A 61-year-old Caucasian woman was diagnosed 13 years earlier with IPF by transbronchial lung biopsy.
The patient had been treated with corticosteroids up to two years before.
She had received continuous home therapy for 20 months.
Six years before she had been diagnosed with duodenal ulcer and had no other relevant history.
In the week prior to admission she had frequent and severe cough attacks, without expectoration, fever or increase in her habitual dyspnea.
On the day of admission she came to the emergency department with sudden swelling in the left cervical region, intermittent midthoracic pain, which increased with cough and respiratory movements, and dyspnea.
Physical examination revealed tachypnea (32 breaths/minute), tachycardia (120 beats/minute), swelling and crepitation in the left cervical region, both auscultatory fields fine.
The most relevant analytical data were: leukocytes 23.9x109/l (80% neutrophils), hemoglobin 132 g/l, hematocrit 39.4%, LDH 342 U/l and creatine kinase 123 U/l.
Basal arterial gas values were: pH 7.47, PO2 54 torr, PCO2 36 torr, HCO3- 27.1 / l and oxygen saturation 90%.
The electrocardiogram showed sinus rhythm at 120 beats per minute, the rest of the tracing being normal.
A neck X-ray showed a predominantly left cervical emphysema.
A chest X-ray showed a pattern of bilateral pulmonary fibrosis more pronounced in the lower lung areas, with evidence of anterior pneumomediastinum in the lateral lung.
A computed tomography (CT) scan of the chest revealed a marked pattern of bilateral pulmonary fibrosis, pneumomediastinum and large air bubbles in the left upper lobe, with no evidence of pneumothorax.
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The evolution was favorable with symptomatic treatment, disappearing after 14 days in controls subcutaneous emphysema and pneumomediastinum.
