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Alveolar proteinosis

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https://www.readbyqxmd.com/read/28514019/does-dust-associated-pulmonary-alveolar-proteinosis-represent-an-autoimmune-disorder
#1
Bilge Uzmezoglu, Cebrail Simsek, Sevtap Gulgosteren, Berna E Gebesoglu
The role of autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF) in the development of secondary pulmonary alveolar proteinosis (PAP) in patients exposed to occupational and environmental dust remains unclear. Herein, we describe two cases of secondary PAP who had GM-CSF antibodies and absence of STAT5 phosphorylation index, suggestive of a potential relationship between the appearance of GM-CSF antibodies and environmental dust exposure. However, whether the presence of GM-CSF antibodies is a part of the pathological process or represents an epiphenomenon is currently unknown...
June 2017: American Journal of Industrial Medicine
https://www.readbyqxmd.com/read/28512724/congenital-pulmonary-alveolar-proteinosis-from-birth-to-ten-years-of-age
#2
Sandra Alavuk Kundović, Ljiljana Popović
Pulmonary alveolar proteinosis is a rare lung disease in which lipoproteinaceous material accumulates within the alveoli, interfering with gas exchange. The disease is classified into congenital, secondary, and acquired. The congenital form includes inborn errors of surfactant metabolism, lysinuric protein intolerance and mutations in the components of granulocyte-macrophage colony-stimulating factor receptor. The main symptoms are non-specific. The radiologic appearance of pulmonary alveolar proteinosis is bilateral, symmetric and perihilar airspace consolidation...
May 17, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28507378/case-series-of-rare-interstitial-lung-disease-ild
#3
Naveed Haroon Rashid, Saba Farooq, Mohammad Ahmed, Ali Bin Sarwar Zubairi
Pulmonary alveolar proteinosis (PAP) is a rare lung disease where periodic acid Schiff (PAS)-positive eosinophilic material accumulates in the alveoli of the lungs. Here we describe two cases of young males who presented with dynpnoea and weight loss. The HRCT scan of the chest in both cases showed the typical "crazy-paving" pattern and lung biopsies confirmed the diagnosis of PAP. They showed remarkable symptomatic improvement with therapeutic whole lung lavage.
May 2017: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/28500076/stat5-is-required-for-cd103-dendritic-cell-and-alveolar-macrophage-development-and-protection-from-lung-injury
#4
William E Eddy, Ke-Qin Gong, Bryan Bell, William C Parks, Steven F Ziegler, Anne M Manicone
We tested the role of Stat5 in dendritic cell and alveolar macrophage (AM) homeostasis in the lung using CD11c-cre mediated deletion (Cre(+)5(f/f)). We show that Stat5 is required for CD103(+) dendritic cell and AM development. We found that fetal monocyte maturation into AMs was impaired in Cre(+)5(f/f) mice, and we also confirmed impaired AM development of progenitor cells using mixed chimera experiments. In the absence of Stat5 signaling in AMs, mice developed alveolar proteinosis with altered lipid homeostasis...
May 12, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28489417/a-semiquantitative-ct-grading-system-for-evaluating-therapeutic-response-in-pulmonary-alveolar-proteinosis
#5
Sayoko Tokura, Masanori Akira, Tomohisa Okuma, Ryushi Tazawa, Toru Arai, Chikatoshi Sugimoto, Akiko Matsumuro, Masaki Hirose, Toshinori Takada, Koh Nakata, Haruyuki Ishii, Yasunori Kasahara, Masayuki Hojo, Shinya Ohkouchi, Yoshiko Tsuchihashi, Masanori Yokoba, Ryosuke Eda, Hideaki Nakayama, Takahito Nei, Konosuke Morimoto, Yasuyuki Nasuhara, Masahito Ebina, Toshio Ichiwata, Koichiro Tatsumi, Etsuro Yamaguchi, Yoshikazu Inoue
RATIONALE: A useful semi-quantitative method of using CT images to evaluate therapeutic response in pulmonary alveolar proteinosis (PAP) has not been established, although the extent score or grading score of ground-glass opacities has been used. OBJECTIVES: The purpose of this study was to establish a semi-quantitative method for evaluating therapeutic response in PAP. METHODS: CT scans obtained within one month before and after the therapy from 32 patients with PAP who participated in a multicenter phase 2 trial of GM-CSF inhalation therapy were evaluated by two chest radiologists independently...
