open access

Vol 81, No 1 (2013)
CASE REPORTS
Published online: 2012-12-13
Submitted: 2013-02-22
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Pneumocystosis in a patient with lymphangioleiomyomatosis

Tomasz Mydłowski, Elżbieta Radzikowska, Karina Oniszh, Ewa Szczepulska-Wójcik, Paulina Jaguś, Elżbieta Wiatr
Pneumonol Alergol Pol 2013;81(1):61-67.

open access

Vol 81, No 1 (2013)
CASE REPORTS
Published online: 2012-12-13
Submitted: 2013-02-22

Abstract

A 47-year-old, non-smoking woman was admitted to the National Tuberculosis and Lung Diseases Research Institute for diagnosis of progressive exertional dyspnoea and numerous small thin-walled, air-filled cysts equally distributed throughout both lungs revealed in HRCT (high resolution computed tomography) examination. Histological assessment of specimens obtained by open lung biopsy revealed proliferation of immature smooth muscle, showing the expression of the antigen HMB45. On this basis, diagnosis of lymphangioleiomyomatosis was established. The disease caused essential ventilation damage of the lungs (FEV1 1.34 L; 39.71% pred, VC 4.02 L; 94.96% pred, FEV1/ /VC 0.33–4 1.81% pred, DLCO 3.65 mmol/min/Kpa 38.35% pred).During the observation, despite the lack of immunological disorders, the patient developed Pneumocystis jiroveci pneumonia (PCP) that was treated with trimethoprimsulfamethoxazole. Lymphangioleiomyomatosis is a rare disease which results from a defect of TSC genes. The disease is not related to immunological defects or disorders. However, the considerable cystic destruction of the lungs can predispose the patient to opportunistic infections such as the one in the presented case.

Abstract

A 47-year-old, non-smoking woman was admitted to the National Tuberculosis and Lung Diseases Research Institute for diagnosis of progressive exertional dyspnoea and numerous small thin-walled, air-filled cysts equally distributed throughout both lungs revealed in HRCT (high resolution computed tomography) examination. Histological assessment of specimens obtained by open lung biopsy revealed proliferation of immature smooth muscle, showing the expression of the antigen HMB45. On this basis, diagnosis of lymphangioleiomyomatosis was established. The disease caused essential ventilation damage of the lungs (FEV1 1.34 L; 39.71% pred, VC 4.02 L; 94.96% pred, FEV1/ /VC 0.33–4 1.81% pred, DLCO 3.65 mmol/min/Kpa 38.35% pred).During the observation, despite the lack of immunological disorders, the patient developed Pneumocystis jiroveci pneumonia (PCP) that was treated with trimethoprimsulfamethoxazole. Lymphangioleiomyomatosis is a rare disease which results from a defect of TSC genes. The disease is not related to immunological defects or disorders. However, the considerable cystic destruction of the lungs can predispose the patient to opportunistic infections such as the one in the presented case.
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Keywords

lymphangioleiomyomatosis; pneumocystosis; opportunistic infections; rare diseases; interstitial lung diseases

About this article
Title

Pneumocystosis in a patient with lymphangioleiomyomatosis

Journal

Advances in Respiratory Medicine

Issue

Vol 81, No 1 (2013)

Pages

61-67

Published online

2012-12-13

Bibliographic record

Pneumonol Alergol Pol 2013;81(1):61-67.

Keywords

lymphangioleiomyomatosis
pneumocystosis
opportunistic infections
rare diseases
interstitial lung diseases

Authors

Tomasz Mydłowski
Elżbieta Radzikowska
Karina Oniszh
Ewa Szczepulska-Wójcik
Paulina Jaguś
Elżbieta Wiatr

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