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Volume 81
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10.5603/ARM.27528
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Advances in Respiratory Medicine is published by MDPI from Volume 90 Issue 4 (2022). Previous articles were published by another publisher in Open Access under a CC-BY (or CC-BY-NC-ND) licence, and they are hosted by MDPI on mdpi.com as a courtesy and upon agreement with Via Medica.
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Case Report

Pneumocystozowe Zapalenie Płuc u Chorej na Limfangioleiomiomatozę

by
Tomasz Mydłowski
1,*,
Elżbieta Radzikowska
2,
Karina Oniszh
2,
Ewa Szczepulska-Wójcik
4,
Paulina Jaguś
5 and
Elżbieta Wiatr
2
1
II Oddział Chorób Płuc i Alergii Układu Oddechowego, Wojewódzki Zespół Zakładów Opieki Zdrowotnej, Centrum Leczenia Chorób Płuc i Rehabilitacji, ul. Okólna 181, 91-520 Łodzi, Poland
2
III Kliniki Chorób Płuc, Instytut Gruźlicy i Chorób Płuc, 01-138 Warszawa, Poland
3
Zakład Radiologii, Instytut Gruźlicy i Chorób Płuc, 01-138 Warszawa, Poland
4
Zakład Anatomii Patologicznej, Instytut Gruźlicy i Chorób Płuc, 01-138 Warszawa, Poland
5
Zakład Diagnostyki Molekularnej, Instytut Gruźlicy i Chorób Płuc, 01-138 Warszawa, Poland
*
Author to whom correspondence should be addressed.
Adv. Respir. Med. 2013, 81(1), 61-67; https://doi.org/10.5603/ARM.27528
Submission received: 19 April 2012 / Revised: 13 December 2012 / Accepted: 13 December 2012 / Published: 13 December 2012

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.
Keywords: lymphangioleiomyomatosis; pneumocystosis; opportunistic infections; rare diseases; interstitial lung diseases lymphangioleiomyomatosis; pneumocystosis; opportunistic infections; rare diseases; interstitial lung diseases

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MDPI and ACS Style

Mydłowski, T.; Radzikowska, E.; Oniszh, K.; Szczepulska-Wójcik, E.; Jaguś, P.; Wiatr, E. Pneumocystozowe Zapalenie Płuc u Chorej na Limfangioleiomiomatozę. Adv. Respir. Med. 2013, 81, 61-67. https://doi.org/10.5603/ARM.27528

AMA Style

Mydłowski T, Radzikowska E, Oniszh K, Szczepulska-Wójcik E, Jaguś P, Wiatr E. Pneumocystozowe Zapalenie Płuc u Chorej na Limfangioleiomiomatozę. Advances in Respiratory Medicine. 2013; 81(1):61-67. https://doi.org/10.5603/ARM.27528

Chicago/Turabian Style

Mydłowski, Tomasz, Elżbieta Radzikowska, Karina Oniszh, Ewa Szczepulska-Wójcik, Paulina Jaguś, and Elżbieta Wiatr. 2013. "Pneumocystozowe Zapalenie Płuc u Chorej na Limfangioleiomiomatozę" Advances in Respiratory Medicine 81, no. 1: 61-67. https://doi.org/10.5603/ARM.27528

APA Style

Mydłowski, T., Radzikowska, E., Oniszh, K., Szczepulska-Wójcik, E., Jaguś, P., & Wiatr, E. (2013). Pneumocystozowe Zapalenie Płuc u Chorej na Limfangioleiomiomatozę. Advances in Respiratory Medicine, 81(1), 61-67. https://doi.org/10.5603/ARM.27528

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