May 10, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28489415/hepatic-steatosis-accompanies-pulmonary-alveolar-proteinosis
#6
Alan N Hunt, Anagha Malur, Tual Monfort, Pavlos Lagoudakis, Sumeet Mahajan, Anthony D Postle, Mary Jane Thomassen
Maintenance of tissue-specific organ lipid compositions characterises mammalian lipid homeostasis. Lung and liver synthesise mixed phosphatidylcholine (PC) molecular species subsequently "tailored" for function. Lungs progressively enrich disaturated PC (DSPC) directed to lamellar body (LB) surfactant stores prior to secretion. Liver accumulates polyunsaturated PC directed to VLDL assembly and secretion, or triglyceride stores. In each tissue, selective PC species enrichment mechanisms lie at the heart of effective homeostasis...
May 10, 2017: American Journal of Respiratory Cell and Molecular Biology
https://www.readbyqxmd.com/read/28469724/simultaneous-presentation-of-two-noninflammatory-lung-diseases-in-an-hiv-infected-patient
#7
Rafael Martínez-Girón, Santiago Martínez-Torre
The simultaneous presentation of two noninflammatory pulmonary diseases, pulmonary alveolar proteinosis and Kaposi's sarcoma (Ks), in an HIV-infected patient, is described. A 29-year-old black race patient was admitted to the hospital because of general malaise, weight loss, dyspnea, chest pain, and cough with hemoptoic expectoration. Chest X-rays revealed a patchy bilateral alveolar pattern with a tendency toward the formation of condensations. The serological test revealed HIV positivity (CD4 counts of 393 cells/mm(3))...
April 2017: Annals of Thoracic Medicine
https://www.readbyqxmd.com/read/28464852/a-newly-identified-novel-variant-in-the%C3%A2-csf2ra-gene-in-a%C3%A2-child-with-pulmonary-alveolar-proteinosis-a-case-report
#8
Adel S Al-Haidary, Wadha Alotaibi, Sami A Alhaider, Suhail Al-Saleh
BACKGROUND: The congenital form of pulmonary alveolar proteinosis due to colony stimulating factor 2 receptor alpha gene mutations is a rare disease with only a few cases reported worldwide. In this study we report a new case of pulmonary alveolar proteinosis with a novel variant in colony stimulating factor 2 receptor alpha gene. CASE PRESENTATION: A 5-year-old Saudi boy presented with a history of progressive dyspnea over 6 months; he was diagnosed as having pulmonary alveolar proteinosis...
May 2, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28421817/sequential-gm-csf-inhalation-after-whole-lung-lavage-for-pulmonary-alveolar-proteinosis-a-report-of-five-intractable-cases
#9
Shinya Ohkouchi, Keiichi Akasaka, Toshio Ichiwata, Shu Hisata, Hideya Iijima, Toshinori Takada, Hiroki Tsukada, Hideaki Nakayama, Jun-Ichi Machiya, Toshiya Irokawa, Hiromasa Ogawa, Yoko Shibata, Masakazu Ichinose, Masahito Ebina, Toshihiro Nukiwa, Hajime Kurosawa, Koh Nakata, Ryushi Tazawa
Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disease characterized by the excessive accumulation of surfactant proteins within the alveolar spaces and by higher titers of autoantibodies to granulocyte/macrophage colony stimulating factor (GM-CSF) in the serum and bronchoalveolar lavage fluid (BALF). The antibodies inhibit the maturation and phagocytosis of alveolar macrophages (AMs). Although the standard therapy for aPAP has been whole-lung lavage (WLL), this procedure is invasive and needs to be repeated for several years...
April 19, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28393663/herpes-simplex-virus-cytomegalovirus-and-pneumocystis-jiroveci-pneumonia-in-a-treatment-naive-hiv-positive-patient-with-pulmonary-alveolar-proteinosis-case-report
#10
Anita Bapat, Eliahu Bishburg, Sandhya Nagarakanti
Infection with multiple pathogens concurrently has become less common since the introduction of potent antiretroviral agent and effective prophylactic agents. We describe a patient with pulmonary alveolar proteinosis (PAP) admitted with pneumonia who was found to have AIDS and diagnosed with Pneumocystis jiroveci pneumonia, human herpesvirus type 1 (HHV-1), and a concomitant cytomegalovirus viremia. Polymerase chain reaction viral load was used for diagnosis of HHV-1 and follow-up. The patient was treated with trimethoprim-sulfamethoxazole and ganciclovir and had a resolution of pneumonia...
January 1, 2017: Journal of the International Association of Providers of AIDS Care
https://www.readbyqxmd.com/read/28379131/-pulmonary-alveolar-proteinosis-role-of-gm-csf-antibodies
#11
REVIEW
Glenda Ernst, Alejandro Salvado, Pedro Grynblat, Gabriela Tabaj, Oscar Garcia, Victor Hugo Cambursano, P Young
Pulmonary alveolar proteinosis is a rare illness characterized by alterations in the normal depuration of surfactants from the alveolar space in a process dependent of the granulocyte-monocyte colony stimulating factor (GM-CSF). The most of the patients develop antibodies that neutralize this factor, avoiding the normal homeostasis of surfactants. Therapeutic options include the total lung washes and administration of GM-CSF when the washes fail. Despite the contribution related with the knowledge about of the nature of the disease, the measurement of serum antibodies anti-GM-CSF is not a routine technique...
