A 21-year-old previously healthy, non-smoking woman with no family medical history presented with lower respiratory infections characterized by recurrent coughing, mucopurulent lesions, and dyspnea, which is why she was studied with radiography.
For this reason he was referred to San Juan de Dios Hospital in July 2009.
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Pathological mapping, biochemical and coagulation studies were performed.
Koch culture and tuberculin test were negative.
In order to complete the study a fibro bronchus was performed showing slightly congestive mucosa. Biopsies showed little fibrin deposit and intra-alveolar pigmented histiocytes.
Bronchoalveolar lavage showed no abnormalities.
The patient was stable, with dry cough and dyspnea on exertion.
The patient was referred to the National Thoracic Institute for further study.
Due to the clinical and radiological characteristics, it was decided to study rheumatologic diseases compatible with this presentation in a young woman, such as Wegener's granulomatosis with pulmonary involvement and antinuclear antibodies EN syndrome that were negative.
The possibility of mixed cryoglobulinemia was raised, so immunoglobulin and serology for hepatitis C virus normal immunoglobulin levels and absence of anti-HCV antibodies were requested.
Considering the possibility of a non-identified infection Aspergillus fumigatus antibodies were measured, which were positive with a value of 0.841 AU (range up to 0.2 AU).
Asperguillus positivity did not explain the picture in its entirety, so we decided to investigate a diffuse lung disease and request DLCO1: 70% FEVDLCO, 77%1/ 59% FEVFLCO),
During the study period, symptoms progressed, adding hemoptoic expectoration, wheezing, weight loss of 8 kilos in two months and progression of dyspnea.
In this context, computed tomography of the chest is requested, which shows multiple nodular lesions of neoplasic appearance.
A new fibro bronchus is performed showing mucosal melanoma 2010 granuloma. Biopsies are taken that report alveolar desquamation, macrophages loaded with hemosiderin and presence of moderate chronic inflammatory infiltrate.
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Given the imaging findings and alterations in pulmonary function, it was decided to perform a surgical lung biopsy, which was performed via endoscopy in February 2010 without incidents.
The biopsy showed a partially distorted pulmonary parenchyma, nodular areas with vascular proliferation and endothelial cells containing a
No mitosis was observed, the study was completed with immunohistochemistry.
This study showed CD31+ and CD34+ in endothelial cells with a positive Ki-67 staining pattern in 2% A and S-100 and cytokeratin negative cells.
These findings are consistent with pulmonary ESA.
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The case was presented to the respiratory disease committee and it was initiated treatment with prednisone and azathioprine, presented good response, but after 5 months it was initially decided bad nausea, vomiting and arthralgias.
Therefore, the patient was presented again in a respiratory disease committee and an expectant management was decided.
The patient decides to become pregnant after 1 year of treatment, comes to control and performs a functional study, showing stable values, so it was kept under strict control by bronchopulmonary and high risk cesarean section, achieving a pregnancy incident.
Currently, she is 27 years old, has been kept under expectant management, has her second pregnancy and attends regular monitoring, showing the absence of progression of lesions or functional impairment.
