, dermatomyositis, and inclusion body myositis. In: Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson JL, Loscalzo J, editors. Harrison's Principles of Internal Medicine. 18 th ed., Vol. 2. New York: McGraw Hills; 2012. p. 3509-18.  Back to cited text no. 1
Fraser RG, Parc JA. Diagnosis of Diseases of the Chest. Philadelphia: Saunders; 1970. p. 867-9, 1159-61.  Back to cited text no. 2
Crisp AJ, Armstrong RD, Grahame R, Dussek JE. Rheumatoid lung disease, pneumothorax, and eosinophilia. Ann Rheum Dis 1982;41:137-40.  Back to cited text no. 3
Ayzenberg O, Reiff DB, Levin L. Bilateral pneumothoraces and pleural effusions complicating rheumatoid lung disease. Thorax 1983;38:159-60.  Back to cited text no. 4
Yarbrough JW, Sealy WC, Miller JA. Thoracic surgical problems associated with rheumatoid arthritis. J Thorac Cardiovasc Surg 1975;69:347-54.  Back to cited text no. 5
Singsen BH, Tedford JC, Platzker AC, Hanson V. Spontaneous pneumothorax: A complication of juvenile dermatomyositis. J Pediatr 1978;92:771-4.  Back to cited text no. 6
Bradley JD. Spontaneous pneumomediastinum in adult dermatomyositis. Ann Rheum Dis 1986;45:780-2.  Back to cited text no. 7
Jansen TL, Barrera P, van Engelen BG, Cox N, Laan RF, van de Putte LB. Dermatomyositis with subclinical myositis and spontaneous pneumomediastinum with pneumothorax: Case report and review of the literature. Clin Exp Rheumatol 1998;16:733-5.  Back to cited text no. 8
Kim HJ, Hong YK, Yoo WH. Dermatomyositis, complicated with pneumomediastinum, successfully treated with cyclosporine A: A case report and review of literature. Rheumatol Int 2009;29:1101-4.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3]


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