2017: Revista de la Facultad de Ciencias Médicas
https://www.readbyqxmd.com/read/28301535/quantitative-assessment-of-pulmonary-alveolar-proteinosis-pap-with-ultra-dose-ct-and-correlation-with-pulmonary-function-tests-pfts
#12
Xin Sui, Qianni Du, Kai-Feng Xu, Xinlun Tian, Lan Song, Xiao Wang, Xiaoli Xu, Zixing Wang, Yuyan Wang, Jun Gu, Wei Song, Zhengyu Jin
BACKGROUND: The purpose of this study was to investigate whether ultra-low-dose chest computed tomography (CT) can be used for visual assessment of CT features in patients with pulmonary alveolar proteinosis (PAP) and to evaluate the relationship between the quantitative analysis of the ultra-low-dose CT scans and the pulmonary function tests (PFTs). METHODS: Thirty-eight patients (mean [SD] age, 44.47 [12.28] years; 29 males, 9 females) with PAP were enrolled and subjected to two scans each with low-dose CT (reference parameters: 120 kV and 50 mAs) and ultra-low-dose CT (reference parameters, 80 kV, 25 mAs)...
2017: PloS One
https://www.readbyqxmd.com/read/28289977/location-function-and-ontogeny-of-pulmonary-macrophages-during-the-steady-state
#13
REVIEW
Natalio Garbi, Bart N Lambrecht
The lung is continuously exposed to potentially hazardous environmental challenges in the form of inert material and microbes. Pulmonary macrophages are critical in maintaining a low inflammatory context in the lung to facilitate optimal gas exchange. During infection, however, they mediate the immediate response to invading microorganisms in coordination with epithelial cells and other tissue-resident immune cells including dendritic cells, innate lymphocytes and memory T cells, and pulmonary interstitial macrophages...
April 2017: Pflügers Archiv: European Journal of Physiology
https://www.readbyqxmd.com/read/28275476/serum-kl-6-in-pulmonary-alveolar-proteinosis-china-compared-historically-with-germany-and-japan
#14
Wen-Liang Guo, Zi-Qing Zhou, Lu Chen, Zhu-Quan Su, Chang-Hao Zhong, Yu Chen, Shi-Yue Li
BACKGROUND: KL-6 is a biomarker of the severity of pulmonary alveolar proteinosis (PAP). We noticed a significant difference in the mean serum KL-6 level between Japanese and Caucasian patients. To assess the clinical value of serum KL-6 in Chinese PAP patients, and to compare the differences in serum KL-6 levels in Chinese patients and patients of other ethnicities. METHODS: From 2014-2016, we prospectively examined 37 Chinese Han patients with PAP, measured their serum KL-6 levels, evaluated the correlation between initial KL-6 levels and clinical variables, and compared our results with studies from Japan and Germany (similar methods were used)...
February 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28270188/pulmonary-alveolar-proteinosis-and-first-successful-whole-lung-lavage-in-sri-lanka-a-case-report
#15
Janith Galhenage, Buddhika Weerasinghe, Wadasinghe Dilesha, Roshana Constantine, Bandu Gunasena
BACKGROUND: Pulmonary alveolar proteinosis is a rare disease characterized by accumulation of lipoproteinaceous material within alveoli. There are three clinically distinct forms: congenital, acquired and secondary. Whole lung lavage is currently the gold standard therapy for severe cases of pulmonary alveolar proteinosis. In Sri Lanka this is the first reported successful whole lung lavage for a patient with pulmonary alveolar proteinosis. CASE PRESENTATION: We describe the case of a 15-year-old Sri Lankan girl who presented with symptoms of progressive shortness of breath and dry cough for 6 months' duration...
March 8, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28264879/abcg1-regulates-pulmonary-surfactant-metabolism-in-mice-and-men
#16
Thomas Q de Aguiar Vallim, Elinor Lee, David J Merriott, Christopher N Goulbourne, Joan Cheng, Angela Cheng, Ayelet Gonen, Ryan M Allen, Elisa N D Palladino, David A Ford, Tisha Wang, Ángel Baldán, Elizabeth J Tarling
Idiopathic pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by accumulation of surfactant. Surfactant synthesis and secretion are restricted to epithelial type 2 (T2) pneumocytes (also called T2 cells). Clearance of surfactant is dependent upon T2 cells and macrophages. ABCG1 is highly expressed in both T2 cells and macrophages. ABCG1-deficient mice accumulate surfactant, lamellar body-loaded T2 cells, lipid-loaded macrophages, B-1 lymphocytes, and immunoglobulins, clearly demonstrating that ABCG1 has a critical role in pulmonary homeostasis...
