Pulmonary Manifestations among Patients with Primary Biliary Cirrhosis

Köksal, Deniz; Köksal, Aydın Şeref; Gürakar, Ahmet

doi:10.14218/jcth.2016.00024

Cited by 9 publications

(9 citation statements)

References 53 publications

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“…Gas exchange abnormality attributed to hypoxaemia and increased breathing efforts may be conducive to pneumothorax. In PBC, it is uncertain but possible that the characteristic CD4 and activated alveolar macrophages predominating inflammatory infiltration would exaggerate pneumatocele wall thinning, which is conducive to pneumothorax [22].…”

Section: Discussionmentioning

confidence: 99%

Pneumocystis jirovecii-related spontaneous pneumothorax, pneumomediastinum and subcutaneous emphysema in a liver transplant recipient: a case report

She

Chok

et al. 2019

BMC Infect Dis

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BackgroundPneumocystis pneumonia (PCP) is a common opportunistic infection caused by Pneumocystis jirovecii. Its incidence at 2 years or more after liver transplant (LT) is < 0.1%. PCP-related spontaneous pneumothorax and/or pneumomediastinum is rare in patients without the human immunodeficiency virus, with an incidence of 0.4–4%.Case presentationA 65-year-old woman who had split-graft deceased-donor LT for primary biliary cirrhosis developed fever, dyspnea and dry coughing at 25 months after transplant. Her immunosuppressants included tacrolimus, mycophenolate mofetil, and prednisolone. PCP infection was confirmed by molecular detection of Pneumocystis jirovecii,in bronchoalveolar lavage. On day-10 trimethoprim-sulphamethoxazole, her chest X-ray showed subcutaneous emphysema bilaterally, right pneumothorax and pneumomediastinum. Computed tomography of the thorax confirmed the presence of right pneumothorax, pneumomediastinum and subcutaneous emphysema. She was managed with 7-day right-sided chest drain and a 21-day course of trimethoprim-sulphamethoxazole before discharge.ConclusionLonger period of PCP prophylaxis should be considered in patients who have a higher risk compared to general LT patients. High index of clinical suspicion, prompt diagnosis and treatment with ongoing patient reassessment to detect and exclude rare, potentially fatal but treatable complications are essential, especially when clinical deterioration has developed.

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Section: Discussionmentioning

confidence: 99%

Pneumocystis jirovecii-related spontaneous pneumothorax, pneumomediastinum and subcutaneous emphysema in a liver transplant recipient: a case report

She

Chok

et al. 2019

BMC Infect Dis

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“…Так, ПБХ-ассоциированные респираторные симптомы могут включать в себя кашель -сухой или с отделением мокроты, одышку при физической нагрузке. Отклонения функциональных легочных параметров, в частности снижение диффузионной способности легких (рестриктивные вентиляционные нарушения), развитие различных вариантов интерстициальной пневмонии, описаны у части пациентов с ПБХ [20,21]. Таким образом, выявление интерстициального поражения легких и/или внутригрудной лимфаденопатии у пациента с морфологической картиной гранулематозного поражения печени и серологическими маркерами ПБХ требует тщательного проведения дифференциальной диагностики ПБХ и саркоидоза.…”

Section: Discussionunclassified

Sarcoidosis and primary biliary cholangitis in a patient with cholestasis

Brovko

Akulkina

Kalashnikov

et al. 2021

Terapevticheskii arkhiv

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Primary biliary cholangitis and sarcoidosis are both cholestatic liver diseases. Currently, there are no established specific criteria for distinguishing the diseases from each other; diagnosis is based on the anamnesis, as well as the results of physical, laboratory and instrumental examination. The case report presents a female patient with a rare combination of histologically verified liver sarcoidosis and primary biliary cholangitis. Despite the similar clinical manifestations, the approaches to the treatment of these diseases are completely different, that underlines the importance of the differential diagnosis to exclude combined liver damage.

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“…Наиболее часто выявляется интерстициальное заболевание легких (ИЗЛ), реже -поражение бронхов или развитие легочной гипертензии (ЛАГ). Различные варианты легочной патологии, описанные при ПБХ, представлены в таблице [7].…”

Section: ïîðàAeåíèå ëåãêèõ ïðè ïåðâè÷íîì áèëèàðíîì õîëàíãèòåunclassified

“…[22] представили клинический случай развития лимфоцитарного бронхита и бронхиолита у пациента с ПБХ. Еще в одном исследовании признаки умеренной бронхообструкции описаны у 4 из 67 пациентов с ПБХ [7].…”

Section: ðèñ 1 óòîëùåíèå ìåAeäîëüêîâûõ ïåðåãîðîäîê ñ ôîðìèðîâàíèåì òðàêöèîííûõ áðîíõîýêòàçîâ è î÷àãîâ òðàíñôîðìàöèè ëåãî÷íîé òêàíè ïî òèïunclassified

“…У значительной части пациентов с ПБХ выявлены признаки субклинической альвеолярной инфильтрации, преимущественно состоящей из CD4-лимфоцитов и активированных альвеолярных макрофагов. Одним из ограничений данного исследования было сопутствующее наличие синдрома Шегрена у 7 из 12 пациентов[7]. Так как до 44% пациентов с синдромом Шегрена имеют признаки субклинического альвеолита, однозначно установить причину развития изменений в легких невозможно[12].…”

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Variety of lung involvement in autoimmune liver diseases

Akulkina

Brovko

Sholomova

et al. 2018

Terapevticheskii arkhiv

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The primary autoimmune liver diseases conventionally include primary biliary cholangitis, primary sclerosing cholangitis and autoimmune hepatitis. Despite of primary autoimmune affection of different parts of the hepatobiliary system, in the recent decades, a lot of data has emerged indicating the presence of extrahepatic manifestations of these diseases, in particular, lung lesions, such as nodular and interstitial changes with possible progression and development of fibrosis and respiratory failure. In case of lungs disease, both pulmonary parenchyma and lung vessels, pleura, and intrathoracic lymph nodes can be involved. The most sensitive and specific procedure to assess the extent of the lung lesions and their evolution is high-resolution computed tomography. Due to the possibility of long-term asymptomatic course of the pulmonary disease with development of irreversible changes in patients with autoimmune liver diseases, it seems reasonable to conduct screening studies aimed at early detection and treatment of lung lesions in this population.

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Pulmonary Manifestations among Patients with Primary Biliary Cirrhosis

Cited by 9 publications

References 53 publications

Pneumocystis jirovecii-related spontaneous pneumothorax, pneumomediastinum and subcutaneous emphysema in a liver transplant recipient: a case report

Pneumocystis jirovecii-related spontaneous pneumothorax, pneumomediastinum and subcutaneous emphysema in a liver transplant recipient: a case report

Sarcoidosis and primary biliary cholangitis in a patient with cholestasis

Variety of lung involvement in autoimmune liver diseases

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