title: [Pneumocystosis in a patient with lymphangioleiomyomatosis].
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.
authors.affiliation: II Oddział Chorób Płuc i Alergii Układu Oddechowego, Wojewódzki Zespół Zakładów Opieki Zdrowotnej, Centrum Leczenia Chorób Płuc i Rehabilitacji w Łodzi. tmydlowski@op.pl
authors.forename: Tomasz
authors.initials: T
authors.lastname: Mydłowski
authors.forename: Elżbieta
authors.initials: E
authors.lastname: Radzikowska
authors.forename: Karina
authors.initials: K
authors.lastname: Oniszh
authors.forename: Ewa
authors.initials: E
authors.lastname: Szczepulska-Wójcik
authors.forename: Paulina
authors.initials: P
authors.lastname: Jaguś
authors.forename: Elżbieta
authors.initials: E
authors.lastname: Wiatr
mesh_terms: D000890:Anti-Infective Agents / Q000627:therapeutic use; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D018192:Lymphangioleiomyomatosis / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D008875:Middle Aged; D011020:Pneumonia, Pneumocystis / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D016896:Treatment Outcome; D015662:Trimethoprim, Sulfamethoxazole Drug Combination / Q000008:administration & dosage
chemical_list: D000890:Anti-Infective Agents; D015662:Trimethoprim, Sulfamethoxazole Drug Combination