May 2017: Journal of Lipid Research
https://www.readbyqxmd.com/read/28230086/classification-of-idiopathic-interstitial-pneumonias-using-anti-myxovirus-resistance-protein-1-autoantibody
#17
Yoshimasa Hamano, Hiroshi Kida, Shoichi Ihara, Akihiro Murakami, Masahiro Yanagawa, Ken Ueda, Osamu Honda, Lokesh P Tripathi, Toru Arai, Masaki Hirose, Toshimitsu Hamasaki, Yukihiro Yano, Tetsuya Kimura, Yasuhiro Kato, Hyota Takamatsu, Tomoyuki Otsuka, Toshiyuki Minami, Haruhiko Hirata, Koji Inoue, Izumi Nagatomo, Yoshito Takeda, Masahide Mori, Hiroyoshi Nishikawa, Kenji Mizuguchi, Takashi Kijima, Masanori Kitaichi, Noriyuki Tomiyama, Yoshikazu Inoue, Atsushi Kumanogoh
Chronic fibrosing idiopathic interstitial pneumonia (IIP) can be divided into two main types: idiopathic pulmonary fibrosis (IPF), a steroid-resistant and progressive disease with a median survival of 2-3 years, and idiopathic non-specific interstitial pneumonia (INSIP), a steroid-sensitive and non-progressive autoimmune disease. Although the clinical courses of these two diseases differ, they may be difficult to distinguish at diagnosis. We performed a comprehensive analysis of serum autoantibodies from patients definitively diagnosed with IPF, INSIP, autoimmune pulmonary alveolar proteinosis, and sarcoidosis...
February 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28216546/bach2-controls-homeostasis-of-eosinophils-by-restricting-the-type-2-helper-function-of-t-cells
#18
Yuki Sato, Hiroki Kato, Risa Ebina-Shibuya, Ari Itoh-Nakadai, Ryuhei Okuyama, Kazuhiko Igarashi
Bach2 is a transcription factor which represses its target genes and plays important roles in the differentiation of B and T lymphoid cells. Bach2-deficient (KO) mice develop severe pulmonary alveolar proteinosis, which is associated with increased numbers of granulocytes and T cells. Bach2 is essential for the regulation of T cells, but its role in the regulation of granulocytes is not clear. Here, we observed increased numbers of eosinophils but not neutrophils in the bone marrow, spleen, peripheral blood, and bronchoalveolar lavage fluids of Bach2 KO mice compared with those of wild-type (WT) mice...
March 2017: Tohoku Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28212655/elderly-onset-hereditary-pulmonary-alveolar-proteinosis-and-its-cytokine-profile
#19
Masayuki Ito, Kazuyuki Nakagome, Hiromitsu Ohta, Keiichi Akasaka, Yoshitaka Uchida, Atsushi Hashimoto, Ayako Shiono, Toshinori Takada, Makoto Nagata, Jun Tohyama, Koichi Hagiwara, Minoru Kanazawa, Koh Nakata, Ryushi Tazawa
BACKGROUND: Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by surfactant accumulation, and is caused by disruption of granulocyte/macrophage colony-stimulating factor (GM-CSF) signaling. Abnormalities in CSF2 receptor alpha (CSF2RA) were reported to cause pediatric hereditary PAP. We report here the first case of CSF2RA-mutated, elderly-onset hereditary (h) PAP. CASE PRESENTATION: The patient developed dyspnea on exertion, and was diagnosed with PAP at the age of 77 years, based on findings from chest computed tomography scan and bronchoalveolar lavage...
February 17, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28202867/gm-csf-autoantibody-positive-pulmonary-alveolar-proteinosis-with-simultaneous-myeloproliferative-neoplasm
#20
REVIEW
Naoto Imoto, Nakashima Harunori, Katsuya Furukawa, Naoyuki Tange, Atsushi Murase, Masaya Hayakawa, Masatoshi Ichihara, Yosuke Iwata, Hiroshi Kosugi
Pulmonary alveolar proteinosis (PAP) is classified as autoimmune, secondary, or genetic. We herein describe a 69-year-old man with autoimmune PAP, simultaneously diagnosed with myeloproliferative neoplasm (MPN). Two years after the diagnosis, the MPN progressed to acute myeloid leukemia, and the patient died from an alveolar hemorrhage during remission induction chemotherapy. Throughout the clinical course, no progression of PAP was observed, despite the progression to leukemia. There are few reports of autoimmune PAP with hematological malignancy, and this case demonstrated that an evaluation for GM-CSF autoantibodies is important for distinguishing the autoimmune and secondary forms of PAP, even if the patient has hematological malignancy...
2017: Internal Medicine
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