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95]

Case R e po r t

Fatal recurrence of pneumothorax in an adult

dermatomyositis
Rakesh Agarwal,
DurjoyLahiri,
AmritaBiswas,
JotidebMukhopadhyay,
Manoj Kumar Roy

Department of General Medicine, IPGMER and SSKM Hospital, Kolkata, West Bengal, India
Address for correspondence:
Dr.Rakesh Agarwal, 243, G.T Road(N), Laxmi Niketan, Flat2E, Liluah, Howrah711204,
Kolkata, West Bengal, India.
Email:dr.agarwal.rakesh@hotmail.com

Abstract
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 Xrays revealed it to be a
case of spontaneous pneumothorax. She had a history of a similar episode 15days 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.
Key words: Dermatomyositis, recurrent pneumothorax dermatomyositis, spontaneous pneumothorax

INTRODUCTION
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
A 54yearold female presented with sudden onset
breathlessness for 6 h before admission. There was no
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DOI:
10.4103/2320-8775.146846

17

history of chest pain, cough or haemoptysis. She was on

oral steroid therapy in the form of prednisolone 50mg/day
for DM with her disease activity. She was diagnosed to be
a case of DM at the age of 53years 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 antiRo 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 [Figure1] 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[Figures2]. Her arterial blood
gas analysis revealed respiratory acidosis.
Routine blood examination was as followshemoglobin
8.1 gm%, Total leucocyte count 8700/cubic mm,
differential count N65, L22, E5, M8, B0, platelets

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Agarwal, etal.: Fatal recurrence of pneumothorax in an adult dermatomyositis

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, SSA/B, Jo1 and antinuclear
antibody were negative. Erythrocyte sedimentation rate
was 48mm/1sth.
On detailed history taking, it was revealed that she suffered
a similar episode of shortness of breath only 15days
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 examinationreport done 1year back
revealed picture consistent with DM.

Figure1: Image of the patient showing heliotrope rash

Immediately an intercostal drain was inserted, and

pneumothorax drained[Figure3]. However, the patient
passed away the next day due to respiratory failure despite
vigorous resuscitative attempts.

DISCUSSION
Dermatomyositis is a clinicopathological 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 druginduced
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. [35] Rupture of a
pleural necrobiotic nodule or sub pleural bleb has been
described to cause pneumothorax in RA, SLE and
scleroderma.[35] 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

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

Figure 3: Image showing right sided pneumothorax (resolving) with

intercostal drain placement

had a good prognosis.[7] Jansen etal. 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

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Agarwal, etal.: Fatal recurrence of pneumothorax in an adult dermatomyositis

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
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DalakasMC. Polymyositis, dermatomyositis, and inclusion body

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4. Ayzenberg O, Reiff DB, Levin L. Bilateral pneumothoraces and
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5. YarbroughJW, SealyWC, MillerJA. Thoracic surgical problems
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6. Singsen BH, Tedford JC, Platzker AC, Hanson V. Spontaneous
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7. BradleyJD. Spontaneous pneumomediastinum in adult
dermatomyositis. Ann Rheum Dis 1986;45:7802.
8. JansenTL, BarreraP, van EngelenBG, CoxN, LaanRF, van
de Putte LB. Dermatomyositis with subclinical myositis and
spontaneous pneumomediastinum with pneumothorax: Case report
and review of the literature. Clin Exp Rheumatol 1998;16:7335.
9. KimHJ, HongYK, YooWH. Dermatomyositis, complicated with
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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.
Source of Support: Nil, Conflict of Interest: Nil.

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