A 33-year-old woman, physical education teacher, with no history of respiratory, neurological or dermatological diseases.
There was no smoking history and no exercise limitations.
In April 2003, she had a 12-week miscarriage.
In July 2003 she consulted for chest pain and respiratory apremium.
Cho dere pneumothorax was confirmed by computed tomography of the chest that showed scarce bilateral cystoid images.
The patient was discharged in good condition.
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The year 2004 was pregnant, with vaginal delivery in December that year, without incidents.
The patient remained asymptomatic until March 2007, when he presented a left pneumothorax, treated with left pleural sealing and was discharged in good condition.
Chest computed tomography was not performed on that occasion.
On May 3, 2008, she presented a new right pneumothorax.
Computed tomography of the lung showed multiple diffuse cystoid wall images, frankly progressive compared to tomography of the year 2003.
Right pleural drainage and videothoracoscopy were performed, and a diffusely erythematous lung with multiple mucous lesions was detected.
Talc sealing was performed and a lung biopsy was performed, which described a pulmonary parenchyma with preserved architecture in part and focal areas with faulty areas characterized by diffused alveolar spaces and confluent bronchiolar spindle cells, with partial rupture of muscle.
No granulomatous lesions or signs of malignancy, highly suspect of pulmonary lymphangioleiomyomatosis.
1.
Immunohistochemical study showed:
(a) HMB-45 (+) cytoplasmic staining in spindle cells. b) Smooth muscle cell (++) cytoplasmic staining in spindle-cells (40% nuclear-estrogen+ receptor)
Alpha-1-antitrypsin was also normal.
Saturation and plethysmography were performed in August 2008; both were normal and had a 6-minute walk test; distance was within the predictive value, without oxygen broth.
The patient was evaluated by a gynecologist who indicated hormonal blockade with tryptorelin 3.75 mg every month (Decapeptil), Gn-RH analogue, to cause hypoestrogenism monthly ibandron oral reversed calcium intake and prevention.
The treating pneumologist recommended initiating empirical treatment with Sirmus (Rapamune) 2 mg daily, providing the available scientific information, which was accepted by the patient and her husband.
These treatments began in August 2008 with serial control of plasma levels of Sirmus, complete blood count and lipid profile.
